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Summarize this medical case report, ensuring to cover the initial presentation
A 9-year-old, previously healthy girl, was admitted to hospital due to group A streptococcus toxic shock syndrome and severe AKI, requiring immediate continuous renal replacement therapy (CRRT; ). She was transitioned to overnight CRRT and daytime PD after approximately 3 weeks. She gradually regained renal function and dialysis was discontinued at approximately 5 weeks after admission.\nAt admission, she presented with seizures and decreased LOC, which improved after 7 days. Brain MRI demonstrated left frontoparietal and right paracentral subdural hemorrhages, with sulcal Fluid-attenuated inversion recovery (FLAIR) hyperintensity.\nThree weeks into admission, while still on overnight CRRT, oral baclofen was started for generalized spasticity, at a dose of 2.5 mg twice daily. Two days later, she was also treated with pregabalin 25 mg daily for neuropathic pain. Neurologically, at this stage, she was opening her eyes spontaneously and to command and followed simple directions.\nOn the fourth day of baclofen treatment, after receiving a total of 8 doses (20 mg) and 2 doses (50 mg) of pregabalin, she developed generalized decreased tone with upper airway obstruction, increased work of breathing, and decreased LOC with no response to painful stimuli. She required nasopharyngeal airway insertion to protect her airway. Blood pressure and heart rate were slightly increased (150/105 mm Hg, and 90-150 bpm, respectively).\nThis episode started 2 and 12 hours after the last dose of baclofen and pregabalin, respectively, while still on intermittent dialysis (serum creatinine 82 µmol/L, eGFR 86 mL/min/1.73 m2).\nBoth medications were discontinued, and within 10 hours, her level of alertness, respiratory, and hemodynamic status returned to her baseline.
Baclofen Toxicity in Children With Acute Kidney Injury: Case Reports and Review of the Literature
Your task is to provide an accurate and brief title of this case report
Our patient was an 83-year-old male with past medical history of hypertension,\nhyperlipidemia, atrial fibrillation, cerebrovascular accident, and end-stage renal\ndisease. He was on scheduled hemodialysis treatment through a catheter on his right\nchest. He was brought to the emergency department for fever and rigors of 1-day\nduration, with a recorded body temperature of 101.8°F. There was no cough, chest\npain, shortness of breath, abdominal pain, diarrhea, vomiting, headache, altered\nmental status, and pain or burning on urination. Physical examination and the\ninitial investigations failed to elucidate any obvious focus of infection. The\npatient was admitted for possible sepsis associated with infected dialysis catheter,\nand treated empirically with intravenous cefepime 1 g every 24 hours, intravenous\nvancomycin 15 mg/kg body weight every 24 hours, and intravenous metronidazole 500 mg\nevery 8 hours. The dose of vancomycin was altered as needed to maintain the\nvancomycin trough level between 15 µg/mL and 20 µg/mL. Two sets of initial blood\nculture and subsequent dialysis catheter tip culture grew Staphylococcus\naureus resistant to methicillin but sensitive to vancomycin,\ndaptomycin, rifampin, and tetracycline, while a transthoracic echocardiography\nrevealed right atrium mass, suggestive of endocarditis. The patient was therefore\ncontinued only on intravenous vancomycin for MRSA endocarditis.\nOn the 11th day of admission, the patient developed palpable purpura on his both\nlower limbs (). He\ndenied any pain or itching. While the erythrocyte sedimentation rate was elevated to\n45 mm/h (normal = 0-30 mm/h), rest of his immunological workup including c-ANCA,\np-ANCA, and atypical ANCA were negative, and C3, C4 levels were normal. Skin biopsy\nwas done, which revealed severe leukocytoclastic necrotizing small cell vasculitis\nconsistent with hypersensitivity vasculitis related to drug therapy (). So his vancomycin\nwas switched to daptomycin. The purpura started to resolve within 3 days of\ndiscontinuing vancomycin. Both his vasculitis and initial clinical condition\ncontinued to resolve during the rest of his hospitalization, and he was successfully\ndischarged after 22 days of hospital stay.
Vancomycin-Induced Leukocytoclastic Vasculitis: A Rare Case\nReport
Your job is to distill the important information into a title from this case report
A 48-year-old male patient with no previous medical history presented to our urology clinic with a penile lesion of 1-month duration. The lesion was a purple-color papule over the glans near the urethral meatus, measuring approximately 1cm. There were no symptoms other than painful erection. No palpable inguinal or iliac lymph nodes were found. No other skin or mucosa lesions were observed during the physical examination. Laboratory tests included normal cell blood count, urine analysis and urine culture. Serologic testing for HIV, hepatitis B virus (HBV), hepatitis C virus (HCV) and treponemal tests were all negative. The lesion was excised, and the histologic examination revealed proliferation and fascicles of spindle cells associated with angiogenesis ( and ). Expression of CD31 and CD34 was detected, and positive HHV-8 nuclear staining was identified, all compatible with KS ( and ). Since the patient had not developed additional lesions, conservative treatment was chosen.
Kaposi sarcoma of th e penis in anHIV-negative patient
Summarize this medical case report, ensuring to cover the initial presentation
Patient 2 was reported previously. Briefly, he is a 12-year old-boy of Tamil descent, born at term and was small for gestational age. He first presented at 11 weeks of age with severe episodes of bilateral pneumonia and continued to suffer from recurrent pneumonia and asthma thereafter. Upon assessment at the age of 5 months, his head circumference was 39.5 cm (below the third percentile for age). Weight was at the tenth percentile for age and length was below the third percentile for age. Developmental assessments, performed at 11 months and 5 years of age, indicated delays in expressive language, gross and fine motor skills, while social skills were appropriate for age. He was found to have characteristic dysmorphic features (Fig. ), spondyloepiphyseal dysplasia (Fig. ), as well as retinal dystrophy. A brain MRI revealed mild ventriculomegaly and prominent extra-axial cerebrospinal fluid spaces. An immunologic evaluation was significant for CD19 lymphopenia and hypogammaglobulinemia. He was unable to mount a long-standing response to tetanus and pneumococcal vaccines, and had non-reactive titres for measles, mumps, rubella and varicella, despite appropriate vaccination. He was therefore started on IVIG treatment at the age of 11 years. Similar to patient 1, the responses to T cells antigens were absent.
A homozygous mutation in the stem II domain of RNU4ATAC causes typical Roifman syndrome
You are tasked with extracting and summarizing the essential information from this case report
The patient was a 53-year-old woman with a past medical history of hypothyroidism and hyperlipidemia who presented to the emergency department (ED) with worsening bilateral upper and lower extremity weakness for three days. Two weeks prior to her presentation, she reported fever, diarrhea, nausea, vomiting, and pain on the right side of the abdomen. She had presented to the ED one week prior due to abdominal pain with a mild elevation in her liver enzymes. No acute pathology was discovered, and she was told to follow-up with her primary care physician (PCP). She then presented to an urgent care facility in which she was prescribed ciprofloxacin with eventual improvement in her diarrhea and vomiting. Three days prior to arrival, she began to note numbness and weakness of her fingers and toes. Her weakness was most noticeable in the distal aspects of her extremities, and she experienced some difficulty ambulating and a prominent decrease in her grip strength. This prompted her to schedule an appointment with her PCP. She followed up with her PCP, who was concerned for demyelinating disease. The PCP urged her to present to the ED for further evaluation. Despite not having any respiratory symptoms or fever, her PCP tested her for COVID-19 and influenza given her abdominal pain and diarrhea. Both tests returned negative.\nFurther history revealed the patient had reported a prior presentation to the ED roughly 10 months earlier for right upper quadrant abdominal pain. She was told she had elevated liver enzymes at that visit and was told to follow-up with her PCP. Her PCP had tested her for hepatitis C, which was negative, and she eventually had resolution of her clinical symptoms. She denied any recent travel, fevers, or shortness of breath. She had mostly been indoors for the past two months, without reported sick contacts.\nPhysical examination upon presentation to the ED was notable for 4/5 strength in the bilateral upper extremities with flexion and extension of the elbow and wrist. She had decreased grip strength bilaterally. Bilateral lower extremities had 3/5 strength with flexion and extension of the hip and knee as well as decreased dorsiflexion and plantar flexion of both feet. She had decreased sensation in her lower extremities with absent reflexes throughout. Initial labs were significant for a normal TSH, free T4, and a mild elevation of her liver enzymes: aspartate transaminase (AST) of 40 U/L (normal range: 8–48 U/L), alanine transaminase (ALT) of 46 U/L (normal range: 7–55 U/L), and alkaline phosphatase (ALP) of 138 U/L (normal range: 40–129 U/L). A hepatitis panel and computed tomography (CT) scan abdomen/pelvis were ordered to investigate her transaminitis. She was positive for hepatitis A IgG and IgM antibodies with no acute pathological process noted on the CT scan.\nInitial differential diagnosis included GBS versus a compressive myelopathy of the spine. Neurology was consulted with recommendations to perform a lumbar puncture and obtain further imaging of the brain and spine. A CT scan of the head was without acute abnormalities, and magnetic resonance imaging of the cervical, thoracic, and lumbar spine was negative for compressive myelopathy or acute abnormalities. A lumbar puncture was performed with an elevated total protein of 72 mg/dL (normal range: 15–60 mg/dL) and white blood cell count of 5 cells/mm3 (normal range: 0–8/mm3) suggesting albuminocytologic dissociation. The patient was diagnosed with GBS and started on intravenous immunoglobulin (IVIG) for five days. She had noticeable improvement in her distal extremity weakness following the first few doses of IVIG and was able to ambulate on her own by the end of her treatment. She was able to discharge following completion of her immunotherapy.
Guillain–Barré Syndrome Likely due to Relapsing Hepatitis A
As a clinical title specialist, your goal is to synthesize the key elements of this case report
A 63-year-old female patient presented intense left-sided otalgia, otorrhoea, retro-orbital pain, and diplopia initiated suddenly a week ago. No other neurological symptoms were referred. The patient denied similar previous symptoms and history of tubercular disease, reporting previous medical history of arterial hypertension and non–insulin-dependent diabetes mellitus with adequate glycemic control.\nAt admission, the patient was afebrile, showing gaze palsy with specific limitation of left eye's abduction movement. Vital signs were normal. The otoscopy revealed at the left side purulent otorrhea with hyperemic and swollen external ear canal, in addition to a posterior lesion in the external conduct suggestive of granuloma and intact tympanic membrane; right ear was normal. No clinical signs of acute mastoiditis were found. Clinical evaluation of the cranial nerves confirmed palsy of the left eye in outward gaze, suggesting dysfunction of the left abducens cranial nerve. The III, IV, VII, IX, X, and XI cranial nerve function was bilaterally preserved. An otorrhea sample was sent to culture.\nBlood analysis revealed WBC 9.190/mm3, CRP 74.60 mg/L, and glycated hemoglobin 6.4%. HIV test was negative. Audiometric evaluation showed left mild conductive hearing loss with pure tone average (PTA, calculated at 0.5, 1, 2, 4 kHz) of 13.75 dB HL in the right ear and 21.25 dB HL in the left side. Speech audiometry confirmed left hearing loss.\nHigh-resolution computed tomography (HR-CT) was performed, showing no evidence of stroke and an opacification of the left mastoid cells. ENT counseling was requested.\nLumbar puncture, performed on the third day of admission, ruled out infectious etiologies. CRP levels were 5.12 mg/L at the end of the first week of treatment. Culture result was available, on the 9th day of admission revealing Proteus mirabilis, Alcaligenis faecalis, Enterococcus faecalis, Citrobacter koseri, and Bordetella trematum.\nTechnetium scintigraphy was performed 2 months after hospitalization, showing a bone gap in the posterior wall of external auditory canal (Figure ) and hypercapture in the left temporal bone with extension to the sphenoid bone ipsilaterally (Figure ). A brain magnetic resonance imaging (brain MRI) was performed on the 8th day of admission (Figure ), revealing T1 isointensity of the left mastoid with partial substitution of the normal hyperintensity of the clival bone marrow due to inflammation. The left petrous apex was hyperintense in T2-weighted scans. After contrast administration, an enhancing rim delineated the presence of a 2 cm diameter abscess in the petrous apex extending to the dura mater and insinuating to the left pontocerebellar cistern, causing anteriorly the obliteration of Meckel's cave and involving the cavernous sinus with no signs of thrombosis. At this point, blood examinations revealed WBC 5.060/mm3 and CRP 6.55 mg/L. Neurosurgical team opted for a nonsurgical treatment due to surgery risks, the difficulty to access the abscess location, and the good outcomes the patient presented at the moment.\nOn the 33rd day of admission, an interval brain MRI was performed (Figure ) showing inflammatory process improvement in the petrous apex with signs of cavernous sinus thrombosis, so the patient was anticoagulated with enoxaparin. White blood cell count was 4.940/mm3, and CRP was 2.12 mg/L. On the 37th day, a new audiogram was executed showing a partial improvement of the left mild conductive hearing loss with PTA of 13.75 dB HL in the right ear and 18.75 dB HL in the left side.\nInterval technetium scintigraphy was performed prior to discharge (Figure ), on day 53, showing hypercapture in the left mastoid and petrous apex suggesting inflammatory process. At the discharge, blood examinations revealed WBC 4.740/mm3 and CRP 1.62 mg/L. Interval brain MRI was performed 8 days after discharge to evaluate response to treatment (Figure ), showing no evidence central sinus thrombosis, complete abscess resolution, no signs of insinuation to the left pontocerebellar cistern, and maintaining a partial obliteration of Meckel's cave.\nA weekly outpatient follow-up was made, and antibiotic therapy was suspended 1 month after discharge. Interval technetium scintigraphy to evaluate response to treatment was performed at the end of the treatment showing no evidence of active inflammatory process in the left temporal bone (Figure ). The patient started to show ocular motricity enhancement and diplopia improvement after discharge, with total left abducens nerve palsy recovery 4 months after the first symptom presentation.\nThe patient was hospitalized, and intravenous antibiotic therapy with combined scheme of ceftriaxone and ciprofloxacin was started with ceftriaxone 1 g twice a day associated with ciprofloxacin 500 mg twice a day. Ceftriaxone was changed to ceftazidime 1 g every 8 hours due to the lack of neurological infection evidence, as the local protocol to treat MEO recommends, in addition to topical antibiotic ear drops twice a day (combination of gentamicin and betamethasone)., , No corticosteroid was administered due to difficult in glycemic control.\nThe antibiotic regimen was readjusted on the 9th day due to evidence of abscess in the brain MRI and swab results to ampicillin 2G every 4 hours, metronidazole 500 mg every 8 hours, and ceftazidime 2 g every 8 hours. On the 27th day of admission, the topical antibiotic was changed to a combination of ciprofloxacin and hydrocortisone twice a day.\nThe patient was discharged after 54 days of hospitalization with a prescription of ciprofloxacin 500 mg twice a day associated with a topical ear drop combination of ciprofloxacin and hydrocortisone.
Conservative management of petrous apex abscess and Gradenigo's syndrome in a diabetic patient: Case report and literature review
Your task is to provide an accurate and brief title of this case report
A 47-year-old female patient was admitted to the hospital for dysphonia and dyspnea that had appeared about 2 years ago. With fiberoptic laryngoscopy, various submucosal tumefactions were observed, involving the supraglottic, glottic and subglottic regions, especially on the right. A biopsy was performed, and no tumor was found. Using endoscopy, with the CO2 laser, the lesions were removed. Histological examination highlighted the presence of extensive accumulations of amyloid substance even on the excision margins.\nNew symptomatic and large amyloid lesions were removed three times from both the vocal folds and at the level of the epiglottic pedicle. Finally, endoscopic CO2 laser treatment was performed in order to vaporize the edematous flap in the left arytenoid region and resect a synechia at the level of the anterior commissure 2 years after the first surgical procedures.\nThe patient remains with dysphonia and glottic insufficiency, but there was no evidence of recurrence.
Focus on localized laryngeal amyloidosis: management of five cases
Your objective is to distill this medical case report into a succinct title
We present a 45 year-old lady who had initially been seen by the respiratory physicians following an admission with acute dyspnoea following influenza infection 1-year prior to her surgery. She had undergone CT scan for abnormal chest radiograph. She had previously worked as a cleaner and was an ex-smoker of 1.5 pack years with no previous asbestos exposure or underlying genetic disease such as tuberous sclerosis or alpha-1 antitrypsin deficiency. She had been followed up in the nodule clinic (a follow-up clinic run by respiratory physicians to monitor solitary pulmonary nodules) with a provisional diagnosis of chronic obstructive pulmonary disease. She had progressive dyspnoea and occasional lower respiratory tract infections treated with courses of antibiotics. Her co-morbidities included previous alcohol dependence and depression. Pre-operative pulmonary function tests demonstrated an FEV1 of 1.53 (53% of predicted), FVC of 2.52 (79% of predicted), diffusing capacity of lungs for carbon monoxide (DLCO) of 5.25 (62% of predicted) and 6 minute walking distance of 414 m. Initial CT imaging findings demonstrated severe, heterogenous right-sided bullous emphysematous changes with a basal predominance and multiple large bullae in the lower lobe () and a benign inflammatory nodule, which was 7 mm in size in the right upper zone presumably right upper lobe. There was associated hyperinflation causing mediastinal shift to the left with complete right middle lobe collapse and marked compression of the right upper and middle lobes. Due to her increasing symptoms and progressive solitary nodule () in the right upper zone she was referred for a surgical opinion. Pre-operative echocardiography did not demonstrate any right ventricular dilatation or impairment and no pulmonary hypertension. She was counselled regarding the progressive symptoms, lung function and risk of pneumothoraces and scheduled for right thoracotomy and lower lobectomy. Operative findings were that of large bullous disease affecting the right lower lobe () with subtotal destruction of the lung parenchyma of the lower lobe. The nodule was present in the apical segment of the lower lobe instead of the upper lobe as expected. Right lower lobectomy was completed un-eventfully and the specimen was sent for histology. The upper and middle lobes were hypoplastic and the post lobectomy space management was a challenge. Post-operatively recovery was complicated by prolonged post-operative air leak managed conservatively and once this eventually settled, the patient was discharged following removal of the chest drain. Histopathological analysis of the right lower lobe specimen demonstrated several large thin-walled cysts with interstitial clusters of spindle cells bordering the cyst and immuno-histological markers (increased expression of VEGF-R3, Podoplanin, LYVE-1 and PROX-1) confirming a diagnosis of LAM. The patient was seen in the thoracic surgery follow-up clinic and a CT abdomen/pelvis was organized to rule out renal angiomyolipomas, associated with LAM in tuberous sclerosis. Referrals were made to the respiratory physicians and the National LAM centre for ongoing management.
Pulmonary lymphangioleiomyomatosis masquerading as unilateral heterogeneous emphysema
As a clinical title specialist, your goal is to synthesize the key elements of this case report
A 2-month-old female presented with 2 days of fever. The fever persisted for more than 5 days after admission. Generalized erythematous macular rash was followed. She was born with 40 weeks of gestational age and 2.7 kg of body weight without significant perinatal illness. She has been completely well without irritability, dyspnea, tachypnea, or cyanosis prior to illness. The height was 57.7 cm (10th–25th percentile) and body weight was 5.4 kg (10th–25th percentile). The blood pressure and the pulse rate were 84/60 mmHg and of 148 beats per minute respectively. The cardiac examination was unremarkable except for sinus tachycardia and breathing sounds was equal bilaterally. The laboratory results were as follows: white blood cell, 9,640/µL; hemoglobin, 9.3 g/dL; platelet, 571,000/µL, erythrocyte sedimentation rate, 61 mm/hr; C-reactive protein, 12.9 mg/dL; procalcitonin, 1.96 ng/mL; prohormone of brain natriuretic peptide, 2,077 pg/mL; creatinine phosphokinase, 19 IU/L; troponin I, 0.024 ng/mL. Electrocardiography showed sinus tachycardia without ST-T changes or abnormal Q wave (). The chest X-ray showed normal heart contour and size. Because of concern of Kawasaki disease and infective endocarditis for prolonged febrile exanthema, transthoracic echocardiogram was done. The origin of the RCA was from the main pulmonary artery with RCA to pulmonary artery steal, predominantly diastolic (). It showed a dilated left coronary artery with normal origin and collaterals from left anterior descending coronary artery to RCA on short axis view (). The cardiac structure, function, and dimension were within normal range but ischemic change of posterior wall of left ventricle was noted (). The fever was subsided on 4th hospital day and the causative infectious agent was concluded as rhinovirus. Coronary angiography was arranged for further evaluation. Aortogram demonstrated prominent left main coronary artery, normal left anterior descending artery and circumflex artery. The left main coronary artery arose from the appropriate sinus without aneurysm or stenosis (). After a slight delay, the posterior descending and RCA were filled up in a retrograde fashion through collaterals of the left coronary artery (). On the 11th hospital day, the patient underwent direct reimplantation of anomalous RCA to aortic root establishing dual ostial circulation (). On the last follow-up, 5 months after the surgery, she was perfectly well on acethyl salicylic acid therapy. The echocardiographic study showed the normal biventricular function. The forward flow was well seen from aorta to RCA.
Anomalous right coronary artery from pulmonary artery discovered incidentally in an asymptomatic young infant
Summarize this medical case report, ensuring to cover the initial presentation
A 29-year-old Hispanic woman presented to the emergency department with a 9-day history of intense holocranial headache associated with nausea and vomiting. She was discharged home with symptomatic treatment after complete resolution of symptoms and exclusion of all neurological causes. However, she subsequently developed horizontal diplopia and gait abnormalities and was readmitted to hospital because of abnormal eye movements and conjugate gaze palsy.\nPhysical examination was otherwise normal, but the mental status examination showed reduced attention. On cranial nerve examination, abduction was bilaterally limited with a compensatory abduction nystagmus. Pupils were normal. Dysmetria and dysdiadochokinesia were found on the left side of the body.\nIncreased signal intensity in the right pontomesencephalic junction was observed on T2-FLAIR and diffusion-weighted MRI sequences (). White matter hyperintensity was also found in the left cerebellar hemisphere; these lesions appeared hypointense on T1 and ADC map sequences, suggestive of infarction. MRA findings were consistent with dissection of the left V4 vertebral artery (). Antiplatelet therapy was initiated with subsequent follow-up in a vascular disease clinic.
Bilateral Internuclear Ophthalmoplegia in a Young Woman with Vertebral Artery Dissection
Your task is to provide an accurate and brief title of this case report
An 83-year-old woman with Alzheimer's disease was brought to the hospital after an overdose of medication. She had mistaken benzodiazepines for candies and taken a one-month supply of the medication. On admission, she was on memantine 20 mg once a day and quetiapine 25 mg twice a day. Her Mini-Mental State Examination (MMSE) was 3. She was admitted for mental status change and vomiting. In the ward, she refused the care especially taking a shower. The patient was agitated and aggressive to nurses with conventional care. When she was provided a shower, it took 300.5 seconds and the patient spent 25% of care time manifesting aggressive behavior. When multimodal comprehensive care—Humanitude—was applied, the duration was 315.6 seconds and no aggressive behavior was observed all through the care episode. In the comparison of percentage of the modalities during care, eye contact was 1.7% in the conventional care and 19.5% in Humanitude, verbal communication was 19.7% and 73.9%, and touch was 0.2% and 60.4%, respectively (). Because of the improvement of her symptom, the dosage of quetiapine was reduced to 25 mg once a day.
Reduction of Behavioral Psychological Symptoms of Dementia by Multimodal Comprehensive Care for Vulnerable Geriatric Patients in an Acute Care Hospital: A Case Series
Summarize this medical case report, ensuring to cover the initial presentation
An 8-year-old neutered male domestic shorthair cat was presented owing to acute-onset lethargy. Physical examination revealed marked dehydration, a grade III/VI left parasternal heart murmur and pain elicited during abdominal palpation. Complete blood count revealed a normal hematocrit (0.44 l/l; reference interval [RI] 0.28–0.49 l/l). Abnormal laboratory findings included a mild leukocytosis (15.6 × 103/μl; RI 4.2–13.0 × 103/μl) and a severe azotemia (urea 202 mg/dl; RI 17–34 mg/dl, creatinine 14.9 mg/dl; RI: 0.7–2.5 mg/dl), hyperphosphatemia (11.8 mg/dl; RI 2.5–7.1 mg/dl) and hypermagnesemia (4.6 mEq/l; RI 1.6–2.2 mEq/l). Urinalysis from a cystocentesis sample revealed a urine specific gravity of 1.015, a pH of 5.5, 1+ Multistix protein, 2+ blood, 3–5 red blood cells/high-power field, and occasional leukocytes and epithelial squamous cells. Abdominal ultrasound revealed a small misshapen left kidney with a large volume of echogenic fluid within the capsule, mild right renomegaly with a mild-to-moderate amount of intracapsular fluid, hyperechoic tissues surrounding the right kidney and a mild volume of peritoneal effusion.\nPrior to performing renal aspirates, coagulation times were measured and prothrombin time (PT) was 16 s (RI 15–22 s) but partial thromboplastin time (PTT) was >500 s (RI 65–119 s). Additional citrated blood was collected from a jugular venepuncture directly into a syringe and transferred to a 3.2% citrate tube to measure FXII activity and perform a kaolin- and TF-activated TEG. FXII levels were measured using a coagulometric assay with human FXII deficient plasma and a standard curve generated with lyophilized pooled cat plasma. FXII activity was 15% (RI 60–140%), consistent with a FXII deficiency. Kaolin-activated TEG exhibited delayed clot formation characterized by markedly prolonged reaction (R) and kappa (κ) times, and a decreased angle compared with normal (; and ), whereas the TF-activated TEG revealed a normal to mildly hypercoagulable tracing characterized by a mildly increased maximum amplitude (; ).\nFluid was drained percutaneously from beneath both renal capsules, and aspirates of the right kidney were performed without evidence of hemorrhage. The next day, an ultrasound examination confirmed re-accumulation of the subcapsular fluid bilaterally and the cat was humanely euthanized. A post-mortem examination revealed bilateral perinephric pseudocysts, pyelectasia with concretions, renal fibrosis with cortical hemorrhage, and partial obstruction of the ureters with proximal ureteral dilation, as well as bilateral parathyroid enlargement.\nA 7-year-old spayed female domestic shorthair cat was presented for inclusion in the study as a healthy control. The cat was apparently healthy at home and physical examination was unremarkable. Complete blood count revealed normal parameters, including hematocrit (0.35 l/l). Serum biochemistry profile and PT results were within the RIs; however, the PTT was markedly prolonged (173 s; RI 24.2–34.9 s). FXII deficiency was suspected; further hemostatic testing was performed. Blood was collected from a jugular venepuncture directly into a syringe and transferred to a 3.2% citrate tube The kaolin-activated TEG exhibited delayed clot formation and decreased clot strength, characterized by markedly prolonged R and κ times, and decreased angle and maximum amplitude (MA) (; ), whereas the TF-activated TEG was normal (; ). FXII activity was 7%, consistent with a FXII deficiency.
Evaluation of thromboelastography in two factor XII-deficient cats
You are to provide a brief yet comprehensive title of this case report
A 62-year-old man (169 cm in height and 69 kg in weight) was admitted to hospital with dizziness and paresthesia of both hands. He was diagnosed with cerebral infarction, and while doing additional examination, cerebral infarction was assumed to be due to infective endocarditis. Transthoracic echocardiography showed large and hypermobile vegetations on anterior mitral leaflet. He was scheduled to have mitral valve replacement using MICS due to tremendous risk of systemic embolism.\nTwo months back, he was admitted to the Department of Neurosurgery in our hospital because of subarachnoid hemorrhage in left central sulcus and precentral sulcus, and blood spots on the lower limbs and thrombocytopenia (33,000 /mm3) were incidentally detected. Although in a bone marrow aspiration, pathologic finding was not observed and the platelet associated immunoglobulins was negative, ITP was clinically diagnosed. However, steroid therapy was not started immediately and his platelet count was examined as needed.\nHis platelet count was 40,000 /mm3 the day before surgery. Because MICS needed the use of heparin prior to cardiopulmonary bypass (CPB), we decided to transfuse platelets after surgery. For premedication, 0.2 mg of glycopyrrolate was injected intramuscularly 30 minutes before the surgery. Vital signs at the time of arrival at the operation room were blood pressure at 125/75 mmHg, heart rate at 98 beats/min, and oxygen saturation (SpO2) as 99%.\nFor the induction of anesthesia, while 0.1 µg/kg/min of remifentanil was infused for three minutes, 10 mg of etomidate and 10 mg of cisatracurium were also injected. Then a left-sided 35-french DLT was intubated with a stylet. The stylet was removed immediately after the tip of DLT passed through the vocal cords. When the tube was inserted at a depth of 29 cm to lips, resistance was felt. Tracheal cuff and the bronchial cuff were inflated with 6 ml and 2 ml of air, respectively. After making a judgment that the location of tube was appropriate by using bronchoscope, the bronchial cuff was deflated in order to prevent bronchial injury from postural changes, and there was no bleeding. Anesthesia was maintained with sevoflurane 1.5 vol%, oxygen 1.0 L/min, medical air 1.5 L/min and remifentanil 0.12 µg/kg/min were continuously infused intravenously. The patient underwent DLT change when we detected leakage of endotracheal balloon. After the exchange of DLT using tube exchange catheter, we confirmed the location of the DLT by using bronchoscope, and there was no bleeding. Insertion of superior vena cava (SVC) drainage catheter and central venous pressure catheter were performed via right internal jugular vein. Heparin 2,100 IU was administered to prevent clot formation of SVC drainage catheter. There was no sign of bleeding during those procedures. At the end of the procedure, we detected bleeding from the left side of DLT. The appearance of the bleeding was blood-tinged or frothy pink like pulmonary edema. The bleeding was sucked via suction catheter and the amount of the bleeding was about 150-200 ml. Due to the bleeding via the left side of DLT, we carried out lung separation immediately and decided to check the location of the bleeding with a fiberoptic bronchoscope. The carinal region and the left and the right bronchus were examined with a fiberoptic bronchoscope, but the focus of the bleeding in the traveling direction from the left mainstem bronchus was not detected. Because fiberoptic bronchoscopic view had poor visibility due to blood, we could not detect the focus of the bleeding and active bleeding from bronchus. Also, there was no bleeding via right side of DLT. Because we could not find the evidence of tracheobronchial laceration or rupture and the patient did not have any subcutaneous emphysema, and pneumomediastium, we excluded the possibility of mechanical injury of airway.\nBlood loss was estimated to be 150-200 ml over 30 min. Therefore, we suspected bronchial arterial injury; MICS was immediately stopped and bronchial artery embolization was scheduled in the angiographic room. Vital signs at that time, were blood pressure (BP) at 105/50 mmHg, heart rate (HR) at 115 beats/min, and SpO2 89%. During the transportation of the patient to the angiographic room, gradual decrease in the bleeding was noted, and the patient's vital signs in the angiographic room were stable (BP at 110-120/50-80 mmHg, HR 100 beats/min, and SpO2 99-100%). Angiography of the bronchial artery was performed, but focus of the bleeding was not found (). With reference to the risk of re-bleeding from the injured bronchial artery and the possibility of uncontrolled bleeding in patient with ITP, we decided to embolize the contributing bronchial arteries. After finishing the embolization of bilateral bronchial arteries, the chest X-ray was checked, and total atelectasis of left lung was observed (). There was no evidence of active bleeding, but blood clot was detected and removed using bronchoscope. After the removal of blood clot, an improvement in lung collapse was noted.\nThe patient was scheduled for MICS 6 days later. Due to lowered platelet count (54,000 /mm3), a high-dose IVIG therapy (35 g/day, 0.5 g/kg) for 2 days was started. Platelet count was increased to 87,000 /mm3 after 6 days. We intubated with single lumen endotracheal tube and lung isolation was achieved with intermittent total lung deflation. The surgery was done safely, and the outcome was favorable for the patient without any postsurgical decrease in platelet count or bleeding. He was extubated on the postoperative day (POD) 2, and transferred from the intensive care unit to the ward on POD 5. The patient left the hospital after making a full recovery on POD 28.
Endobronchial hemorrhage after intubation with double-lumen endotracheal tube in a patient with idiopathic thrombocytopenic purpura for minimally invasive cardiac surgery: a case report
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A 28-year-old woman was referred for a gait disturbance with reduced range of motion (ROM) of bilateral hip joints and bilateral hip pain. At the age of 2 years, she had been diagnosed as having bilateral hearing loss and had undergone surgery to correct a cleft palate. From the age of 20 years, she had noticed stiffness of the hip joints and difficulty in walking. At the age of 23 years, clinical geneticists had diagnosed her as having OSMED and identified a COL2A1 mutation relating to type II collagen.\nAt the time of the referral, although she could walk for a few minutes using 2 Lofstrand crutches, she used a wheelchair in her daily life. Her height was 155 cm (−0.1 SD), and her weight was 47 kg (−0.8 SD). Physical examination revealed significantly reduced ROM in the limb joints and spine; ROM of both hip joints showed 60° of flexion contracture, 80° of flexion, 0° of abduction, and 10° of adduction. The Harris Hip Score (HHS) [] was 19 on the right and 20 on the left. Radiographs showed multiple changes of the skeleton, including end-stage osteoarthritis of the hip joints () and knee joints and hyperlordosis of the lumbar spine. The computed tomography (CT) images of the hip joints revealed significant retroversion of the acetabulum (). A three-dimensional motion analysis system (VICON MX, SGI, Tokyo, Japan) was used to analyze her standing up and her gait; fixed anterior tilting of the pelvis and limited mobility of the lumbar spine were demonstrated. Furthermore, it also revealed that her center of gravity deviated anteriorly because she could not compensate for severe flexion contractures of bilateral hip joints due to limited mobility of the lumbar spine. Therefore, it was possible to identify that hip dysfunction had a major impact on gait disturbance ().\nThus, bilateral THA was considered the treatment of choice. For planning, full-scale three-dimensional plaster models of the pelvis and femora were produced with an inkjet printer method using CT data (Next21 K.K., Tokyo, Japan). The acetabular cavity was aspherical, and the wall was thin even in the safe zone [] (). To fill the vault, the diameter of the acetabular component needs to be more than 66 millimeters. When a 68-millimeter diameter hemisphere was set at 0° of anteversion, its posterior part projected backward from the acetabulum, and its anterior part was deep in the bony wall (). The goal was to gain good ROM without impingement. Thus, the plan was to fix a 68- or 70-millimeter diameter acetabular shell at 40° of abduction and 0° of anteversion in terms of the anatomical measurements [], remove a significant amount of the bone from the anterior and anteroinferior parts of the acetabulum, use a 44-millimeter head, and fix the stem at 30° of anteversion (). It was also considered vital to avoid reaming too deeply to avoid penetration of the acetabular wall.\nThe patient underwent right uncemented THA through a posterolateral approach. The acetabular component was a 68 mm, hemispherical shell (Trident, Stryker Orthopaedics, Mahwah, NJ, USA) with a highly cross-linked polyethylene liner (X3, Stryker Orthopaedics) and two transfixing screws. The femoral component was a tapered wedge stem with a neck shaft angle of 127° (Accolade TMZF, Stryker Orthopaedics) with a 44 mm (+4) modular head. These components were fixed at the same angle as at preoperative planning. Passive motion was possible from 30° to 90° in the sagittal plane.\nTwo months later, she underwent left THA through a posterolateral approach. The acetabular component was a 68 mm Trident shell with an X3 liner and 2 transfixing screws. The femoral component was an Accolade TMZF stem with a neck shaft angle of 132° with a 44 mm (+0) modular head. This time, the shell was placed at 35° of abduction and 10° of anteversion. Passive motion was possible from 30° to 100° in the sagittal plane. Although the surgery was uncomplicated, migration of the shell was found 2 weeks later. Thus, the unstable shell was removed, and a 70 mm Trident shell was placed with 6 transfixing screws at 45° of abduction and 10° of anteversion.\nAt 1.5-year follow-up, the patient had no hip pain and could walk without walking aids for about 10 minutes. Even though the flexion contracture had remained in both hip joints after surgery, ROM improved with active exercise. Thus, passive ROM of the right hip showed 100° of flexion with no flexion contracture and 20° of abduction and that of the left hip showed 80° of flexion with no flexion contracture and 10° of abduction. The HHS was 74 on the right and 73 on the left. Radiographs demonstrated stable components (). Three-dimensional motion analysis demonstrated that her center of gravity had shifted posteriorly because of the improvement of the flexion contractures of bilateral hip joints and that her gait pattern improved significantly ().
Total Hip Arthroplasty in a Patient with Oto-Spondylo-Megaepiphyseal Dysplasia Planned by Three-Dimensional Motion Analyses and Full-Scale Three-Dimensional Plaster Model of Bones
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A 52-year-old woman was seen in the outpatient orthopedics clinic for evaluation of right upper extremity swelling and pain of 3-week duration (pain score of six at its worst), which initially extended from her right shoulder down to the fingers, and resolved without treatment, albeit with residual pain at presentation. The patient reported subjective fevers that occurred at the onset of swelling and spontaneously resolved within 24 h. Physical examination of the right shoulder revealed full active and passive range of motion with mild tenderness over the greater tuberosity of the right humerus. Laboratory tests including complete blood count with differential, and serum protein electrophoresis were unremarkable except for elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Magnetic resonance imaging (MRI) of the right shoulder demonstrated a nonspecific intramedullary mass in the greater tuberosity of the proximal humerus, measuring 3.2 × 2.1 × 2.8 cm (Fig. A and B). Unenhanced computed tomography (CT) showed an osteosclerotic intramedullary mass, with a corresponding SUV of 7.4 on 18-fluorodeoxyglucose positron emission tomography (PET) (Fig. C and D).\nCore needle biopsy and cultures of the right proximal humerus lesion were performed (Fig. A and B). The aspirate demonstrated numerous atypical promyelocytes with convoluted nuclear contours and abundant cytoplasm with large granules. A few cells with numerous Auer rods were also present. Flow cytometry revealed that these promyelocytes expressed CD33, CD117, CD71, cytoplasmic MPO and CD34 (partial), but were negative for HLA-DR, CD11c, and CD11b. Fluorescence In situ-Hybridization (FISH) was promptly performed on the aspirate, and identified a t(15;17) translocation with fusion of PML/RARA in 79% of interphase cells.\nA bone marrow biopsy and aspirate from the patient's right iliac crest were subsequently performed to evaluate for systemic disease (Fig. C and D). Interestingly, neither the peripheral blood nor the bone marrow of the iliac crest showed morphologic or cytogenetic evidence of promyelocytic leukemia.\nFISH studies were negative for t(15;17), and RT-PCR was negative for the PML/RARA fusion in both the peripheral blood and bone marrow specimens. These findings confirmed that the patient had promyelocytic sarcoma involving the right humerus but without the typical leukemic presentation of APL.\nAdditional CT scans performed for staging revealed abnormally increased metabolic activity in the sternum and manubrium, acromioclavicular joints, left acromium, right humeral head with mild increased osseous density, bilateral humeral diaphysis, bilateral proximal ulnar and radial heads, bilateral distal femurs, right distal femoral metaphysis, and bilateral proximal tibias (SUVs ranging from 7.4 to 27.4). No other abnormalities were noted.\nThe patient was started on treatment as per Lo-Coco et al. with all-trans retinoic acid (ATRA) and arsenic trioxide which she tolerated well without any complications . The overall clinical course from the time of onset of pain to initiation of differentiating agents was 4 weeks. Disease response was monitored by PET/CT evaluation which was repeated at day 30 of treatment and showed incomplete response, with persistent asymmetric increased metabolic activity in the bone marrow of the distal right femur and proximal right tibia, compatible with residual leukemia. The previously described diffuse metabolic activity in the remainder of the osseous and bone marrow structures including the right humerus (Fig. F), sternum, manubrium, and acromioclavicular joints was no longer present. After completing induction, treatment was held for 2 weeks. Patient was then continued onto consolidation therapy with ATRA and arsenic, consisting of four 8-week cycles for a total 28 weeks of consolidation, which she tolerated well without complications. Our patient exhibited an excellent treatment response, with PET/CT scan after completion of consolidation therapy showing a complete resolution of all previously seen abnormal metabolic activity, with no new metabolically active lesions. She is planned to proceed with maintenance therapy with 2 weeks of ATRA alone given every 3 months for the next year.
Promyelocytic sarcoma of the right humerus: an unusual clinical presentation with unique diagnostic and treatment considerations
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An 84-year-old Japanese man was admitted to a hospital's emergency medical center with a complaint of stomach ache. The patient was diagnosed with acute aortic dissociation (Stanford type A) and treated in the ICU. An analgesic effect of pentazocine was observed. However, he had sleep disturbance in the night, and the patient's topic of conversation was inappropriate. Therefore, he was referred to the hospital's department of psychiatry. There, he was disoriented and agitated. To treat his delirium, he was administered fluvoxamine (50 mg, twice a day) and flunitrazepam (1 mg, at night). At 1 day after treatment, his sleep disturbance improved, and his Delirium Rating Scale (DRS) [] score decreased dramatically from 16/32 to 6/32. After ICU discharge, his condition was good. His Mini-Mental State Examination (MMSE) [] score was 25/30.
Sigma-1 receptor agonist fluvoxamine for delirium in intensive care units: report of five cases
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A 75-year-old female patient presented at the surgical consultation for perianal hitching and bleeding. The patient's complaints started 12 months prior, with progressive worsening. Her past medical history included hypothyroidism, glaucoma, and hemorrhoidectomy 30 years previously. Her family medical history included hepatic cirrhosis (father) and coronary artery disease (mother) but no cancer. Physical examination showed a 3 cm plaque-like eczematous lesion, circumferentially involving the perianal skin and extending to the anal verge (). A biopsy of the perianal mass demonstrated a well-differentiated mucinous adenocarcinoma. Serum carcinoembryonic antigen level and other laboratory tests were within normal range. A pelvic MRI showed that the lesion had invaded the skin tissue without involvement of perirectal structures and anal sphincters (). An endoanal ultrasound confirmed the absence of invasion into the anal canal and anal sphincters. A colonoscopy showed two polyps in the ascending colon that were both removed and histologically examined; they were both tubular-villous adenomas with low-grade dysplasia. A total-body CT scan did not show any associated visceral malignancy or lymphadenopathy. The patient was discussed within a multidisciplinary team including surgeons, oncologists, radiotherapists, radiologists, and pathologists. Since no anal canal involvement was identified and because of the patient's refusal of a permanent colostomy, a wide local excision was planned with a flap to close the perianal defect, and a protective colostomy. A biopsy of the inguinal sentinel node was also scheduled to rule out inguinal lymph node metastasis.\nThe day before the operation, a lymphoscintigraphy was performed which showed uptake in the right inguinal region. The patient underwent surgery the following day under general anesthesia. A sentinel inguinal lymph node was retrieved at the right groin region. A laparoscopic lateral colostomy was performed on the sigmoid colon. The patient was then placed in a jackknife position. The neoplasia was resected with 1 cm of free margins over the skin and the anal verge, including the dermis and the subcutaneous tissue. Once the excision was completed, multiple biopsies inside the anal canal were taken to exclude invasion. Then, stiches were placed at the four cardinal points on the mucosa of the anal canal. A double V marking was drawn, and the flaps were dissected with vascular pedicle preservation, based on random inferior gluteal perforator vessels and peripheral skin undermining (). The flaps were extended medially until complete coverage of the defect was attained and were then sutured tension free to the mucosa of the anal canal. The subcutaneous tissue was approximated, and the dermis was closed with separate sutures of reabsorbable material.\nThe pathological report was mucinous adenocarcinoma of the perianal skin involving the subcutaneous tissue with pagetoid spread with negative resection margins (). The lesion presented the following immunohistochemical pattern: CK7+, CK20+, CDX2+, CEA+, and GCFDP15-, suggestive of secondary Paget's disease (Figures –). No metastasis on the inguinal sentinel node was demonstrated. The postoperative course was uneventful, and the patient was instructed to avoid recumbent or sitting position for the first 4 days and was discharged on postoperative day 12.\nAfter 3 months (), random biopsies were taken in the flaps, in the skin proximal to the flaps, and in the anal canal, and the histopathological examination showed no sign of disease. The colostomy was then closed 2 months later. She was followed up at 3-month intervals; at the last follow-up, 14 months after surgery, the patient was fully continent and did not show any sign of recurrence.
Local Excision of a Mucinous Adenocarcinoma of the Anal Margin (Extramammary Paget's Disease) and Reconstruction with a Bilateral V-Y Flap
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A 29-year-old woman was admitted to our hospital with a fever of up to 38.5°C, body aches, sore throat, and a slight cough for 2 days. Twenty days before the current admission, she delivered a healthy baby at 35.6 weeks of gestation by cesarean section. On physical examination, temperature was 39.8°C, radial pulse 104, respiratory rate 18, and blood pressure 104/68 mmHg. Blood test showed white blood cell count 9.3 × 109/L, with 78.3% neutrophil. She was given oseltamivir and paracetamol. On day 3, after the onset of fever, she developed convulsions and an altered mental state. Neurological examination revealed signs of dysarthria, nystagmus, bilateral extremities, and trunk ataxia, with negative meningeal signs. On day 4, brain MRI showed well-defined lesions of hypointensity in T1-weighted and hyperintensity in T2-weighted imaging without enhancement, and hyperintense in diffusion-weight imaging (DWI) with low apparent diffusion coefficient (ADC) value of bilateral middle peduncle and SCC (Figure A–H). MRA/MRV was normal. CSF revealed a WBC count of 29 cells/mm3. Gram staining of CSF was negative. Antibodies to EB, CMV, and VDRL were negative. EEG showed diffused slow waves and focal spike waves. On day 6, she complained of recurrent thunderclap headache, and blood pressure of 150/90 mmHg was noted. Dexamethasone IV 10 mg Q12H was added with the tentative diagnosis of AC. After 5 days of treatment, she was free of fever, headache, seizures, and confusions, but she could not walk without help and had prominent dysarthria. A follow-up brain MRI revealed increased lesion in middle peduncle with lower intensity in SCC compared with 6 days prior (Figure I–J); hyperintensity of bilateral hemicerebellum was noted in T2-weighted sequences (Figure M–P); no enhancement in these lesions; dilated lateral and third ventricles; and hyperintense periventricular white matter changes (Figure ). On day 20, CSF test revealed no significant finding. On day 45, she could walk without help; brain MRI showed complete recovery of SCC lesion and attenuated lesions in cerebellum and middle peduncle.
Lesions more than cerebellum in a puerpera of acute cerebellitis: concurrent encephalitis, not a subtype of ADEM
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A 19-year-old male patient presented with slow-growing asymptomatic skin-colored nodules with punctum on the right temple, chest, back, and gluteal regions for five years. He was not aware of the mass on the back and gluteal regions until he experienced a vague sensation originating from the back and gluteal regions when lying and sitting down. The lesions on the right temple and chest first appeared as skin-colored papules which slowly multiplied and increased in size. The patient had not sought treatment for the lesions. He has history of acne vulgaris since adolescence. There was no previous history of similar complaint in the patient nor his family. He did not experience pain or other complications of skin lesions, such as secondary infection, inflammation, and suppuration. There was no history of malignancy in the patient nor his family. He did not experience hidradenitis suppurativa, pilonidal sinus disease, or any syndrome. There was no history of consanguineous marriage in the mother’s family nor his father’s family.\nClinical examination of the lesions revealed painless skin-colored nodules on the right temple, chest, back, and gluteal regions with a central black punctum (). The size varied from 0.3 x 0.2 x 0.1 cm to 6 x 5 x 3 cm. There was no definite distortion of the surface anatomy from visual examination nor a non-tender firm-to-hard mass fixed to the underlying structures on palpation. Comedones were identified on the face, chest, and back, whereas scars were identified on the face, lower back, and gluteal regions. Histopathological examination of the three skin-colored nodules on the back revealed cysts containing keratin lined by wall composed of stratified squamous epithelia and the nuclei of epithelial cells were within normal limit (). This histopathological finding supported the diagnosis of EC. Therefore, the diagnosis of multiple ECs with a giant EC was established. However, further investigation with genetic analysis to rule out possible genetic causes were not possible due to the unavailability of a genetic laboratory in our center.\nSurgical excision was performed for the mass on the back (on the seventh day) (). Extirpation of cysts with conventional wide excision was performed for the mass on the upper left gluteal region (on the 48th day), chest (on the 55th day), and lower left gluteal region (on the 61st day) (–) to produce less scarring for optimal cosmetic results. He also received 0.2 mL of triamcinolone acetonide 10 mg/mL intralesional injection for the skin-colored nodule on the right temple (on the 58th day) to achieve optimal cosmetic results. Two weeks after the interventions, the wound healed well and there were no signs of infections. After four months of follow-up, there was no recurrence and all skin-colored nodules were completely removed.
An Unusual and Rare Case of Generalized Multiple Epidermoid Cysts with a Giant Epidermoid Cyst
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A 26-year-old-woman, with history of labial herpes and asthmatic bronchitis, presented with bilateral retroocular pain, odynophagia, fever, vaginal discomfort and vulvar ulcers. The ulcers continued to progress despite treatment with nonsteroidal anti-inflammatory drugs and valacyclovir, prescribed at the primary healthcare site. Two days later, after initial consultation at our emergency room, she was admitted immediately at the Vulvar Clinic of our institution, with increasing vulvar pain, without other symptoms. Physical examination of the vulva showed extended vulvar oedema and kissing pattern ulcers on labia minora and majora, vagina and cervix (). Inguinal lymph nodes were also bilaterally swollen. The patient denied the use of other medications and sexual activity in more than 6 months. First blood tests only showed C-reactive protein 12.35 mg/dL (normal: <0.5 mg/dL). Serologies for herpes virus 1 and 2, Ebstein-Barr virus (EBV; IgG+), cytomegalovirus, mycoplasma pneumoniae, parvovirus B19, toxoplasmosis, rubella, hepatitis, human immunodeficiency virus, and syphilis (using the Venereal Disease Research Laboratory test) were negative. After this, a multidisciplinary approach was performed. When directly asked, she complained about mild eye dryness and she often felt a discomfort of dry mouth. There was no familial history of autoimmune diseases, but her father had some episodes of oral aphthosis. Immunological examination was positive for rheumatoid factor (RF – 22.3 UI; normal: <15 UI), anti-nuclear antibodies [ANA (speckled, titer 1:320)] and antibodies to SSA/Ro (SSA 3+/ Ro52KD 3+)—initial screening step of ANA by indirect immunofluorescence on HEp-2 cells (Euroimmun ®, Germany); autoantibodies confirmation assay by line immunoblot (ANA profile 3 - Euroimmun ®, Germany)—antibodies detected on strips were evaluated semiquantitatively (negative, 1+, 2++, and 3+++). Anti-SSB/La, anti-RNP, anti-Sm, anti-dsDNA, antineutrophil cytoplasmic, anticardiolipin, and anti-beta(2)-glycoprotein1 antibodies were negative. Serum C3 level was 1.79 g/L (normal: 0.9-1.8 g/L) and C4 level was 0.31 g/L (0.1-0.4 g/L). Immunoglobulins (IgG, IgM, IgA) were measured and a high IgG level was found (20.50 g /L; normal: 7-16 g/L). Erythrocyte sedimentation rate was also high (45 mm/h; normal: <16 mm/h). Lupus anticoagulant and HLA-B27 were both negative. Ophthalmological evaluation was refused by the patient. Labial salivary gland (LSG) biopsy revealed focal lymphocytic sialadenitis (FLS), with a focus score (FS) =1 (per 4 mm2) obtained by four LSGs (3-5 mm). No other histopathological features were reported. The patient was diagnosed as having pSS on the basis of dry eyes and dry mouth, positive anti-SSA/Ro antibody, and typical histopathologic abnormalities on LSG biopsy. Prednisolone 20 mg/day was prescribed and vulvar healing appeared within 2 weeks with partial loss of left labia ().
Lipschütz Genital Ulceration as Initial Manifestation of Primary Sjögren's Syndrome
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Second case was a 53-year-old female (height 165 cm, weight 85 kg) without relevant past medical history suffering from dyspnea caused by a giant retroperitoneal liposarcomas that progressed over 2 years after invalid conservative treatment. A hard mass occupying the entire abdomen was palpated on physical examination and the patient had cachexia. Abdominal CT revealed a PAR of 0.68 (Fig. ) and the ratio of maximum anteroposterior to transverse abdominal diameter was 1.21 (Fig. ). Preoperative biopsy showed a mesenchymal tumor. Laboratory tests showed anemia and severe hypoproteinemia. Left chamber enlargement and impaired left ventricular relaxation were showed by echocardiography. The patient was planned for en bloc resection surgery under general anesthesia without TAP block on August 12, 2019. Arterial blood gas analysis showed arterial partial pressure of carbon dioxide of 40.8 mm Hg and arterial partial pressure of oxygen of 50.0 mm Hg in room air. With the FloTract/VigileoTM system, the invasive blood pressure was 150/80 mm Hg. In consideration of the stable hemodynamic parameters and our experience from the previous case, the same general anesthesia protocol was implemented as described in the first case. After the anesthesia induction, the patient was ventilated in a volume-controlled mode. As the tumor weight was estimated around 40 kg, the TV was set to 450 mL, with respiratory rate of 16 beats/min, PEEP of 5 cmH2O and airway pressure of 30 cmH2O. A FiO2 of 0.8 showed a PaCO2 of 44.7 mm Hg, PaO2 of 68.9 mm Hg and the CVP was measured as 25 cmH2O. During surgery, a midline xyphoid to pubic incision was performed, and the airway pressure decreased gradually therewith. To obtain a sufficient arterial oxygenation, we couldn’t decrease the FiO2 until the tumor was excised partly 3 hours later. A typical sandwich structure was formed by the intestinal canal and the mass (Fig. ). As the tumor encased the entire right kidney and had invaded the small intestine, a right nephrectomy and combined resection was performed, and there was massive blood loss during the whole surgery. Her total blood loss was 12800 mL and 64 units of red cell concentrate, 4800 mL of fresh frozen plasma, 40 units of cryoprecipitate and 3 units of platelet concentrate were transfused. Preoperative hemoglobin concentration was 106 g/L after blood transfusion, while it was only 82.0 g/L. The tumor was removed mainly, weighed 35 kg (Fig. ), and was identified by pathological examination as dedifferentiated liposarcoma. On completion of the surgery she was transferred to the ICU with intubation. She was extubated on POD 3 and discharged from the ICU on POD 6. She underwent another surgery, because of anastomotic leakage resulting from bowel resection on POD 10.
Successful intraoperative management in patients with abdominal compartment syndrome induced by giant liposarcomas
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A 49-year-old Chinese man, who had a 2-month history of SLE and treated with oral prednisone (30 mg/day), was admitted to a local hospital in February 2017 due to repeated fever, cough and fatigue. He was a farmer living in Guangxi. During hospitalization, Gram-negative bacteria were isolated from his blood culture and antibiotic (meropenem) for septicemia administered during a period of 3 weeks without improvement. He was then referred to our hospital for further treatment.\nUpon admission to our hospital, physical examination showed annular erythema of the inner canthus and eyelids, a mouth ulcer and massive ecchymosis of the forearms. Routine blood examination revealed the following: white blood cells, 3.65 × 109/L with 81.4% neutrophils; hemoglobin, 85.80 g/L; and platelets, 649.20 × 109/L. The c-reactive protein (CRP) level (38.99 mg/L) and erythrocyte sedimentation rate (>140 mm/hour) were elevated. The 24-hour urine protein level was 2655.0 mg, anti-dsDNA antibody titers were normal, and serum complements were low (C3, 0.519 g/l; C4, 0.125 g/l). Assessment for anti-nuclear antibody and anti-SS-A indicated positivity while anti-HIV antibody was not detected. The liver functions (alanine aminotransferase, 144 U/L; aspartate aminotransferase 677 U/L) were deranged, and blood coagulation (thrombin time >120 s, fibrinogen 0.69 g/l) was impaired. The CD4+ T-lymphocyte count (95 cells/µL) was lower than normal. A sputum smear contained hyphae. Chest computed tomography (CT) scan revealed infiltration in the upper lobes of the lungs, swollen mediastinal lymph nodes, and a slight pleural effusion. A diagnosis of active SLE with lupus nephritis, pneumonia, and septicemia was established.\nHe received intravenous methylprednisolone (80 mg/day), empirical intravenous antibacterial (meropenem 1gq8h), and antifungal (fluconazole 200 mg/day). His temperature subsequently returned to normal. However, his platelet count progressively declined to 42.4 × 109/L and he developed hemoptysis. Nine days post-admission, chest CT showed increased infiltration and pleural effusion. Finally, the sputum and bone marrow cultures were positive for T. marneffei.\nMethylprednisolone was changed to prednisone 50 mg/day. Intravenous amphotericin B (5 mg/day gradually increased to 25 mg/day) and oral voriconazole (400 mg/day) were administered for 2 weeks, followed by oral itraconazole (400 mg/day) for 11 months. His condition improved and there was no recurrence of T. marneffei infection at the 3-year follow-up.
Talaromyces marneffei Infection in Systemic Lupus Erythematosus Patients: Report of Two Cases and Review of the Literature
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A 27-year-old man (75 kg) presented to our emergency department and was monitored by a cardiologist. Dissection occurred following weightlifting across his chest and shoulders. He described multiple episodes of faintness over the preceding hours. These were associated with sweating, dyspnea, and temporal right eye vision deficit. The first episode began during heavy weight lifting (180 kg) and lasted 10 minutes before resolving with rest and drinking fluids. The second episode began while lifting weights again, which was more intense in severity and lasted for 20 minutes. The third fainting episode occurred when he left the gym, whereby he fell over the floor outside the club. The patient was a coach and a professional bodybuilder. During the first lifting, he remembered weight training heavier than his normal regimen involving squatting with weights of around 140 kg in the supine position across the anterior chest wall. He initially denied taking illicit drugs but, upon insistence, he confessed that he took intramuscular 250 mg testosterone twice per week and human growth hormone 20 mg/day for two years. In the emergency room, the patient’s vital signs were a blood pressure of 90/60 mmHg, heart rate of 120 beats/min, respiratory rate of 26 breaths/min, and temperature of 36 oC. Blood chemistry revealed increased levels of cardiac troponin (up to 7.6 ng/mL) and creatine kinase (CK) (up to 597 IU/L). Transthoracic echocardiography (TTE) revealed ascending aorta dissection with severe aortic regurgitation. The patient underwent emergency cardiac surgery.
Anabolic Steroid Use and Aortic Dissection in Athletes: A Case Series
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A 20-year-old woman injured her right leg in a car accident. This was a circumferential degloving of the skin of the calf (). The patient received FTSGs made from the avulsed flap for coverage. On postoperative day 6, ongoing necrosis on the non-defatted area of the right calf was noted (). Debridement was performed and VAC was applied. At postoperative day 23, final coverage was done with acellular dermis and STSG from a thigh. At a follow-up at 6 months, no limitation of ambulation was evident. The patient requested camouflage surgery of the STSG area ().
A Simple Strategy in Avulsion Flap Injury: Prediction of Flap Viability Using Wood's Lamp Illumination and Resurfacing with a Full-thickness Skin Graft
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A 72-year-old man presented with a 3-year history of intractable left-sided facial pain. His pain was characterized by stabbing, burning, and dysesthesia, and involved the V1, V2, and V3 distributions of the trigeminal nerve. In the early stage, he was misdiagnosed with trigeminal neuralgia and underwent Gamma knife radiosurgery at an outside hospital. When the procedure and pharmacologic measures failed to provide pain alleviation, He was reexamined with paranasal sinus MRI and his diagnosis was confirmed with a biopsy of the nose and neck. At that time, his final diagnosis was inoperable hard palate cancer with intracranial extension, stage IVB (T4bN1M0). Since then, he has received concurrent chemoradiation therapy as another treatment option. During the treatment, his cancer-related facial pain became aggravated and refractory to the maximum dose of opioids including oxycontin, durogesic patches, and so forth. He was then referred to the neurosurgery department for surgical treatment of the intractable pain.\nWhen we checked his neurologic status, left hemifacial pain corresponding to a visual analogue scale (VAS) score of 9 combined with mild hypoethesia (decreased to 80 percent compared with the right side) and left masseter muscle atrophy with decreased mastication were identified. After careful consideration of the factors, including the factors involved in multiple clinical disciplines, the surgical option of a mesencephalotomy was discussed with the patient and his caregivers and they decided to proceed with it as a last resort due to the severity of his symptoms and the refractoriness to medical treatment.\nOn the morning of the operation, the patient's head was fixed parallel to the line between the infraorbital margin and the upper margin of the external auditory meatus in a Leksell stereotactic frame (Elekta Instruments, Atlanta, GA, USA), and coronal and axial T2-weighted MRI images were then obtained. The surgical target based on the MR image was chosen 5 mm behind the posterior commissure, 6 mm lateral to, and 5 mm below the intercommissural plane (). An MR navigation system (Stryker Instrument, Freibury, DEU) was used to define the appropriate trajectory. The surgery was carried out under local anesthesia. When the underlying dura and pia were opened with bipolar cautery, three straight microelectrodes (FHC Inc., Bowdoin, ME, USA) were inserted into the center, 2 mm medial, and 2 mm lateral to the target, for electrophysiologic mapping. After stimulation with the electrode, typical tingling and a warm sensation in the left upper arm and face were noted in the center tract (6 mm lateral to the right of the midline). After confirming the final target based on the electrophysiologic mapping findings, a lesion was made using a 2 mm diameter tip monopolar straight electrode with a 4 mm uninsulated length (Leibinger GmbH, Freiburg, Germany) and raising the temperature of the electrode to 80℃ for 60 seconds. And the lesion was increased in length by 2 mm by moving the electrode along the dorsolateral direction and by repeating the heating (). At the end of the operation, a navigation-guided Ommaya reservoir was inserted for intraventricular injection of morphine to aid in the control of the remaining cancer pain. The patient remained stable throughout the procedure.\nOn the first day after the operation, the left hemifacial pain had dramatically improved according to the VAS score, which dropped from 9 to 1, and the hypoesthesia on the left face showed a noticeable decrease of 50 percent compared with the right side. Besides, there was no evidence of any new gaze paresis or left-sided extremity dysesthesia. However, the pain slightly recurred with a VAS score of 3 a week after the surgery and the pain progressively increased, with the VAS increasing to 5 at postoperative two months. Until a recent date, the pain has been relatively well controlled, staying at VAS 4 or 5, by intermittent intraventricular morphine injections and oral opioids; also, there have been no neurologic deficits detected.
Stereotactic Mesencephalotomy for Cancer - Related Facial Pain
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We describe a 28-year-old Bangladeshi male, with no significant past medical history, that was referred to our institution with a 4-day history of fever, and a 1-month history of generalized fatigue and 6 kg weight loss. The patient denied night sweats, cough, dyspnea, shortness of breath, chest pain, headache, dysuria, diarrhea, vomiting, or constipation. His history was negative for a viral prodrome of including rhinorrhea or sore throat. He also denied sick contacts or recent travel. Examination revealed painless bilateral cervical lymphadenopathy, in addition to splenomegaly, jaundice, and pallor.\nThe patient was found to have severe anemia Hgb 5.9 (13-17 g/dL), associated with an increased reticulocyte count Retic # 177.1 (50.0-100.0 × 109/L), Retic % 5.4 (0.5%-2.5%), Bilirubin T 57.1 (3.4-20.5 µmol/L), Bilirubin D 9.0 (0.0-8.6 mg/dL), Lactic acid dehydrogenase (LDH) 546 (125-220 U/L), direct antiglobulin positive and decreased haptoglobin <10 (30-200 mg/dL). Additionally, laboratories revealed mild thrombocytopenia 108 (150-400 × 103/uL), markedly elevated ferritin 1222 (48-420 ug/L), low iron, Total iron-binding capacity (TIBC), and transferrin, as well as low B12. A peripheral blood smear revealed anisocytosis and poikilocytosis, target cells, oval, teardrop cells, spherocytes, basophilic stippling, Howell-Jolly bodies, polychromasia, and red cells fragments with many Nucleated red blood cells (NRBCs) seen with dysplastic forms and was suggestive of hemolysis. Direct Coombs test was positive.\nComputed tomography (CT) scan of the thorax and abdomen revealed centrilobular lung nodular opacities (likely infectious/inflammatory), bilateral hilar and mediastinal lymphadenopathy, in addition to prevascular, right paratracheal, and subcarinal lymphadenopathy, and marked osteopenia. Autoimmune hemolytic anemia secondary to lymphoma was suspected, and the patient underwent an excisional biopsy of a left cervical lymph node.\nThe patient was initially admitted at one of institutions hospitals, where he was admitted for a week, received multiple transfusions and underwent US-guided needle biopsy and then excisional biopsy, his Hb was stable over for than 72 hours and was discharged for follow-up of the excisional biopsy pathology report in the clinic. However, after 1 week the patient was seen in the clinic, laboratories revealed an Hb <7, he was admitted to the hospital, received transfusions, and his first excisional biopsy revealed Histiocytic Necrotizing Lymphadenitis. His Lymphadenopathy was still significant was the same overtime (not improving or worsening) on daily physical examinations. The suspicion for lymphoma was high so we did not give steroids (same reason as above) and underwent a second excisional biopsy which confirmed the diagnosis. He was admitted the second time for 2 weeks. After the 2nd excisional biopsy, he did not need blood transfusions and his Hb was >7 for more than 72 hours, steroids were not started and he was discharged. He was seen in the clinic again 1 week later, Hb was stable, still had lymphadenopathy that has not improved or worsened. Then, he was lost to follow-up.\nPathology review of the sample at our institution revealed unusual patchy paracortical areas of atypical cells with increased apoptotic debris and histiocytes, in addition to lymphocytic infiltrates. The differential of which includes lymphoproliferative disorders vs reactive conditions such as the proliferative phase of KFD. The sample was sent to Mayo Clinic for expert evaluation. The lymphocytic infiltrates contained abundant CD2, CD3, CD5, and CD7 positive T cells, with CD8 positive T cells that outnumber CD4 positive cells in these areas. Histiocytes were positive for CD68, and a subset was positive for myeloperoxidase. Hodgkin cells were not identified in the stains for CD30 and CD50, and no Epstein-Barr virus-positive cells were identified. A diagnosis of Histiocytic Necrotizing Lymphadenitis was made. The immuno-architectural features could represent KFD; however, identical morphological features can also be observed in cases of systemic lupus erythematosus.\nUpon further investigation, the patient's ANA, antidouble-stranded DNA, and anti-Smith antibodies were negative. A Positron Emission Tomography—Computed Tomography (PET/CT) revealed multiple hypermetabolic nodes, included bilateral cervical nodes, the largest measuring 1.3 cm (SUV up to 12.2) located in the left upper neck (Figure ,B), and a node measuring 9 mm (SUV extending up to 8.5) in the right lower neck (Figure ). Hypermetabolic nodes were also identified in the axilla (Figure ), mediastinum, left hilum, and portocaval region, raising the suspicion for lymphoma, despite a negative excisional biopsy.\nBone marrow aspiration (BM) showed that the aspirate smears were cellular, partially hemodilution with increased erythroid precursors with some dysplastic changes, but no increase in blasts. The report stated: "The suboptimal BM biopsy reflects hypercellular marrow (almost 100% cellularity) showing trilineage hemopoiesis with increased erythroid precursors with focal areas showing increased in reticulin fiber and marrow vasculature. No lymphoid aggregate. No definite granuloma noted. The erythroid hyperplasia is secondary to the hemolytic anemia (hemoglobinopathy). Causes of increased fibrosis may include medication, immune disease, chronic disorders, chronic inflammatory diseases, and infection, among other causes."\nThe patient underwent a second excisional biopsy of the right cervical lymph node. As stated in the report, “sections show a lymph node in which the paracortical areas are expanded by a polymorphous lymphohistiocytic infiltrate with abundant karyorrhexis and apoptosis." The histiocytic cells are positive for CD68 and CD163, and a subset is positive for myeloperoxidase. TdT, c-myc, ALK-1, EBV LMP1, HHV8 are negative. A diagnosis of Histiocytic Necrotizing Lymphadenitis was made.\nDuring his 4-week hospital course, the patient did require multiple blood transfusions but did not receive steroids. Toward the end of his hospital course, his hemoglobin stabilized without transfusions or steroids. Upon follow-up in the clinic 2 weeks after discharge, he reported overall improvement of his symptoms but continues to be jaundiced, and examination did reveal persistent cervical lymphadenopathy, in addition to clubbing, and splenomegaly. His hemoglobin was stable and is being monitored during his hematology follow-up appointments.\nHe was jaundiced but his Hb remained stable, and because of the self-remitting characteristics of the disease we decided to observe without intervention.
An atypical presentation of Kikuchi-Fujimoto disease: A case report & literature review
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A 52-years old man presented to our clinic with chest and back pain lasting for 3 months. On the chest radiography, pleural fluid was detected in left hemithorax (). On thorax CT, it was also detected that there were lytic bone lesions at level of 11th and 12th ribs and pleural thickening at paravertebral site on the left. In laboratory evaluations, following findings were observed: hemoglobin: 7.1g/dl.(14–18), white blood cells:11.26 × 103/μL(4.8–10.8), platelets: 787 × 103/μL., creatinine: 3.1mg/dl.(0.6–1.1).Uric acid: 6.1 mg/dl (2.6–6), calcium: 14.7mg/dL (8.8–10.6), total protein:10.7g/dL (6.4–8.3), albumin: 1.8g/dL (3.5–5.2). Serum protein electrophoresis revealed a hypoalbuminemia (21.1%; range; 55.8–66.1) associated with an increase in γ globulins (42.1%; range; 11.1–18.8). IgG-Kappa monoclonal paraproteinemia was detected in immunofixation tests (IgG: 4460mg/dL. reference interval, 850–1330; Kappa: 1640mg/dL, reference interval, 630–1350). ß-2 microglobulin was found as 2.71 mg/dlL (reference interval: 1.42–3.21). Bence-Jones protein was found to be negative in 24-hours urine collection. A thoracentesis was performed, which revealed serofibrinous fluid with a protein level of 6.3gr/dl and white blood cell count of 6.65 × 103μL consisting of lymphocytes (80%) and neutrophils (20%).\nBacterial and micobacterial culture tests of pleural fluid were reported as negative. Biopsy was performed on the mass at rib, which was reported as CD138(+), CD20(−) plasmocytoma. Pleural fluid flow cytometry was performed by using FACSCalibur flow cytometer (Becton-Dickinson, Erembodegem, Belgium). In the flow cytometry, CD56, CD38 and CD138 found to be positive, while CD19 was negative. Bone marrow biopsy was reported as CD38 (+) and CD20(−) atypical plasma cell infiltration. T(4;14), 17p13,1 (p53 gene) and 13q14,3(Rb gene) was found as negative by FISH method.\nThe patient was scheduled for VAD (vincristine, doxorubicin, dexamethasone) chemotherapy, biphosphonate therapy and involved-field radiotherapy.
Flow Cytometry Method as a Diagnostic Tool for Pleural Fluid Involvement in a Patient with Multiple Myeloma
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A 53-year-old Caucasian male with a past medical history significant only for heterozygous FVL deficiency presented with a complaint of typical angina of two weeks duration. The patient reported an athletic lifestyle, with recent exertional chest pain while kayaking and cycling. The patient reported that his most recent episode of pain awoke him from his sleep, prompting him to seek evaluation in the emergency department.\nLaboratory testing revealed an elevated troponin I of 0.43 ng/mL (normal: <0.08 ng/mL), troponin T of 0.13 ng/mL (normal: <0.01 ng/mL), and pro B-type natriuretic peptide of 898 pg/mL (normal: <125 pg/mL). ECG showed normal sinus rhythm with deep symmetrical T wave inversions across anterior precordial leads suggestive of Wellens syndrome with Type B findings (Figure ).\nSignificant risk factors were evaluated and not found to be present. The patient was found to have a lipid profile of cholesterol 145 mg/dL, high-density lipoprotein (HDL) 32 mg/dL, low-density lipoprotein 95 mg/dL, non-HDL 113 mg/dL, triglycerides 88 mg/dL, very low-density lipoprotein 18 mg/dL, and hemoglobin A1c of 5.6%. The patient denied any history of hypertension and presented with a blood pressure of 122/79 mmHg on admission. He was recorded to have a body mass index of 26.3 kg/m2 and denied any smoking or alcohol history. No familial history of premature CAD was present. The patient was considered a moderate risk based on the HEART Score for six-week risk of major adverse cardiac events.\nThe patient underwent emergent cardiac catheterization, which revealed one-vessel CAD with a complete ostial occlusion of the LAD (Figure ). The right coronary artery (RCA) and left circumflex artery did not have any angiographically significant disease. Well-established collaterals were present from the dominant RCA, which supplied the mid-segment of the LAD (Figures , ). Due to the proximal location and nature of the occlusion, no intervention was attempted, and the patient was immediately referred to cardiac surgery for coronary artery bypass graft (CABG).\nThe patient underwent successful CABG with the left internal mammary artery to the LAD. He was ultimately discharged home on guideline-directed medical therapy, dual anti-platelet therapy with aspirin and clopidogrel, and without any complications from the procedure.
An Unexpected Factor to Wellens Syndrome
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A 27-year-old Caucasian woman presented with a 2 week onset of diffuse itchy papules, initially on the eyelids, then on the trunk and extremities. Her medical history included mild atopic dermatitis and 3 month previous fulminant hepatitis with cholestasis and multi-organ involvement secondary to trimethoprim and sulfamethoxazole hypersensitivity. A hepatology consultation and liver biopsy confirmed the diagnosis. For this reason, prior to appearance of the cutaneous lesions, the patient was taking prednisone 7.5 mg PO daily (initially started at 60 mg PO daily 3 months prior) and cholestyramine 12 g PO daily. Family history was negative for dyslipidemia or early cardiovascular events.\nOn physical examination, she presented diffuse icterus of sclera and skin as well as numerous 1 to 2 cm scaly mildly erythematous papules and plaques on the abdomen, back, limbs and face, mimicking pityriasis rosea (). Furthermore, she had palmar xerosis and discrete yellowish papules in the palmar creases, suggestive of xanthoma striatum palmare ().\nThe patient was concurrently under investigation for hyponatremia; plasma sodium was very low at 123 mmol/L (normal: 134–144) with normal plasma osmolality at 298 mOsm/kg. Workup revealed extremely elevated total cholesterol at 46.84 mmol/L (normal: 3.8–5.2), elevated triglycerides at 5.47 mmol/L (normal: 0.6–2.3), low high-density lipoprotein at 0.19 mmol/L (normal: 0.9–2.2), elevated alanine aminotransferase at 72 U/L (normal: 3.5–50), and elevated conjugated bilirubin at 136 µmol/L (normal: 1.7–8.6), thus confirming a pseudohyponatremia secondary to hypercholesterolemia in the context of hepatic cholestasis. A lipoprotein electrophoresis demonstrated abnormal migration of beta and pre-betalipoproteins in only one band as well as absence of alpha-lipoproteins, which was compatible with lipoproteinemia X dyslipidemia.\nA skin biopsy on the flank revealed mild verrucous epidermal hyperplasia with hyperkeratosis and a prominent papillary dermal infiltrate of xanthomatous cells, compatible with verrucous xanthomas ().\nA trial of topical corticosteroids prior to confirmation of diagnosis provided mild relief of itch. Given the development of dyslipidemia while already on cholestyramine for 3 months, decision was made to initiate weekly apheresis. As lipid apheresis was not available at our center, conventional apheresis (1 plasma volume exchange per procedure with albumin) was used and still lowered her total cholesterol from 46.84 to 5.74 mmol/L in 10 weeks. It was administered every week for 6 weeks, then every 2 weeks for 6 weeks. After the second procedure, the patient already noticed improvement of the pruritus. After 10 weeks, her lesions improved greatly, leaving residual hyperpigmented macules (). To this date, the patient was free of relapse 2 months after discontinuation of apheresis.
Verrucous plane xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis: A case report
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A 84-years-old Caucasian man presented in our unit with a chronic scalp eruption characterized by erythematous patches and crusting (). The eruption compared few weeks after topical treatment with imiquimod cream used for actinic keratosis and lasted for several months without spontaneous resolution. The dermatosis did not respond to treatments with topical antibiotic and steroid. Bacteriological and mycological tests were negative. Histology was nonspecific and direct immunofluorescence was negative. On the basis of the clinical and laboratory data a diagnosis of EPDS was made. According to timing of clinical manifestations and anamnestic data, we could infer that the disease was associated with a history of actinic keratosis and androgenetic alopecia and had been triggered by a previous treatment with imiquimod. Systemic steroid therapy with prednisone 0.75 mg per kg was started and a complete resolution of the disease was observed after few weeks. Therapy was then slowly reduced and the clinical manifestations of EPDS completely resolved in about 2 months (). The patient did not show cutaneous exacerbations of the disease and is currently in complete remission.
Erosive Pustular Dermatosis of the Scalp Induced by Imiquimod
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A 28-year-old male was referred to the emergency department of our hospital presenting with severe headache that occurred five hours ago when he was driving a car. The headache was of sudden onset and associated with intermittent vomiting. Non-contrast head computed tomography (CT) scan performed in the local hospital showed diffused hyperdensity in anterior and posterior fissure, Sylvian fissure, and around the brainstem, indicating the diagnosis of subarachnoid hemorrhage (SAH) (Figures -). Twelve months ago, the patient suffered right-sided weakness. Brain magnetic resonance imaging (MRI) performed in the other hospital demonstrated acute ischemic stroke in the left frontal lobe. The hemiparesis has recovered completely and he has been taking aspirin 100 mg per day ever since. The patient has no other remarkable past medical history.\nThe patient was admitted to the Department of Neurology for further management. On admission, he was afebrile and drowsy, with heart rate of 69 beats/min, respiration rate of 22 breaths/min, and arterial blood pressure of 129/65 mmHg. A 3/6 pansystolic apical murmur was heard by auscultation. Neurological examination revealed neck stiffness. On day two after admission, digital subtraction angiography (DSA) was performed for evaluation of intracerebral aneurysm. But no aneurysm or cerebral vascular malformation was found on the initial DSA (Figures , ). Results of the laboratory test were unremarkable except for a slightly elevated white blood cell count (11.9 * 109/L). The electrocardiography (ECG) showed nodal tachycardia. Brain MRI performed on day four revealed a previous infarction in the left frontal lobe (Figures , ). Transthoracic echocardiography (TTE) showed a dilated heart and detected a left atrial mass measuring 5.09 * 3.34 cm in size attached to the lower part of interatrial septum, oscillating with heat contraction (Figure ). Moderate-to-severe mitral regurgitation and mild tricuspid regurgitation were observed too (Figure ). A diagnosis of left atrial myxoma was considered.\nThe patient was transferred to the Department of Cardiac Surgery and underwent atrial tumor resection soon. A gray, polypoid, colloid-like left atrial mass of 6 * 5 * 2.4 cm in size was excised. No residual tumor tissue was detected by transesophageal echocardiogram (TEE). Pathological examination confirmed the diagnosis of atrial myxoma (Figure ). The patient’s symptoms improved in the following two weeks. On day 24 after onset, brain CT angiography (CTA) was performed in order to re-evaluate intracerebral aneurysm, but there was no aneurysm or any other vascular malformation found (Figure ). On day 28, the patient was discharged with no residual neurological symptoms.\nWe have followed the patient for one year. He remains asymptomatic since discharge. At six months after discharge, TTE was performed revealing no recurrence of the cardiac tumor. And at 12 months, no abnormality was found by a repeated brain CTA (Figure ).
Subarachnoid Hemorrhage Following Ischemic Stroke Caused by Atrial Myxoma
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An 11-year old boy was admitted to our Neurosurgical Department after he was break-dancing with his brother. During a head-spin stunt he injured his neck. After this performance, he was talking about pain in his neck. We did not found any neurological deficits like paresis, except the violent pain in his neck. Because of the fast paced trauma, we decided to perform a computed tomography (CT) examination of the neck-spine.\nThe CT shows a C1/C2 dislocation with a combined dens fracture (). The dens axis lies in front of the atlas. To foreclose an injury of the vertebral artery, a CT-angiography has been performed ().\nSurgical repositioning was performed. In a first step the patients head was fixed into a Mayfield clamp in the supine position. Under X-ray control we started the reposition itself by axial extension. After this maneuver we were able to get the dens axis back in his normally position.\nIn the next step the patient was turned into the supine position. Another X-ray control shows that the dens axis and the atlas arch are well positioned.\nA translaminar fixation of C1/C2 had been performed. Two 16 mm screws were placed into the C2 arch and two smaller ones with a length of 14 mm were screwed into the arch of C1. This construction was finally fixed with side and cross rods. The post-operative CT control is shown in .\nIn the 3-, 6- and 12-month follow-up examinations the patients does not show neurological deficits. He is wearing his soft collar for protecting the neck. We decided to treat him with this collar, longer than usual, because of missing experiences in the literature. We are planning to remove the screws in December, one year after the accident.
Can Break-Dance Break Your Neck? C1/C2 Luxation with a Combined Dens Fracture Without Neurological Deficits in an 11-Year Old Boy After a Break-Dance Performance
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In February 2003, a 57-year-old man (height, 168 cm; body weight, 77 kg) presented\nwith painless gross hematuria. Ultrasonography indicated a solid mass in the left\nkidney. He took Chinese herbal medicines for several months, but the hematuria\ncontinued. When he revisited our clinic in October 2003, a computed tomography (CT)\nscan showed a solid mass of about 8×7 cm in the lower pole of the left kidney (about\n14×11 cm) infiltrating the major psoas muscle, with a long embolus in the inferior\nvena cava (IVC) that reached the entrance of the hepatic vein (). He had a Karnofsky Performance Status (KPS) score of\n70%, and Eastern Cooperative Oncology Group (ECOG) performance status of 2. The\npatient had a history of chronic gastritis (diagnosed in 1998), hyperlipidemia\n(diagnosed in 2002), fatty liver disease (diagnosed in 2004), right carotid artery\natherosclerosis (diagnosed in 2005), benign prostatic hyperplasia (BPH, diagnosed in\n2005), and a left maxillary sinus cyst (diagnosed in 2006), but he is currently\ntaking no medications for these conditions.\nIn November 2003, the patient underwent radical left nephrectomy and removal of the\ntumor embolus in the IVC. The tumor was stage IV according to the 2002 American Joint\nCommittee on Cancer (AJCC) staging system. Postoperative pathological examination\nconfirmed advanced clear-cell carcinoma (pT4N0M0 Fuhrman grade II). Forty days later,\nCT scans revealed no residue of tumor in the surgical site, but one small (about 0.6\ncm) nodule was discovered in the left lower lung. Fortunately, the patient was\nstratified as favorable according to Memorial Sloan-Kettering Cancer Center RCC\nnomogram. He was started on a combined immunotherapy regimen (interleukin-2 plus\ninterferon), an adjuvant protocol that followed the treatment guidelines for renal\ncancer recommended by the Chinese Urological Association, and which was adapted for\nChinese patients from National Comprehensive Cancer Network (NCCN) practice\nguidelines (). The protocol included two\ncycles of recombinant human interleukin-2 (18 million IU, sc for 5\ndays in the first week, followed by 3 weeks of 18 million IU for 2 days and 9 million\nIU for 3 days) and recombinant human interferon-α2b (9 million IU,\nim, 3 times per week for 12 weeks). Close follow-up was\nmaintained, including disease history, physical examination, routine blood tests,\nkidney and liver function tests, and CT imaging every 3 months. In August 2005, a\ncystic change was noted in the right kidney. Urodynamic tests revealed increased\nresidual urine (70 mL), and ultrasonography suggested BPH.\nIn June 2006, two solid masses (1.0×1.0 cm and 1.5×1.5 cm in size) appearing to be\nmetastases were found, in the middle and upper pole of the right kidney (). The small nodule in the left lower\nlung had not changed significantly. The patient underwent a second surgical procedure\nvia the primary abdominal incision to enucleate the two masses in the right kidney.\nIntraoperative frozen sections confirmed metastatic clear-cell carcinoma. The patient\ncontinued the previous immunotherapy for two cycles after the surgery. Unfortunately,\nin November 2006, three lung nodules involving both lungs, each smaller than 1 cm,\nwere found.\nSorafenib is recommended as a first-line targeted therapy for mRCC by the NCCN\nguidelines, and received marketing approval in China in November 2006. As the\npatient's mRCC diagnosis satisfied the 2002 AJCC criteria, he began sorafenib\ntherapy, 400 mg twice daily, in January 2007, when the largest of the 3 lung nodules\nbecame more apparent, growing to about 1.3×1.5 cm in size ().\nTreatment response was evaluated according to the response evaluation criteria in\nsolid tumors (RECIST) by an expert team that included both a radiologist and a\nurologist. The disappearance of all known lesions within 1 month was considered a\ncomplete response. A greater than 30% decrease in the total length of longest lesion\ndiameter was considered a partial response, and a greater than 20% increase in the\nsmallest sum as progressive disease. Changes between these limits were defined as\nstable disease. Adverse events were assessed by National Cancer Institute's Common\nTerminology Criteria for Adverse Events (CTCAE) 3.0. Grade 1, 2, 3 adverse events\nthat occurred in this patient were described as mild, moderate and severe,\nrespectively.\nOne week after starting sorafenib therapy, the patient experienced grade 1 hand-foot\nsyndrome, stomatitis, fatigue, and grade 2 diarrhea. These reactions were controlled\nby symptomatic treatment without the need for dose reduction or therapy interruption.\nTwo months after starting sorafenib therapy, a small embolus was found in the IVC,\nbut the size of the nodules in the lung had decreased. The RECIST evaluation\nindicated stable disease until January 2011, when CT images revealed a nodule, about\n2.9×3.0 cm in size, in the left adrenal gland, an oval, low-density lesion in the\ntail of the pancreas, and several small nodules in the left lower lung. The RECIST\nevaluation clearly confirmed progressive disease. A larger dose of sorafenib (600 mg\ntwice daily) was recommended. However, the patient could not tolerate the higher dose\nowing to uncontrolled severe diarrhea (grade 3), and so in February 2011 sunitinib\nwas carefully selected as a sequential targeted agent.\nThe patient started sunitinib therapy at a dose of 50 mg/day in February 2011 and\nexperienced mild adverse effects, grade 1 diarrhea. In November 2011, metastases were\nconfirmed in the right kidney, left adrenal gland (), tail of the pancreas, and the liver, and the embolus in the\nIVC was enlarged. The nodules in both lungs had disappeared. One month later, the\nmetastases in the left adrenal gland and tail of the pancreas had shrunk, but the two\nmetastatic nodules in the liver and the embolus in the IVC were still present. As the\ncomprehensive evaluation at this time was stable disease, the patient continued\nsunitinib therapy.\nIn December 2012, the patient was hospitalized for acute urinary retention.\nUrodynamic tests revealed a maximum voiding flow rate of 1.7 mL/s and residual urine\nof 36 mL consistent with bladder outlet obstruction induced by BPH. He then underwent\na third operation, transurethral resection of the prostate as treatment of the BPH.\nPostoperative pathological examination confirmed the BPH diagnosis. The patient\nrecovered well and regained normal urination.\nBy January 2013, the metastasis in the right kidney had increased to 2.7×2.2 cm\n(), at which time the patient\nunderwent a fourth operation for cryoablation of metastases in both the right kidney\nand the right hepatic lobe ().\nSunitinib therapy was continued until disease progression was confirmed in July\n2013.\nIn August 2013, the patient was started on everolimus at a dose of 10 mg/day. He\ntolerated this treatment well; the only adverse events were grade 2 fatigue and\nstomatitis. As of 10 February 2014, the patient continues on everolimus therapy with\nstable disease and ECOG and KPS score of 1 and 90%, respectively. His quality of\nlife, evaluated using the WHOQOL-100 questionnaire, is satisfactory. Close follow-up\nof the patient is ongoing.
More than 10 years survival with sequential therapy in a patient with\nadvanced renal cell carcinoma: a case report
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A 66-year-old man with the diagnosis of Fuchs' endothelial dystrophy presented with low visual acuity and pain in both eyes. The visual acuities were 20/63 in both eyes and did not improve with refraction. Both corneas appeared thick central and inferiorly with epithelial microbullae and stromal edema (). There was mild nuclear sclerosis in both eyes and dilated fundus examination of both eyes was normal. The central corneal thicknesses (CCT) of the right and left eyes were 575 and 550 μm and the endothelial cell counts (ECC) were 1722 ± 32 and 1711 ± 36 cells/mm2.\nSince the patient did not wish to be fit with bandage contact lenses for pain relief, he was offered CXL. Standard CXL was performed on both corneas of this patient 1-week apart. Reepithelization was complete by day 4 in each eye and the bandage contact lenses were removed. Postoperatively, the visual acuities in both eyes were 20/63 at all follow-up examinations from month 1 to month 12. The CCT measurements were 580, 586, 579, and 582 μm in the right eye and 570, 580, 578, and 585 μm in the left eye at months 1, 3, 6, and 12 (). The ECC was 1705 ± 43 and 1703 ± 40 cells/mm2 in the right and left eyes at postoperative month 12. Since no clinical improvement could be obtained, a repeat CXL procedure was performed in the left eye of this patient. During surgery, following riboflavin 0.1% application for 30 minutes, CCT was measured as 534 μm. Glycerol 70% drops were applied every 15 seconds until the corneal thickness was 445 μm. This was followed by UVA irradiation (365 nm, 3 mW/cm2) for 30 minutes. At the end of the procedure the CCT was measured as 421 μm. Reepithelization was complete by day 4, and the contact lens was removed. The visual acuity was again 20/63 throughout the postoperative follow-up. There was no change in corneal edema at slit lamp biomicroscopy, no change in CCT, and no change in ECC until the last follow-up at month 6 (). The patient did not wish to undergo a repeat CXL procedure in the right eye.\nsummarizes the results of the CXL procedures performed in Cases 1 and 2.
Outcome of Two Corneal Collagen Crosslinking Methods in Bullous Keratopathy due to Fuchs' Endothelial Dystrophy
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A 54-year-old Caucasian woman therapist with no past history of serious illness was referred for Holter monitoring in September 2008 after complaining of episodic palpitations over 4 years, resulting in disruption of daily activities. There was no history of syncope and no family history of arrhythmia or sudden death. At the time of presentation, she was taking no medication. Routine biochemistry and hematological analysis proved normal. A physical examination was normal.\nA 24-hour Holter electrocardiogram (ECG) demonstrated frequent VEB, couplets and frequent runs of 3–5 beats of monomorphic NSVT. In total 2448 ectopic beats were present, all of the same morphology, with a relatively narrow ventricular ectopic QRS interval (VEQSI) of 160 ms. Transthoracic echocardiography demonstrated a structurally normal heart, confirmed by cardiac magnetic resonance scan. Our patient declined curative ablation. Because of a history of suspected asthma, beta-blocking drugs were relatively contraindicated. She was discharged on a slow-release preparation of verapamil 240 mg daily for control of symptoms. This reduced the frequency of ventricular ectopy and diminished the symptoms to a tolerable level.\nOne month later, our patient was readmitted after suffering an out-of-hospital VF cardiac arrest from which she was successfully resuscitated (Fig. ). Coronary angiography demonstrated normal coronary arteries. On recovery, our patient accepted our recommendation that she undergo ablation of the focus of ventricular ectopy but she refused implantable cardioverter defibrillator (ICD) therapy.\nAt electrophysiology study, a decapolar diagnostic electrophysiological catheter (Bard, Electrophysiology Division, Lowell, MA, USA) and a cryoablation catheter (Freezor Extra™, Medtronic, Minneapolis, MN, USA) were used. We found electrogram amplitude to be normal throughout the right ventricle. Frequent ventricular ectopy was present; activation mapping traced the site of origin to the anterior aspect of the RVOT approximately 1 cm below the pulmonary valve.\nCryotherapy to –80 °C for 3 minutes was delivered at this site with complete suppression of ectopy during delivery but recurrence on rewarming. A second delivery at an adjacent site resulted in persistent elimination of ectopy (Fig. ). No further ventricular ectopy occurred during 40 minutes of observation in the electrophysiology laboratory or during the 4 days of that hospital admission.\nHolter recordings 1 day and 1 week after ablation recorded only three ectopic beats on each occasion, none of which appeared to be of the clinical morphology. After reflection, our patient decided to accept ICD therapy. A single-lead ICD was implanted 10 days post-ablation. At 7.5 years post-ablation, interrogation of her ICD shows no record of any sustained ventricular arrhythmia. Our patient has requested that the device not be replaced when the battery becomes depleted.
Ventricular fibrillation treated by cryotherapy to the right ventricular outflow tract: a case report
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Patient 7 was a 30-year old male born and brought up in Uganda but was a UK resident at the time of the malaria episode. He was diagnosed with falciparum malaria in Switzerland in September 2007 after travel to Uganda, and reported full adherence to a three day therapeutic course of AP. Parasite clearance was not confirmed microscopically. He presented again in London 21 days after treatment with 0.2% P. falciparum parasitaemia and was treated with a course of quinine. DNA was extracted for analysis from a blood sample collected at the time of his recrudescent infection.
Mutations in the Plasmodium falciparum cytochrome b gene are associated with delayed parasite recrudescence in malaria patients treated with atovaquone-proguanil
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A 33-year-old woman affected by hypergonadotropic hypogonadism (FSH 71.38 mIU/L; LH 35.50 mIU/L; estradiol <7 pg/mL) had been treated with estroprogestinic therapy “Triminulet” for nine years. Treatment was then suspended to allow a reevaluation of ovarian functionality. Upon drug withdrawal the patient presented secondary amenorrhea and was referred to our unit for an indepth diagnostic evaluation. A written informed consent was obtained.\nMedical history revealed that the patient was born with normal delivery, bottle-fed, and showed standard psychosomatic development, menarche at age of 13 and oligomenorrhea since then up until estroprogestinic therapy was established. Pelvic US demonstrated a normally placed and normally developed uterus with a slightly dishomogeneous structure. The ovaries were not visible.\nThe patient was also affected by autoimmune thyroiditis, treated with levotiroxin. Ten years earlier (2002) the patient received a diagnosis of lactotrophic microadenoma (PRL 125 ng/dL); brain MRI “dubious small area of enhancement in the lower right paramedian pituitary” and was treated with different dosages of cabergoline. This treatment was suspended in august 2010. In 2011 a brain MRI was substantially unmodified compared with with the previous one. In addition the patient reported myopia and arthralgia since she was 15 years old Family history showed familiarity for thyroid, neurological cardiovascular diseases (see pedigree: ).\nPhysical examination: height 158 cm (3°–25°p), weight 64 Kg (50°p), BMI 25,63 Kg/m2, waist circumference 92 cm, cranic, and upper and lower limb measurements between 50° and 75° percentile. No alterations detected in fingers and toes. Apparently a normal development was observed for secondary sex characteristics. Facial features revealed no dysmorphisms, moreover neither mental impairment or cognitive deficit was evident. Vital signs were normal.\nA blood sample was drawn in Sodium (Na) heparin coated tube (vacutainer) for mononuclear cells culture and GTG karyotyping. High-definition GTG banding analysis of 100 metaphases (Leica CW4000 software) demonstrated a 45,X (41%)/46,X,rea(X) (59%) mosaicism ().\nCBG staining of a new lymphocyte preparation demonstrated the presence of two centromeres in the X rearranged population [46,X,rea(X)] (). Finally FISH analysis (kit ToTelVysion (TM) Multicolor DNA probe mixtures (Vysis)), confirmed the presence of two X centomeres in rearranged X chromosomes with 2 Xp extremities and no Xq extremity. In the rearranged X chromosome the Xq extremity was substituted by Xp and pericentrometric Xq region. After FISH analysis the subpopulation previously indicated as 46,X,rea(X) was then redefined as 46,X,idic(X)(q24) ().\nThe final definition of the karyotype as released to the is mos 46,X,idic(X)(q24)?[59]/45,X [41], compatible with a “Turner like” condition that can account for the clinical and endocrinological condition of the patient.\nAfter a written informed consent was obtained from first degree relatives of our patient (parents and sister), a GTG karyotype analysis extended to family members demonstrated that the phenotype observed in our patient was the result of a de novo alteration.
A Case of Premature Ovarian Failure in a 33-Year-Old Woman
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A 17-year-old female presented to the ED with one day of dull, intermittent, 6/10 abdominal pain originating in the periumbilical region and migrating to the right lower quadrant. She complained of nausea and decrease in appetite but no vomiting or diarrhea. She reported she had not had a bowel movement in three days, a normal pattern for her. She denied fever, chills or dysuria. Her medical history was positive for depression and shellfish allergy. Family history and review of systems were negative. Vital signs were normal, and the only pertinent physical exam finding was moderate tenderness in the right lower quadrant with voluntary guarding without rebound. Laboratory data revealed a normal white blood count (WBC), urinalysis, electrolytes, blood urea nitrogen and creatinine and a negative urine pregnancy test. Computed tomography (CT) of the abdomen and pelvis showed no evidence of appendicitis nor renal, hepatic, adrenal, pancreatic or bladder abnormalities. The radiologist noted a low-lying cecum dilated to 8cm but no evidence of obstruction (). A pelvic ultrasound revealed two 1.5cm right ovarian cysts. With time the patient reported pain relief, and re-examination of her abdomen was unremarkable. She was discharged with instructions to follow up with her pediatrician and gynecologist.\nThe patient returned to the ED 11 hours later reporting recurring abdominal pain. The pain had increased in intensity from the previous day and was now associated with vomiting. Physical exam was significant for tenderness in the right lower quadrant with voluntary guarding. The patient refused a pelvic examination during her first visit, and the exam now failed to reveal any cervical motion tenderness, adnexal masses or tenderness. Complete blood count and chemistry profile were again normal and the patient was discharged once her pain improved with a diagnosis of abdominal pain secondary to ovarian cysts.\nThe patient returned to the ED 27 hours after the second visit reporting severe abdominal pain in the right lower quadrant, nausea, vomiting, and no bowel movement in four days. Her abdominal exam revealed decreased bowel sounds, distention and tenderness in the right lower quadrant with voluntary guarding. WBC was elevated (13.6) and a repeat CT showed marked dilatation of the cecum up to 10.5 cm with a transition point in the right lower quadrant suspicious for obstruction and cecal volvulus ().\nThe patient was taken to the operating room for laparatomy and reduction of cecal volvulus. Surgical findings revealed a mobile cecum with serosal tears and marked distention, and the patient underwent a right hemicolectomy. Pathologic findings of the surgical specimen revealed markedly dilated ileum and cecum with evidence of ischemic necrosis. The remainder of the patient’s hospital course was unremarkable, and she was discharged on post-operative day five.
Cecal Volvulus In Adolescence Presenting As Recurring Visits For Abdominal Pain
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A 71-year-old hypertensive lady presented to us with an abscess over her right knee after 2 years of asymptomatic period following an uneventful bilateral TKA. Clinical examination revealed tender swollen knee joint with pus drainage from the surgical scar which grew Staphylococcus aureus on bacterial culture. However, polymerase chain reaction (PCR) and culture for acid-fast Bacilli were negative. Laboratory investigations demonstrated raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (89 mm/h and 55 mg/L, respectively), with synovial fluid leukocytosis. Radiograph suggested evidence of loosening of the implant []. She underwent resection arthroplasty where loose implants were extracted and substituted with gentamicin impregnated cement spacer []. Surprisingly, intraoperative specimens failed to grow an organism. Postoperatively, she was given antibiotics (cefoperazone-sulbactam and linezolid) as per earlier antibiotic susceptibility test. However, ESR and CRP stayed high (92 mm/h and 7.36 mg/L, respectively). In contrast, total leukocyte count remained normal at all times.\nShe redeveloped the abscess over right knee after 3 months. Mycobacterium was isolated this time from synovial fluid aspirate on BACTEC MGIT 960 system containing Middlebrook 7H9 broth base with OADC (oleic acid, bovine albumin, catalase, dextrose, and polyoxyethylene stearate) enrichment and PANTA (polymyxin B, amphotericin B, nalidixic acid, trimethoprim, and azlocillin) antibiotic mixture. Further test over culture isolate using SD TB Ag MPT 64 rapid assay using mouse monoclonal anti-MPT antibody tested positive for MOTT. Species identification using Hain test which is based on reverse hybridization of PCR products with their complementary probes targeting 23S rDNA revealed M. abscessus []. Subsequently, she was started on second line anti tubercular drugs (clarithromycin, levofloxacin, and amikacin) based on in vitro antimicrobial susceptibility test. Amikacin was substituted with imipenem after 3 weeks due to raised creatinine.\nPersistence of symptoms and raised ESR and CRP (66 mm/h and 4.02 mg/L, respectively) after 6 weeks of anti tubercular treatment (ATT) led to re-debridement with change of cement spacer when necrotizing granulomas consistent with tuberculosis were observed on histopathological examination []. Following surgery, she was maintained on same anti tubercular drugs; however, imipenem was discontinued after 3 weeks. After 2 months, the ESR and CRP improved (32 mm/h and 0.8 mg/L, respectively) and revision TKA was performed with long stemmed tibial and femoral components []. Intraoperative tissue cultures were sterile. ATT was stopped after 6 weeks of negative culture report. She was reviewed at regular intervals for 2 years and no clinical relapse was noted. At her last followup, she had stable knee with 0–90° flexion []. Informed consent was obtained from the patient for reporting her case including clinical photographs.
Prosthetic knee joint infection due to Mycobacterium abscessus
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The female patient, aged 49, reported pains in her second and third finger of the right hand that she had had for 2 years. Initial diagnosis revealed isthmus carpalis and the patient was operated on without much success—no improvement was noticed.\nA year later, intensifying root pains of Root C5 and C6 on the right side were the reason for performing an MRI scan of the cervical spine. The findings from the study showed an intramedullary lesion of 1.5 cm in diameter, localized at the height of C5–C6 with accompanying syrinx stretching from C3 to C7. The patient was consulted neurosurgically several times, but refused to submit to a surgical treatment. Gradually, the pains extended to the entire forearm of the upper right limb, and numbness appeared of both lower limbs. She met the clinical diagnostic criteria for VHL disease []. Sequencing of the VHL gene revealed the presence of the 393C>A (N131K) missense mutation (Fig. ) []. VHL disease was also diagnosed in her relatives (shown in Fig. ).\nThe patient was treated surgically several times at the Ophthalmological Department because of gradual deterioration of vision in the left eye. Presently, the patient claims to be blind in the left eye. An ophthalmological examination proved the presence of a vast retinal angiomatosis of the left eye.\nMoreover, a CT scan of the abdominal cavity showed renal cysts.\nIn the clinical examination carried out on admission to the Department of Neurosurgery the following conditions were found: amaurosis oculi sinistri, paraesthesiae to algetic stimuli in the medial part of the right forearm and first , second and third fingers of the right arm increased deep reflexes in the upper right limb along with decreased muscle power of this limb, as well as Babiński syndrome on the right side; RR: 134/85 mmHg; pulse: 76/min.\nAn MRI scan of the cervical spine showed a contrast enhancing image of an intramedullary lesion located near C5/C6 and extending to the dorsal part of the spinal cord on the right side to the subarachnoid space. Upwards to C3 and downwards to C7 syrinx was identified. An MRI of the head demonstrated an angioma of 1.5 cm in diameter, localized in the choroid fissure on the right side with a small cyst.\nThe patient was treated surgically. A laminectomy was performed from C3 to C6. Then the spinal cord was incised along the sulcus medianus posterior to uncover a lesion of the type of haemangioblastoma and a syrinx filled with yellow liquid. The neoplastic lesion was localized excentrically in the dorsal part of the spinal cord on its right side; it reached the subarachnoid space and was connected to Root C5 originating in the spinal cord. The tumour was excised completely. In the postoperative course, some paraesthesia and pains in the upper left limb appeared, which gradually subsided within 3 weeks.\nAfter the surgery, the upper right limb pain subsided and the right-sided hemiparesis retreated. A follow-up MRI showed a total resection of the tumour.\nBelow we present an MRI image of the cervical spine before and after the surgery (Fig. a, b).\nFindings from a histopathological test showed haemangioblastoma.\nAt a follow-up examination estimating how complete the excision had been, a tumour was discerned at the common carotid artery bifurcation on the right side. As a result, a detailed examination of the patient was made at the Radiodiagnostics Department of the Comprehensive Cancer Centre in Gliwice.\nThe radiological examination showed a carotid body paraganglioma of 45 × 32 × 24 mm3 on the right side (Fig. a, b)\nThe tumour can be seen in the right carotid space, in the right common carotid artery (CCA) bifurcation into the internal carotid artery (ICA) and the external carotid artery. The tumour appears hypointense in unenhanced T1-weighted images. “Salt and pepper” signal pattern of the tumour is visible in T2-weighted images.\nThe tumour appears fast and intense after contrast enhancement.\nTime signal intensive curves calculated from the tumour, CCA, ICA appear to be of the same shape, maximum value of the signal intensity and maximum contrast enhancement at the same time. These signs evidence the heavy vascularization of the tumour. There are irregular no-signal spots in T2 in the lesion, which may be consistent with haemosiderin. The tumour models and translocates outwards the neighbouring vascular structures without infiltrating them; it models the parapharyngeal space and the right-sided wall of the pharynx, as well as to some extent the pterygeal muscles. The upper pole of the tumour is adjacent to the cranial basis near the carotid canal inlet, without traces of infiltration. The posterior part of the tumour leans against the I and II vertebra arches on the space of about 1.5 cm, without traces of infiltration. No traces of infiltrating the intervertebral foramen were found. The lower pole of the tumour is located about 2 cm above the division of the common carotid artery. The tumour had not given any symptoms until then. The patient refused to agree to the treatment of the carotid body paraganglioma. Laboratory testing of the noradrenaline level, adrenaline in blood and HVA in the 24-h urine collection failed to give evidence of catecholamine production by the paraganglioma.\nThree weeks after the surgery of the haemangioblastoma of the spinal cord the patient was discharged from hospital in good overall condition.
A case of carotid body paraganglioma and haemangioblastoma of the spinal cord in a patient with the N131K missense mutation in the VHL gene
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A 56-year-old female presented for evaluation of a scaly, thickened rash on the hands and feet. It started approximately four months previously on the left first finger and rapidly developed on the other hand and both feet. She reported pruritus but no tenderness. Two months into the course of the rash, she was diagnosed with cervical SCC (FIGO stage IIB).\nPhysical exam revealed scaly hyperpigmented plaques on the ulnar surfaces of the bilateral hands and the periungual skin of the fingers, with some fissuring. There was irregular pitting of the right fifth fingernails (). The bilateral plantar feet demonstrated hyperkeratosis extending to the lateral surfaces of the feet and toes (). Xerotic scaling and excoriations were present on the bilateral upper arms, lower legs, and lower back. The xerosis was accentuated on the lower legs with fine, polygonal cracks ().\nShe was started on betamethasone dipropionate cream for her palms and soles, as well as triamcinolone 0.1% cream for pruritic lesions on the body. Additionally, generous emollients were recommended.\nPelvic external beam radiotherapy (EBRT), brachytherapy, and chemotherapy were initiated to treat her cervical SCC. EBRT was prescribed to 4500 cGy in 180 cGy fractions, followed by five high dose rate (HDR) brachytherapy treatments of 550 cGy to a modified point A of 1.9 cm completed in less than eight weeks, along with six cycles of cisplatin chemotherapy at 40 mg/m2.\nHer cutaneous manifestations mostly resolved throughout treatment; however, two weeks following completion, lesions were noted to have recurred on her bilateral hands and right foot. Along with the addition of urea cream 40% to her treatment regimen, recommendations were made to continue aggressive moisturization and the application of topical betamethasone dipropionate. Due to continued flaring of her disease, PET scan was performed to rule out persistence or recurrence of her cervical cancer. The PET scan was negative, indicating that her lesions persisted despite remission of her malignancy. Subsequently, her skin responded to therapy and completely resolved approximately two months after completion of chemoradiotherapy.
Acrokeratosis Paraneoplastica Associated with Cervical Squamous Cell Carcinoma
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A 51-year-old Caucasian man was transferred to our unit from a different hospital in November 2012. His medical history included occasional events of hypereosinophilia, recorded at 2021/mm3, over the previous 6 mo that were observed during periodic routine check-ups when the patient was noted as being completely asymptomatic. At 3 mo prior, the patient made complaint of fatigue and unintentional weight loss. At 2 mo prior, the patient made complaint of musculoskeletal pain and the treating physician noted new appearance of cutaneous infiltrated nodules; at this point, the patient was treated with symptomatic and local therapy. At 1 mo prior, the patient was admitted to a different hospital for severe dyspnea with elevated cardiac enzymes and echocardiographic signs of infiltrative myocarditis. Allergy, parasitology and hematology testing, including bone marrow biopsy, showed no evidence of allergic/atopic disorders, parasites, or blast cells absence (Figure ). The test for antineutrophil cytoplasmic antibodies (ANCA) was negative. Analysis of the bone marrow specimens showed a predominant eosinophilic component. Lymphocyte immunophenotyping showed normal T lymphocyte populations both in peripheral blood and bone marrow. Testing by fluorescence in situ hybridization (commonly known as FISH) for PDGFR rearrangement was negative. Further molecular analysis for rearrangements in BCR-ABL, PDGFRA, PDGFRB, WT1 and c-KIT were performed, and all results were negative. During this hospital stay, the patient developed hyposthenia of the left leg and right arm, which was recorded as associated with paresthesias and depressed level of consciousness. A brain computed tomography (CT) scan revealed multiple lesions suspected for vasculitis of the central nervous system (CNS).\nTransfer to our unit was requested. Examination upon arrival showed paresis of the left leg and right arm; the patient was also confused and complained of visual disturbances. Eosinophil count was 25.990/mm3. Magnetic resonance imaging (MRI) confirmed the suspicion of CNS vasculitis (Figure ). Re-test for ANCA produced the same negative result. High-dose methylprednisolone (1 g/d for 3 d), followed by medium-dose methylprednisolone (1 mg/kg per day i.v.) induced rapid improvement of the patient’s neurological condition. However, after 6 d of the medium-dose steroid therapy, the eosinophil count increased to 50.570/mm3. At the same time, hemoglobin dropped suddenly, from 10.9 g/dL to 6.7 g/dL. Abdominal CT indicated active bleeding in the retroperitoneum that was secondary to vasculitic involvement of the lumbar arteries; angiography and embolization was immediately performed and successfully resolved the bleeding. To control the severe hypereosinophilia, hydroxyurea was added to the (steroid) treatment regimen, but was stopped after a short course due to inefficacy. The option of interferon was precluded by the ongoing neurological involvement. Therefore, despite the negative results of the molecular test for PDGFR rearrangement, we decided to begin a regimen of low-dose imatinib mesilate (200 mg/d). We could not perform, to gain further proof of clonality, the G6PD analysis because the patient was a male. After 2 d of treatment, the patient’s eosinophil count normalized, to 100/mm3, and after 1 wk of treatment, the imatinib mesilate dose was reduced to 100 mg/d.\nThe patient recovered rapidly and was discharged after a total of 22 d hospitalization in our unit, with prescription for continuance of the imatinib mesilate. He continued to have mild visual disturbances and hyposthenia of left leg; the latter of which resolved nearly completely after 3 mo of physical therapy. At 2 mo after discharge, the dose of imatinib mesilate was reduced to 100 mg/twice a week, and at 3 mo after discharge to 100 mg/wk; the dosage then continued for the next year on a tapering-down schedule until the patient’s symptoms resolved completely with consistent normal eosinophil percentage. The lowest dose used was 100 mg every 10 d. At this point, we tried to stop therapy but observed a modest upward trend in the patient’s eosinophil count. Therefore, the imatinib mesilate was reintroduced at the same dose of the last assumption (100 mg every 10 d), and a subsequent normalization of eosinophil count occurred and was maintained thereafter. After 6 mo, we made another attempt to stop the therapy, and the result was better. The stopping of therapy occurred 6 mo prior to the writing of this report. We have a total period of ambulatory follow-up of 36 mo. Currently, the patient is completely asymptomatic, having full recovery of mobility of the involved limbs and no evidence of cardiac sequelae by multiple echocardiographic tests.
Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib
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A 43-year-old man with a history of dilated cardiomyopathy (functional class IV) who poorly responded to full-dose treatment for heart failure was admitted to Masih-Daneshvari Hospital for heart transplantation in August 2012. The mean pulmonary artery pressure (PAP) of the patient was 24 mmHg (peak PAP = 35 mmHg), and echocardiography showed an ejection fraction of 15%.\nThe donor was a previously healthy 22-year-old man who died of traumatic brain injury. The evaluation of the donor indicated a compatible blood group with the patient (both B+) with acceptable body surface area.\nThe transplantation was performed via orthotopic bicaval heart transplantation technique. A patent foramen ovale (PFO) was found in the heart of the donor in the operating room, which was closed up. The duration of clamping ischemia was 130 minutes. The heart transplantation procedure was performed properly with no unusual complication, and the patient was transferred to the intensive care unit (ICU) and was extubated after 2 days.\nTen days after heart transplantation, the patient complained of pain in the upper left limb, ague, and sweating. Initial assessment indicated that he was febrile with tachycardia. Examination of the extremities revealed coldness with decreased sensation in the left upper limb. Paraclinical assessments showed leukocytosis (white blood cell = 35000/mm3 and neutrophil = 87.4%). Doppler ultrasound of the left upper limb was performed to rule out arterial occlusion. In Doppler, an obstruction due to clot formation in the brachial artery, 2 cm away from the axillary fold, was confirmed.\nWith regard to the urgency of the condition, the patient was prepared for embolectomy of the brachial artery. A Fogarty arterial embolectomy catheter was utilized, and a clot and some tissue-like material were removed. The normal blood flow of the brachial artery was reestablished. After a few minutes, the pulses of the brachial and radial arteries disappeared once again and new arteriotomy and embolectomy were performed at the same place. Again, the same material was removed, and normal blood flow was returned. A day after the procedure, transesophageal echocardiography (TEE) was carried out and showed multiple hypoechoic and mobile masses in the left atrium (LA) above the left atrium auricle and within the right atrium (RA) just adjacent to the inferior venae cava ostium (cardiac suture lines). Moreover, a 2 × 2 cm loculation in the posterior aortic wall in the transverse pericardial sinus was seen. All of the valves were normal. Plain brain computed tomographic (CT) scan was performed to assess the probable presence of cerebral embolism and it had no apparent abnormality. In addition, chest spiral CT was conducted to investigate the possibility of pulmonary embolism and it demonstrated no active parenchymal infiltration and no pleural effusion.\nGiven the TEE result, the patient was prepared for emergent surgery to remove the heart masses. Both venae cavae and aorta were cannulated. Within an aortic clamping time of 25 minutes, the RA was opened and the masses on the suture line and near the inferior vena cava were removed. The tricuspid valve was normal. The septum was assessed for the presence of the PFO and it was completely closed. Then the septum was opened, and the mobile masses were removed at different points in the LA. The mitral valve was also normal. All spaces in the LA and RA were irrigated with saline. The septum was closed in two layers and air was drawn. Finally, TEE was performed and no lesion was seen. During the surgery because of coldness and disappearance of the radial pulse in the left upper limb, the Fogarty catheter was used in the previous site of arteriotomy once again and a large clot was removed from the brachial, radial, and ulnar arteries.\nThe patient was transferred to the ICU with a good hemodynamic condition. Frozen samples showed an exudate fluid with a great number of white blood cells, which favored infection. Culture of the samples was positive for Acinetobacter baumannii. After 7 days of observation in hospital and proper antibiotic regimen, the patient was discharged home with no additional complaints and normal physical examination. A 3-month follow-up showed normal good condition of the patient without the recurrence of the symptoms.
Limb Ischemia after Heart Transplantation: An Unusual Case of Tissue Embolism
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An 11-year-old female was referred to our center with multiple skin papules of 11 months duration. She was on no medications at the time. Past history was only significant for early puberty treated with gonadotropin releasing hormone agonists. Examination revealed widespread follicular papules which were located mainly on the elbows, knees, buttocks, and lower back (Figures -). Close examination (including evaluation with a dermatoscope) revealed multiple small papular lesions on an erythematous base measuring approximately 0.4 mm (Figure ).\nA 3-mm punch biopsy was performed and showed mucinous degeneration of the hair follicles and perifollicular chronic inflammation with no atypical cytological features. The upper dermis showed slight edema with minimal lymphocytic infiltrates. The overlying epidermis showed no significant abnormalities with no obvious spongiosis or hyperkeratosis (Figures -). Other routine laboratory investigations were essentially normal.\nThe diagnosis of FM was established. This young woman was managed with topical steroids, phototherapy (natural sunlight), hydroxychloroquine, and emollients for symptomatic relief. At 4 months followup, there was progressive improvement of symptoms with only mild residual papules of the elbows and buttocks.
Follicular Mucinosis: A Case Report
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We report a case of a 64-year-old Caucasian man with a past medical history of gout, dyslipidemia, hypertension, obesity, gastroesophageal reflux disease and in previous left hemicolectomy for colon cancer nine years prior to presentation. His past surgical history includes two laparoscopic hernia repairs for incisional hernias after the hemicolectomy. He was referred to the surgery service for new diagnosis of myasthenia gravis and the consideration for possible thymectomy. The thymus was without evidence of thymoma, however, the work-up included radiographic studies demonstrating a centrally located splenic mass measuring 8 × 6.8 cm. The density of the mass was consistent with those found in soft tissue tumors (Fig. ). A comparison CT study from 9 years previous demonstrated a smaller mass (4.7 cm) in the same anatomic location with similar density levels. There had been no prior treatments. The patient's case was presented at our multi-specialty tumor board and elective resection of the spleen was recommended due to the possibility of malignancy.\nExploratory laparoscopy was performed revealing significant adhesions necessitating conversion to laparotomy. An uncomplicated splenectomy was performed. Before surgery the patient was vaccinated against (Streptococcus pneumonia, Haemophilus influenza and Neisseria meningitides). Grossly, a 380-g spleen measuring 15 × 13 × 7 cm with intact splenic capsule was removed. Serial sections reveal a yellow-tan to red-tan, well-circumscribed tumor mass, measuring 10 × 10 × 7 cm. The tumor was grossly surrounded by a thin rim of dark red splenic parenchyma with splenic capsule. All sections showed a well-circumscribed area of myelolipoma completely contained within the splenic capsule (Fig. ). The tumor consisted mainly of adipose tissue with multifocal areas of non-dysplastic hematopoietic tissue (confirmed with immunohistochemical assay positive for glycophorin and Factor VIII demonstrating erythroid elements and megakaryocyte presence). Containment within the splenic capsule was microscopically confirmed. These findings confirmed the diagnosis of intrasplenic non-neoplastic myelolipoma.
Myelolipoma in the spleen: a rare discovery of extra-adrenal hematopoietic tissue
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A 79-year-old woman with bilateral lower extremity weakness due to cervical myelopathy presented at our department in 2002 after multiple reconstructive procedures in both hips for developmental dysplasia of the hip. In 1993, a bulk allograft in combination with an acetabular cage and a cemented cup were used to treat the left massive acetabular bone loss. The defect was type IVb by the classification of the American Academy of Orthopaedic Surgeons () and . In 2000, the acetabular construct failed mechanically while the existing cemented femoral stem remained well fixed (Figure ). Removal of the acetabular hardware was followed by implantation of a whole acetabular allograft. The allograft was stabilized with plates and screws, and a new cemented cup was inserted. 2 years later, allograft fracture and acetabular failure occurred again.\nIn 2002, a high hip center bipolar hemiarthroplasty was performed via a standard posterior hip approach. Failed acetabular component and hardware were removed but the femoral stem was left in situ as it was found to be stable. Capsular and periacetabular scar tissues were preserved as much as possible to create a soft tissue cavity to seat the bipolar head into. A 60-mm bipolar femoral head was inserted onto the femoral component to articulate with the periacetabular soft tissues in a high hip center mode. Its position was further augmented with capsular repair around the neck of the prosthesis (capsular noose). A femoral condyle allograft was fixed to the ilium to serve as posterior superior acetabular wall. Postoperatively, the patient was advised to gradually increase her weight bearing using a walker or crutches. No casts or braces were applied. Within 6 months, the bipolar component migrated out of the acetabulum and articulated with the iliac soft tissues (Figure ). Although the patient had limb shortening, she had no pain and declined further surgery.\nThe right hip required 7 reconstructive procedures, which led to pelvic discontinuity (type IVb) and resection arthroplasty in 1999 (Figure ). In 2000, a re-implantation was performed by using a reinforcement ring with a cemented polyethylene cup and a long cemented femoral prosthesis. 4 years later the acetabular construct failed. A 56-mm diameter bipolar head was inserted onto the previously implanted and stable femoral stem and articulated with the soft tissues adjacent to the lateral ilium (Figure ). After surgery, the patient was able to transfer independently and ambulate short distances in her home with a walker.\nAt 3 years postoperatively (right hip) and 5 years postoperatively (left hip) the patient had no pain, relatively equal leg lengths, and could sit comfortably. Due to complete loss of lower extremity motor function associated with failed spine surgery and cervical myelopathy, the patient was non-ambulatory. However, the Harris hip score (HHS) of the patient’s left hip had improved from 39 preoperatively to 58 postoperatively. Similarly, the HHS of the patient’s right hip increased from 14 preoperatively to 58 postoperatively.
High hip center bipolar hemiarthroplasty for non-reconstructable pelvic discontinuity
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A previously healthy 6-year-old boy visited Samsung Medical Center with complaints of dyspnea and a barking cough lasting for 3 days. Chest radiography revealed a mass-like lesion in the right upper mediastinum. Chest computed tomography demonstrated a well-defined posterior mediastinal mass measuring 4 cm, which was suspected to be a neural foraminal extension to the thoracic spine (T3–T4). Magnetic resonance imaging was conducted for further evaluation; it showed a right upper paravertebral enhancing mass with an adjacent neural foraminal extension to the T2–T5 spine (). A preoperative neurologic examination revealed that the patient had no abnormalities. He underwent mediastinal mass excision by video-assisted thoracic surgery. The mass was in the right posterior mediastinum, was located at the T2–T5 level of the spine, and did not extend to the neural foramen. It was resected by electrocautery. The sympathetic chain at the T2 level was inevitably resected as part of the complete resection of the mediastinal tumor during the operation, because the tumor originated from this sympathetic chain. The intraoperative course was uneventful, with a stable hemodynamic status throughout the surgery. After the patient was sent to the post-anesthesia recovery unit, a sharp midline facial demarcation was observed, and the left face, neck, and chest (contralateral to the operation site) became flushed and warm (). These findings were noted to increase in intensity when the patient cried. In contrast, the right face, neck, and chest (ipsilateral to the operation site) were pale and cool and did not change in color when the patient cried. A neurologic examination including that of the cranial nerves was normal, and miosis and ptosis were absent. The patient’s vital signs and laboratory tests were normal. The symptoms, including the color and the warmness of the face, started to show minimal improvement within an hour and completely resolved without any treatment 3 hours after surgery. The patient was discharged on postoperative day 2. The permanent pathologic findings revealed that the resected tumor was a ganglioneuroma. During follow-up on postoperative day 30, there was no sign of color change on his face, neck, or chest.
Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma
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A 39-year-old pregnant Caucasian woman presented to the Obstetric Emergency Room reporting vertigo, vomiting, nystagmus, dizziness, and hindrance in the execution of fine movements of the right arm. The maternal parameters on admission are regular: pulse 98 beats per minute, pressure 110/68 mmHg, and temperature of 36.2 °C. She had an obstetric history of a first-trimester spontaneous abortion and a medical history of tension headache. She is married and graduated. She has a high socioeconomic status and is employed as an engineer.\nThe ongoing pregnancy coursed physiologically until that moment. The fetus was screened for aneuploidy with a noninvasive prenatal test (NIPT), while second- and third-trimester ultrasounds for the study of malformations were both normal. The oral glucose tolerance test at 24 weeks was negative. At 38 + 4-week gestation, the patient was hospitalized due to suspected vestibular neuritis diagnosed by an otolaryngology (ORL) specialist. Following diagnosis, the patient started therapy with corticosteroids, including prednisone 5 mg two times per day and levosulpiride 25 mg two times per day, that continued for 3 days.\nOn day 2, the patient developed diplopia and worsening of vomiting and dizziness, with improvement of symptoms in left lateral decubitus. The ORL revaluation excluded peripheral vestibulopathy and progressed to an urgent brain computed tomography (CT) scan without contrast that excluded ischemic or hemorrhagic brain lesions.\nOn day 3, due to further worsening of symptoms, urgent neurological counseling was performed. Viral examinations of herpes simplex I and II, herpes zoster, and herpes virus VI were negative. The neurologic examination showed the left eye adducted and elevated, vertical diplopia, and presence of rotatory nystagmus accentuated on the right gaze and dyssynergia in the cerebellar maneuvers of the right upper limb. After a detailed medical history, the patient stated for the first time that she had undergone cervical spine manipulations by an osteopathic specialist in the days preceding the beginning of the symptoms. Magnetic resonance imaging (MRI) of the brain was urgently performed, showing a punctate lesion hyperintense on diffusion-weighted imaging (DWI) (Fig. A–B), characterized by a reduction of apparent diffusion coefficient (ADC) on the colorimetric maps. This finding was suggestive of ischemia in the posterolateral right medulla oblongata, which is consistent with the symptoms of Wallenberg syndrome, although the patient did not have the full spectrum of symptoms. On MR angiography, the intracranial V4 segment was normal, but the right posteroinferior cerebellar artery (PICA) was not present (Fig. E). Urgent thrombolytic therapy or emergency revascularization was not deemed necessary by the neurologist colleague. Acetylsalicylic acid (ASA), 100 mg, therapy was then prescribed. Subsequently, echo-color Doppler ultrasound of the supra-aortic trunk detected no alterations of the cervical vessels, and transthoracic echocardiogram with exclusion of patency of the foramen ovale was performed. During this observation period, the patient was continuously monitored with noninvasive blood pressure monitor and pulse oximeter to detect hypotensive state and/or desaturation episodes early. Considering the clinical condition of the patient, the term gestational age, and initial onset of prodromal contractions, an elective cesarean section (CS) under subarachnoid anesthesia was performed, given the inability of the patient to deliver vaginally because of the ischemic cerebral event and obligatory left lateral decubitus, diplopia, and dizziness. The intraoperative and postoperative courses were uneventful. Six hours after the CS, prophylaxis with low-molecular-weight heparin was prescribed.\nThe neonate showed good adaptation to postnatal life with an Apgar score of 9 at 1′, 10 at 5′, and 10 at 10′; blood gas analysis was regular both in the artery and vein. Normal anthropometric parameters were present: 3250 g (52nd centile), length of 49 cm, and cranial circumference of 36 cm.\nOn day 4, for better study suspected dissection on small vertebral vessels, the patient underwent CT angiography of the neck, which showed a focal dissection at the V2 distal segment of the right vertebral artery (Fig. C–D). The puerperium course was normal, and the midwife helped the patient during breastfeeding because of the difficulty of standing up and walking due to the diplopic symptom. Psychological support was offered during the hospitalization, with daily physiotherapy rehabilitation and orthoptic evaluation. Congenital and acquired thrombophilia tested negative. After 12 days of rehabilitation, the patient was discharged with continued complaints of diplopia and a walker for mobility. After the VAD diagnosis and for the entire length of hospitalization, the patient was treated with Cardioaspirin 100 mg/day and prophylactic enoxaparin 4000 UI/day subcutaneous injection for 60 days. At the subsequent neurological evaluation, during the follow-up of 2 and 4 months, the patient showed persistence of vertical diplopia and a circumspect and wide gait, and life-long ASA was prescribed. A follow-up MRI was scheduled for 6 months after the stroke, which confirmed the signs of the previous ischemic lesion on the posterolateral right medulla oblongata. The remaining findings are unchanged.\nEthical approval was obtained, and the patient gave written informed consent to publish this case and any accompanying images.
Vertebral artery dissection in term pregnancy after cervical spine manipulation: a case report and review the literature
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We present a case of a seventy-three-year-old Saudi man who has started visiting the primary health care center in our institution twenty-five years ago. He has been concerned with having a cancer that would give him only few days to live. At the beginning, the patient was evaluated medically through detailed history and documentation of his symptoms and then a management plan was created accordingly to exclude cancer. Full history, physical examinations, and radiological and pathological investigations were ordered and the results were all negative for cancer. The physician explained the results of the investigations to the patient but he refused them and continued to insist that he had cancer regardless of the results. The patient was then referred to Psychiatry Department to be evaluated but he could not realize that his symptoms might be of a nonorganic cause, either psychological or mental.\nThe patient continued to visit the general hospital, emergency department, and the primary health care in the institution and was still occupied with the idea of cancer presence. Although the patient was seen by many physicians, the patient was always not satisfied with their conclusions. Eventually a physician reported the case to the department of medical eligibility addressing the issue of continuous primary health care center visits with very variable symptoms, nonconclusive diagnosis, and an unconvinced patient. The department of medical eligibility in the hospital administration took a decision to temporarily limit the patient's file to the psychiatry department to drive the patient to visit the psychiatrist to be evaluated psychologically in order to make his file eligible again. The patient was unhappy at the beginning but he had to visit the psychiatrist. So, an appointment with the psychiatrist was booked and a full psychological and social evaluation was performed by taking a thorough history from the patient. This revealed that the patient fit the criteria of the illness anxiety disorder in which he had a minimum of six months of a persistent belief of having a serious disease which he specifically named. Moreover, this persistent occupation with this belief was disabling and limiting him from having a normal life and thought that his days were counted in this life. Also, a persistent refusal of any medical advice or explanation for his symptoms and fears was neither related to schizophrenia nor related to mood disorders.\nA diagnosis of illness anxiety disorder was made despite the patient's strong refusal. The family and social history evaluation also revealed a very low socioeconomic status and similar conditions in the family. Interestingly, two of the patient's daughters had similar reported conditions which could raise the suspicion of the possible genetic predisposition that could be triggered by shared environmental factors between him and his daughters. Following this extensive detailed history and evaluation, the treating psychiatrist contacted the department of medical eligibility to make the patient's file eligible again as promised by them with an unlimited eligibility to all specialties and primary care center. For the past twenty-five years of continuous hospital visiting, he has had almost weekly and even daily visits to the primary care. The patient kept complaining of variable symptoms every week and specifically asking for certain lab tests and radiological studies. Also, he successfully convinced some physicians to order a biopsy for him.\nIt is reported that physicians in the primary health care center used different techniques of counseling and they applied the biopsychosocial model to overcome the patient's fears but often failed to reach an achievement as the patient continued to complain of different symptoms and became a burden on the physician's clinic time and the hospital resources. It is also reported that the patient was extremely difficult to handle and convince. In the absence of institution guidelines to deal with such cases, all physicians tended to yield to the patient's persistent complaints and accepted his demands and fulfilled his desires by requesting whatever investigations the patient asked for. Over the years, the patient's investigations have cost approximately $178200 due to the system negligence to such cases. The patient's most requested investigations were mainly invasive radiological studies specifically abdomen and pelvis CT (18 times) and wide range of other invasive and noninvasive studies like chest CT (11 times), brain CT (7 times), chest MRI (4 times), lumbar spine MRI (6 times), and other studies. It is important here to put emphasis on the potential harms of radiation from radiological studies that are frequently requested by the patient in the absence of clear guidelines to be followed by practitioners for that group of patients. In addition, the patient asked for a variety of lab tests including renal profile, parathyroid hormone, complete blood count, estimated glomerular filtration rate, coronary risk profile, 25-hydroxy vitamin D, thyroid stimulating hormone, prostate specific antigen, free T4 level, and other very wide range of lab tests. Moreover, the patient underwent several biopsies like renal biopsy, prostate biopsy, gastric biopsy, and other different types of biopsies.\nThe patient was abusing the system in which resources and time were wasted. The patient kept overstepping each barrier by the hospital's staff to minimize his burden on the hospital by manipulating staff and deceiving and misleading them by giving incorrect information. Also, he was targeting junior practitioners and disturbing them and even threatened to sue the administration if restricted from health care. Five years ago, an experienced board-certified family physician was shocked when he looked at the patient's file and decided to put an effort to solve this problem by taking some actions. First action considered, after getting the approval from the family medicine department, was to limit the patient's primary care visits to only one clinic and to refer him to the supervising physician himself. Then, building strong and effective patient rapport based on trust and honesty was initiated. After negotiations, the patient agreed to enroll in cognitive behavioral therapy (CBT) for six sessions ninety minutes each. The patient was advised to have a scheduled sleeping time, ensure healthy eating habits with regular physical exercise, be involved in social activities, stay away from stressors like searching web for symptoms, and avoid TV health shows and health magazines during active cycles of disorder. After that, there was an agreement with the patient not to disturb the clinic and he would get to see the physician regularly for checkups every three months.\nAfter five years of implementing the new rules, the patient is still visiting the clinic for regular follow-up and sometimes he breaks the rules and comes regularly to the clinic especially in active cycles of the disorder. On following the patient's condition in the last five years, it is noticed that there is a dramatic decrease in the total financial cost due to the relative decreasing number of visits, less time spent on arguing with the patient, and the filtration of his requests. Moreover, there is a remarkable improvement in the patient's condition because of CBT and relative compliance to health advice. We think that this rare case of extremely persisting illness anxiety is worth reporting because the patient managed somehow to escape attention and made a huge financial burden on hospital resources in the absence of clear guidelines in such conditions. Also, this case provides useful insight for future guidelines development.
A Recommendation for the Management of Illness Anxiety Disorder Patients Abusing the Health Care System
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A 77-year-old male presented to the urology clinic in August 2011 with voiding symptoms and a known history of nephrolithiasis. A CT of the abdomen and pelvis demonstrated bilateral extensive nephrolithiasis with a large left staghorn renal calculus, 3 cm bladder stone, and a markedly enlarged prostate. The patient was recommended surgery for the bladder and renal stones although was lost to follow-up.\nHe re-presented 22 months later and repeat noncontrast CT abdomen and pelvis revealed stable appearance of his large left staghorn stone, right kidney stones, and bladder stone (). New diffuse bladder wall thickening was also noted. Transurethral resection of the prostate and cystolitholapaxy were performed along with bladder biopsy given CT findings. Prostate pathology demonstrated focal squamous metaplasia and the bladder showed atypical squamous proliferation with areas of necrosis—both consistent with chronic inflammation.\nHe subsequently underwent left PCNL with removal of the staghorn calculus. However, an incidental renal pelvis mass was encountered. The mass was biopsied and resected using a bipolar resectoscope. The immediate postoperative course was complicated by acute blood loss anemia requiring blood transfusions and angioembolization of the left kidney. Pathology ultimately revealed multifocal, invasive poorly differentiated SCC of the left renal pelvis with spindle cell features. Renal parenchyma was seen without carcinoma invasion.\nA restaging CT abdomen and pelvis demonstrated a pseudoaneurysm of the left kidney, residual renal pelvis tumor, as well as significant bladder wall thickness concerning for tumor (). There was no evidence of lymph node involvement or metastatic disease.\nCystoscopic evaluation of the bladder revealed diffuse tumor. Tumor resection pathology revealed high grade SCC invading the lamina propria without muscle invasion.\nAn attempted robotic left nephroureterectomy with cystoprostatectomy and urinary diversion was performed ∼3 months following PCNL. The delay in definitive management was attributed to the need for further staging and medical optimization for major surgery. The intraoperative course was complicated by a difficult dissection and hemodynamic instability following nephroureterectomy. Unfortunately, the procedure was aborted before cystectomy and lymph node dissection.\nThe left kidney and ureter pathology revealed pT4NX disease for invasion into the ipsilateral adrenal gland. The invasive SCC demonstrated sarcomatoid features. Also noted was focal intestinal metaplasia of the renal pelvic epithelium.\nHis postoperative course was complicated by acute blood loss anemia and severe sepsis. A CT of the abdomen and pelvis (3 months following last staging CT) demonstrated increased lymphadenopathy, and new lesions throughout the liver with peritoneal carcinomatosis consistent with metastatic disease (). The patient expired 21 days following surgery.
Unsuspected Malignancy During Percutaneous Nephrolithotomy: The Snake in the Grass
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A 28-year-old female presented because of brown pigmentation and sclerosis of the right lower limb. She had a past medical history of Caesarean sections (at 22 and 24 years), without particular familial medical history. The history of current illness was as follows: She noticed brown pigmentation of the right thigh in 2008. It gradually expanded to her right lower back, but there was no tenderness, spontaneous pain or pruritus. The patient visited a dermatologist and was administered a vitamin C preparation, but it was ineffective for treating the pigmented lesion. Since sclerosis developed in the pigmented region along the course, the patient visited our clinic in September 2011. Upon first examination, brown pigmentation with a slightly clear boundary was present on the right thigh and crus (fig. ). No general symptoms, such as Raynaud's phenomenon, were noted.\nHer serum IgG level was 1,764 mg/dl, and antinuclear antibody titers (FA method) were 160 times, which was slightly elevated, but no specific autoantibody was detected, and no other abnormal findings were noted. For histopathological examination, biopsy specimens were collected from two sites: (1) In the sclerotic region (fig. ), flattened epidermis and increased collagen fibers directly below the epidermis over the deep dermis were noted, and appendages, such as sweat glands, had been lost. (2) In the pigmented region (fig. ), the sweat glands and adipose tissue had been excluded by increased collagen fibers. Under high magnification, many infiltrating inflammatory cells were noted around sweat glands, vessels, and adipose tissue in the deep dermis. On Fontana-Masson staining, melanin deposits were noted throughout the epidermal layer in the pigmented region.\nBased on clinical and histopathological findings, the patient was diagnosed with localized scleroderma. Oral treatment with etretinate 20 mg/day was initiated. The reasons for etretinate selection were that she did not want more children and that acitretin or isotretinoin are not approved in Japan. The skin sclerosis started to improve 28 days after treatment initiation. Oral treatment was continued for about 5 months, and the skin sclerosis and pigmentation improved. Topical corticosteroids were not administered during this period, and no adverse events, such as pruritus, skin thinning, gastroenteritis, paronychia or angular cheilitis, developed. 15 months after the discontinuation of etretinate treatment, although the brown pigmentation remained, improvements in skin eruption were maintained compared to those upon first examination (fig. ).\nBiopsy results upon first examination, after 4 months of oral treatment and 9 months after discontinuation of oral treatment were compared (fig. ). Under low magnification, epidermal thickening was noted after 4 months of oral treatment and 9 months after treatment discontinuation, and sweat glands were present in the dermis. In addition, collagen fibers in the dermis had become bulky and dense (fig. ). Under high magnification, infiltrating inflammatory cells around vessels and in lobular fat in the deep dermis had decreased and atrophied appendages had recovered.
A Patient with Localized Scleroderma Successfully Treated with Etretinate
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A 65-year-old woman was transferred to our department for PEG insertion during nasogastric tube feeding because of dysphagia caused by ICH. She was taking medication for hypertension and osteoporosis for the last 7 years. She was diagnosed with SIT during a colonoscopy performed 2 years prior; she has no family history of this condition. At the time of transfer, she was maintaining a blood pressure of 130/80 mm Hg, heart rate of 65 to 80 beats per minute, body temperature of 36.5℃, and respiratory rate of 18 breaths per minute with alert consciousness. Her heart rate was normal without heart murmur. No difference was found from the previous examination of the chest, and no abnormal findings were detected in the abdomen, limbs, and skin. Analysis of peripheral blood showed a white blood cell count, platelet count, and hemoglobin of 5,170/mm3, 9.7 g/dL, and 290,000/mm3, respectively.\nThe liver function test results were as follows: aspartate aminotransferase/alanine aminotransferase, 13/9 IU/L; prothrombin time-international normalized ratio, 1.08; and partial thromboplastin time, 32.4 seconds (reference range, 20 to 38). The renal function tests were within the reference ranges. Dextrocardia was observed on a chest radiograph (), and complete right to left reversal of internal organs was detected on abdominal computed tomography (CT) scan, indicating a finding of SIT (). No abnormal findings were found on the CT scans of the spleen and major blood vessels. Upper gastrointestinal endoscopy was conducted identical to a general endoscopic procedure, with the patient in a left lateral decubitus position. Along with preoperative monitoring of pulse rate and oxygen saturation, midazolam (3 mg) and propofol (50 mg) were administered via intravenous injection as a pretreatment. The endoscope was turned counterclockwise when it was passed from the bulb to the descending part of the duodenum. The gastric angle, when observed with a J shaped retroflection at the antrum, was mirrored from its normal position. No specific findings were detected during the endoscopy except for a mild chronic superficial gastritis. A puncture site was chosen with the help of an endoscopic light after manually pressing the stomach wall to find the thinnest site. Subsequently, a gastrostomy feeding tube (PEG-24-Pull; Wilson-Cook Medical GI Endoscopy, Winston-Salem, NC, USA) was inserted into the anterior wall of the lower gastric body, which looks like the posterior wall of the lower gastric body in a normal person (). The nutrition was delivered directly into the stomach the next morning after PEG insertion, and no specific complications were observed.
Gastrostomy in a Patient with Situs Inversus Totalis
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An 82-year-old man (height, 167 cm; weight, 58 kg) was scheduled for mitral valve repair due to exacerbated heart failure symptoms. His past medical history included aortic valvular stenosis with post-aortic mechanical valve replacement and atrial fibrillation treated with oral warfarin. The patient had no upper gastrointestinal symptoms or related medical history. Written informed consent was obtained from the patient.\nFour days before mitral repair surgery, warfarin was replaced with intravenous heparin (10,000 units/day), which was administered until 6 h preoperatively. Under general anesthesia, a TEE probe was inserted, without resistance, to evaluate perioperative cardiac function and residual regurgitation using mid-esophageal and trans-gastric views, as appropriate. The inferior vena cava (IVC) could not be visualized in trans-gastric view, despite several manipulations of the probe with the venous cannula placed into the IVC. After cardiopulmonary bypass (CPB), an unexpectedly large echo-free space in the stomach was detected on TEE. The mitral valve repair was successfully undergone, the TEE probe was removed, and a nasogastric tube was inserted. Surprisingly, 700 mL of dark brown blood was aspirated from the nasogastric tube. We suspected upper gastrointestinal injury, but the patient was transferred to the intensive care unit, as the blood was dark brown and the bleeding appeared to have stopped.\nApproximately 6 h postoperatively, another 100 mL of fresh blood was aspirated from the nasogastric tube. Thus, we immediately performed an esophagogastroduodenoscopy (EGD) examination and computed tomography (CT) scan. The EGD examination showed a mucosal laceration spanning from the esophagogastric junction to the gastric body, with a perforation in the center of the laceration (Fig. ). On this occasion, a clip was placed to stop the hemorrhage from the injured area, but the perforation was too large to close with the clip under EGD. The CT scan showed localized free air in the abdominal cavity, which helped establish a definite diagnosis of gastric perforation (Fig. ). Initially, surgical treatment was considered for the repair of the perforation. However, we selected conservative management to avoid increasing operative stress, to minimize the need for total gastrectomy (including the lower esophagus), and to reduce the risk of infection spread. Fortunately, spontaneous closure was expected, as there were no abdominal symptoms and the perforation site was covered with the lesser omentum and inferior surface of the liver.\nNasogastric tube aspiration was continued postoperatively, and the patient was administered total parenteral nutrition. On the day of surgery, the patient received hemostatic therapy with carbazochrome sodium sulfonate hydrate (100 mg) and tranexamic acid (0.5 g), as well as routine antibiotics including sulbactam sodium (0.5 g) and ampicillin sodium (1.0 g). Tazobactam sodium (0.5 g) and piperacillin sodium (4.0 g) were initiated after diagnosis of gastric perforation and continued until the perforation site had healed. Intravenous omeprazole sodium (20 mg) was used as a proton pump inhibitor until oral intake. On postoperative day 1, hemostatic therapy included carbazochrome sodium sulfonate hydrate (200 mg), tranexamic acid (1.5 g), and menatetrenone (20 mg). As there were no persistent abdominal symptoms and the nasogastric aspirate contained no blood, the tracheal tube was removed on postoperative day 3. A follow-up CT scan revealed a reduction in the echo-free space in the abdominal cavity, and upper gastrointestinal examination revealed no leaking of the contrast medium into the abdominal cavity. Thus, the patient began enteral nutrition on postoperative day 10 and oral intake on postoperative day 12. To prevent rebleeding, anticoagulation was restarted only after enteral nutrition was established (warfarin, 3 mg). To protect the gastric mucosa, oral esomeprazole (20 mg) and ecabet sodium (2000 mg) were started upon establishment of oral intake and continued until after discharge. A follow-up EGD examination on postoperative day 23 revealed that the perforation site had healed, with scarring (Fig. ). The patient recovered without any complications and was discharged from the hospital on postoperative day 24.
Conservative management for iatrogenic gastric perforation by transesophageal echocardiography
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A 66-year-old Caucasian male presented with a palpable, painless slowly growing mass in the left side of the neck. He denied any local or systemic symptoms of thyroid dysfunction or neck compression. He did not report any history of radiation to the neck. His past medical history was remarkable for hypertension, hypercholesterolemia, and benign vocal cord polyps. He was taking verapamil, hydrochlorothiazide, atorvastatin and aspirin. He admitted to smoking 2 packs of cigarettes a day for 40 years and was a habitual alcohol user. Clinical examination revealed a firm, palpable, nontender nodule in the left lobe of the thyroid gland measuring 2.5 cm in diameter. The nodule was mobile upon swallowing. No palpable cervical nodes were appreciated. The rest of physical exam was unremarkable.\nA thyroid ultrasound demonstrated multiple thyroid nodules within both thyroid lobes. The largest was in the left lobe, measuring 2.5 x 2.1 x 1.1 cm, and was hypoechoic and heterogeneous in nature. Fine-needle aspiration cytology was consistent with papillary thyroid carcinoma. Thyroid function tests at that time were normal.\nThe patient underwent an uncomplicated total thyroidectomy. The surgical and postoperative courses were unremarkable. Pathological examination confirmed a multifocal papillary thyroid carcinoma, involving both lobes and the isthmus. The surgical margins were negative. One anterior cervical (Delphian) lymph node was positive for metastatic disease (stage pT3N1a, stage III). A focus of metastatic adenocarcinoma was identified in the right lobe, characterized by glandular formation with central necrosis and hyperchromatic malignant cells. The histological and immunophenotypical features of this second tumor were consistent with adenocarcinoma of the colon ().\nAfter this finding, the patient underwent a colonoscopy which revealed a mass in the descending colon consistent with adenocarcinoma. A Computed Tomography (CT) scan of the abdomen and pelvis revealed an 8 cm mass in the upper pole of the right kidney consistent with renal cell carcinoma. The patient underwent a left hemicolectomy, and a right nephrectomy. During this surgery a solitary 1.5 cm metastasis in the left lobe of the liver (segment III), was identified and resected. Pathologic examination confirmed the diagnosis of a colonic adenocarcinoma without nodal metastasis in the 31 nodes isolated (pT4N0M1), liver metastasis having a colonic origin with negative margins, and renal cell carcinoma (pT2N0).\nAfter surgery the patient underwent radioactive iodine treatment and chemotherapy. He was disease-free for three years. After three years, the patients Carcinoembrionic Antigen (CEA) levels increased to 76.1 ng/ml (upper limit 5 ng/ml), with concomitant normal thyroglobulin levels. A CT scan of the abdomen and pelvis revealed a 3 cm mass in the left lobe of the liver. A Positron emission tomography (PET) scan confirmed a single area of abnormal tracer accumulation in the left lobe of the liver. He underwent an uneventful left hepatectomy. The lesion contained histopathologically proven metastatic colonic carcinoma with negative margins.
Metastatic Colon Cancer to the Thyroid Gland in the Setting of Pathologically Diagnosed Papillary Thyroid Cancer: A Review and Report of a Case
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A 35-year-old male with a history of AICD placement for long QT syndrome by a local cardiologist six months ago presented to the ED by ambulance for altered mental status and frequent shocks from AICD secondary to a loperamide overdose. He was unable to provide a history of more than “yes" or "no” because of his agitation, so the most history was obtained from emergency medical services (EMS), with confirmation by the patient. EMS stated that the patient was having dinner with his spouse when the spouse noticed that he had a brief loss of consciousness for several seconds. The spouse stated that when the patient awoke, he became very anxious, diaphoretic, and irritable; it was unclear if the defibrillator had fired at that time. On arrival, EMS gave the patient 2 mg of naloxone IV without any effect. They stated that en route to the facility, they noted that the AICD had fired several times, causing the patient much anxiety and pain. EMS also stated that they found a bag full of loperamide tablets inside a purse-string bag near the patient, which they brought into the department for identification. They stated that the patient had a history of IV drug abuse and had overdosed on loperamide in the past when he had been unable to obtain opiate medications. In the ED, the patient was anxious and diaphoretic and was shocked by his defibrillator every three to four minutes for runs of polymorphic ventricular tachycardia (Torsades). A review of symptoms was negative, except for tachycardia, anxiety, and diaphoresis. Initial vital signs were a heart rate of 120, respiratory rate of 24, oxygen saturation 99% on room air, and blood pressure 138/64. Physical exam showed that the patient could not hold an extended conversation; he was anxious, had reactive mydriasis, and an AICD in place in the left chest, without signs of infection; otherwise, the physical was normal.\nPotassium was found to be 2.7 so IV potassium, magnesium, bicarbonate, and saline were started with lidocaine chosen as an antiarrhythmic (with which cardiology and poison control had agreed). The remaining basic labs, as well as toxicology and cardiac enzymes, were normal. Lorazepam was also used to control agitation. The electrocardiogram (ECG) showed tachycardia, which was different from the patient’s baseline atrial-paced rhythm. This ECG (Figure ) was obtained when the patient was not in polymorphic ventricular tachycardia, which was only shown on the monitor for several seconds before being detected and electrically cardioverted by the AICD. Chest X-ray was normal. After treatment in the ED and intensive care unit (ICU), the shocks eventually became less frequent as electrolytes normalized. The patient was eventually removed from the lidocaine drip and discharged home five days later with the normalization of ECG over time (Figures -).
Loperamide Overdose
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GAA, a 35-year-old woman with 10 years of schooling, dextral, and retired packer,\nsuffered an ischemic cerebrovascular accident (CVA) in the region of the middle\ncerebral artery () in 1995.\nMagnetic resonance imaging (MRI), (24/10/2001) revealed lesions in cortical and\nsubcortical areas of language (). On\nSingle Photon Emission Computed Tomography (SPECT) (31/10/2001) (), there was a large perfusion deficit in\nthe left hemisphere (). Besides global\naphasia syndrome, diagnosed in the acute phase and which evolved to Broca's aphasia\nwith severe agrammatism, GAA had persistent right hemiplegia, predominantly in the\nupper limb. Ten years had elapsed between the CVA and the reported therapeutic\nintervention.\nProcedures. Before training production of sentences with the\nSPPA, GAA was evaluated by\nthe Boston Diagnostic Aphasia Examination-Short Form (BDAE-SF) which includes\nsub-tests of oral comprehension of words, sentences and texts, oral confrontation\nnaming, repetition, production of descriptive (The Cookie Theft scene) and written\nlanguage. These data were\nregistered on videos. An additional description of the Cookie Theft scene was used\nas an independent measure to analyze generalization and transfer of learning.\nSeverity of aphasia was rated by the Boston Aphasia Severity Rating Scale, a measure\nused as a summary of sub-tests. Previously, she was submitted to a conventional\nmultimodal stimulation therapy\nto elicit language production through repetition, phonemic cueing, reading in a\nvariety of linguistic and situational contexts, and consequently improved repetition\nof short phrases and global communication (initiative to introduction, maintenance\nand diversity of topics, increasing of partners of conversation).\nOn the SPPA, eight types of sentence were trained. Each type contained 15 stimuli\n(total of 120 figures of scenes+ target sentences). Each type of sentence was\npresented at two levels of difficulty, as prescribed by the method: 1) Level A - the\ntarget sentence was presented simultaneously with an action scene, depicting its use\nin context, for repetition after the speech therapist had presented the story. 2)\nLevel B - the story had to be complemented with the target sentence, without the\nbenefit of repetition. (See examples online - Figure 3).\nThe sentences of each type were presented in blocks. When GAA answered correctly at\nlevel A (facilitation by repetition), the stimulus level B (confrontation of scene\nfor spontaneous emission of the target sentence) was immediately presented. The SPPA\nwas planned for 32 sessions distributed in weekly sessions of 30 minutes (about four\nsessions per sentence).\nPerformance was scored according to the following criteria: 1 point for correct\nanswers (including successful self-correction, after initial error); 0.5 for\npartially correct answers (only one word was omitted or mistakenly produced,\ncompromising both the syntax and the meaning of the sentence); 0 for incorrect\nresponses in which two or more words were omitted or incorrectly produced, or if the\ntarget sentence contained only one word and it was not produced correctly.\nSituations when GAA failed at level A and where consequently level B was not\napplied, were considered "not applicable" (NA). The criterion for the continuation\nof the blocks was a score of at least 13 out of 15 items (85% correct).
Sentence production in rehabilitation of agrammatism: A case\nstudy
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This 39-year-old Caucasian woman presented with complaint of floaters. She had had laser in situ keratomileusis (LASIK) surgery for high myopia in India. She had had bilateral BrightOcular® iris implant 1 year prior to presentation. Best corrected visual acuity was 6/7.5 (20/25) in both eyes with a manifest refraction of −6.00 −0.75 ×45° in the right eye and −4.25 −1.00 ×130° in the left eye. Intraocular pressure measured 17 mm Hg in the right eye and 15 mm Hg in the left eye. Slit-lamp examination revealed a quiet anterior segment in both eyes, clear corneas, and well-centered BrightOcular® implants (Fig. ). Both eyes were phakic with no cataract. Fundus exam revealed unremarkable posterior pole with cup/disc of 0.4 and limited view of the retinal midperiphery. Gonioscopy showed open angles (Schaffer grading 3) in both eyes. Retinal nerve fiber layer thickness by optical coherence tomography (OCT) was 84 μm in the right eye and 85 μm in the left eye. Using anterior segment optical coherence tomography (AS-OCT), anterior chamber depth was 3.29 mm in the right eye and 3.38 mm in the left eye and moreover, the iris diaphragm showed no impingement of the angle (Fig. ). Visual fields were constricted from the fixed pupillary opening (Fig. ). Specular microscopy demonstrated a normal endothelial cell count (central corneal endothelial count 2,471 cells/mm2 in the right eye and 2,338 cells/mm2 in the left eye) with normal morphology in both eyes (Fig. ). Central corneal pachymetry was 553 μm bilaterally. No treatment was given besides close monitoring. The exam was unchanged at the last follow-up at 12 months.
BrightOcular® Cosmetic Iris Implant: A Spectrum from Tolerability to Severe Morbidity
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A 61-year-old non-smoking woman was admitted to the hospital with a 2-week history of facial edema, dry cough, and dyspnea on exertion. She was experiencing tachypnea and moderate respiratory distress; her vital signs were as follows: blood pressure 100/60 mm Hg, heart rate 110 beats per minute, respiratory rate 28 breaths per minute, and oxygen saturation 94% on room air. Physical examination revealed facial edema and distended non-pulsatile superficial veins over the neck and chest. There was no lymphadenopathy and the systemic examination was unremarkable. Routine laboratory test results were within normal ranges. A chest radiograph revealed marked widening of the mediastinum without any lung parenchymal lesions (). Computed tomography (CT) of the chest revealed enlargement of the right upper paratracheal (45 mm in diameter) and both lower paratracheal lymph nodes (right lower paratracheal: 20 mm in diameter, left lower paratracheal: 23 mm in diameter), which were compressing the SVC. Each lymph node showed marginal contrast enhancement with low central attenuation (). Positron emission tomography showed abnormal fluorodeoxyglucose uptake in the upper paratracheal (standardized uptake value [SUVmax]=16.1), lower paratracheal (SUVmax=14.4), and hilar (SUVmax=11.0) lymph nodes. Owing to the short history and SVC obstruction with mediastinal lymph nodes, the differential diagnosis was considered in the following order: lymphoma, tuberculosis, and bacterial infection. Initial acid-fast bacillus staining of the sputum as well as the results of blood culture were all negative. The patient underwent a median thoracotomy to establish a diagnosis and relieve the symptoms of SVC syndrome. During the operation, the enlarged paratracheal lymph nodes were removed; histopathology of a biopsy specimen revealed chronic granulomatous inflammation and central caseous necrosis consistent with tuberculosis (). An acid-fast stain of the biopsy specimen was negative, but a nested polymerase chain reaction for Mycobacterium tuberculosis was positive. After the surgical diagnosis, we began anti-tuberculosis treatment (i.e., isoniazid, rifampicin, ethambutol, and pyrazinamide). The symptoms of SVC syndrome all improved during hospitalization. The patient completed a 9-month course of anti-tuberculosis treatment and was in good condition at the 3-year follow-up.
Superior Vena Cava Syndrome Due to Mediastinal Tuberculous Lymphadenitis
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A 38-year-old woman who initially presented for evaluation of left-sided proptosis was found to have a left orbital mass depressing the globe inferiorly on MRI. There was an extension of enhancement in the left sphenoid along the orbital, lateral frontal, and temporal dura (Figures -). A biopsy was performed that identified the mass as an orbital meningioma, WHO grade I. An orbital-zygomatic craniotomy with lateral orbitotomy was performed for resection of the tumor. Over the next three years, the patient developed episodes of intermittent headaches and seizures, after which she began developing blurriness and a sensation of fullness in the left eye, as well as worsening of her headaches. An MRI was performed that demonstrated an enhancing extra-axial mass along the left greater sphenoid wing with extension into the left aspect of the left orbit measuring 4.0 × 2.7 cm, consistent with recurrence of her meningioma. A left frontotemporal craniotomy was performed and the tumor was resected followed by postsurgical radiation.\nThe first orbital resection specimen in 2013 was most consistent with meningioma, CNS WHO grade 1. The microscopic evaluation demonstrated meningothelial cells infiltrating bone and muscle with bland cytology. No mitotic figures were identified. However, there were areas of fibrous change consistent with transitional meningioma. The tumor demonstrated a Ki-67 proliferation index of 4-6% with focal EMA and S-100 positivity. PR immunohistochemistry was negative. The second orbital resection specimen was most consistent with atypical meningioma, WHO grade 2. The microscopic evaluation demonstrated islands of cells with round to oval nuclei with poorly defined cell borders and nuclear pseudo-inclusions adjacent to muscle. Up to seven mitotic figures were identified in 10 HPFs. The tumor demonstrated a Ki-67 proliferation index of 12-15%. The tumor cells were positive for EMA and focally for PR.\nDuring her one-year follow-up after the second resection, the patient denied any worsening of her vision. However, her headaches, which she had been experiencing for several decades, persisted; this was attributed to a medical history of trigeminal neuralgia.
Clinical, Pathologic, and Radiologic Features of Orbital Solitary Fibrous Tumors and Meningiomas
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A 61-year-old male patient was admitted of bradycardia underwent a permanent double chamber pacemaker implantation. An intracardiac ultrasound proprietary catheter (CARTOSOUND; Biosense Webster) was advanced to the right atrium through femoral vein access. Meanwhile, the CARTO 3 mapping system (Biosense Webster) was applied to reconstruct the ventricle and interventricular septum models. Then, the 3830 lead (Select-Secure™ lead, Medtronic) was connected to the CARTO 3 mapping system and advanced into right ventricle (RV) through sheath 315HIS (Medtronic) under ICE real-time imaging guidance. During the operation, the sector of ICE was adjusted to clearly display both sides of the ventricular septum, and the position of the lead was observed directly (Figure ). The sheath was adjusted to the upper region of the IVS under ICE real-time imaging guidance. At this site, the paced QRS morphology usually demonstrates a “w” pattern in lead V1. The sheath was rotated counterclockwise to maintain the orientation of the lead tip perpendicular to the septal surface. During the time, EMG morphology changes of V1 lead, LBB potential, lead depth, and pacing impedance were closely monitored. Confirmation of the LBB capture was done according to Vijayaraman P and Huang., Including: Paced morphology of right bundle branch block pattern; Identification of the LBB potential; Stim-LVAT (stimulus to left ventricular activation time, defined as the interval from the pacing stimulus to the QRS upstroke in V5/V6) that shortens abruptly with increasing output or remains shortest and constant both at low and high outputs. Then, the pacing and EMG parameters of LBBP were measured. In this case, Stim-LVAT was 68 ms, and paced QRS was 115 ms (Figure ). The sensed R-wave amplitude was 7.1 mV, capture threshold was 0.7 V/0.4 ms, and lead impedance was 726 Ω. Ultrasonic images showed the lead tip was perpendicular to the interventricular septum, with depth of 8 mm (Figure ). LBBP was successfully achieved, with a radioactive exposure time of 1.5 minutes, and total operation time 76 minutes.
Intracardiac ultrasound-guided left bundle branch pacing in a bradycardia patient
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A one-year-old girl presented with a right cheek mass that had been growing for three weeks. She had fallen from her bed one month ago. Clinical examination revealed a 3x2.5 cm, oval, ill-defined, reddish-purplish colored mass involving the right cheek and extending from the medial canthus to the right malar region, causing facial disfigurement (). On palpation the mass was tense, firm, non-tender and pulsatile. Keeping differentials of A-V malformation and pseudoaneurysm in mind, color Doppler USG and CT scan were performed.\nColor Doppler USG revealed a well-defined vascular cystic lesion in the right cheek highly suggestive of a pseudoaneurysm. CT images were suggestive of a large vascular mass; however, the exact origin of the lesion could not be confirmed (). The child was taken up for surgical excision under general anaesthesia. The mass was dissected in toto and was found to be arising from the distal branch of the facial artery (, ). A standard pressure dressing was applied for 48 hours. The postoperative period was uneventful, and the child recovered well. Histopathological examination confirmed the diagnosis of pseudoaneurysm.
The Presentation and Management of Facial Artery Pseudoaneurysm: A Review of the Literature
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A 46-year-old Caucasian man presented with a 10-year history of mild gait ataxia and undirected vertigo after fast head movements. The patient had suffered from disturbed urination for the previous 25 years. He had a past medical history of resection of a thyroid adenoma and also for benign polyposis of the sigmoid colon. At the age of 41 years, a seborrheic keratosis was excised from his right ear. The patient had no familial history of LDD or hereditary disease. Physical inspection revealed megalocephaly, congenital facial asymmetry and left thenar aplasia. At the latest presentation, the neurological examination showed minimal intention tremor, gait ataxia without visual compensation, an undirected imbalance on Romberg's test and bradydiadochokinesia.\nWith approval of the local ethic committee and with the patient's informed written consent, MRI examinations of the brain were performed on a 1.5T scanner (Avanto, Siemens Medical Solutions, Erlangen, Germany) in combination with using a vendor supplied 12-channel receive-only head coil and then MRI examinations of the brain were done on a 7T scanner (Magnetom 7T, Siemens Medical Solutions, Erlangen, Germany) in combination with an 8-channel transmit-and-receive head coil (Rapid Biomed, Wuerzburg, Germany). The gradient-echo and turbo spin echo sequences were performed to obtain the axial proton density (PD), T2, T2* and susceptibility weighted images (SWI), which were optimized for each field strength ().\nIn addition, proton (1H) MR spectroscopy (MRS) was performed at 1.5T. The spectroscopic data was acquired from the patient's cerebellar lesion using a single-voxel, point-resolved technique (TE = 135 ms; TR = 1500 ms). The resulting prominent resonances representing choline (Cho), creatine (Cr) and N-acetylaspartate (NAA) within the lesion were compared to the mirror image voxels on the white matter of the normal contralateral hemisphere. Spectral post-processing was performed using the software provided by the MRI system manufacturer (Siemens Syngo, VB 15, Siemens Medical Solutions, Erlangen, Germany).\nFor 11 years, repeated 1.5T MRI examinations revealed a slowly growing, non-enhancing tumor mass in the left cerebellar hemisphere with preservation of the gyral pattern. Thus, the present study was done without administration of contrast media. On MRI at 1.5T and 7T, the posterior fossa tumor (49×34×32 mm in size) appeared mainly hyperintense on the T2-weighted images () and iso-hypointense on the proton density images (not shown). The characteristic striated pattern of the lesion was best displayed on the T2-weighted images at both field strengths (). The tumor caused descensus of the cerebellar tonsils, but any obstructive supratentorial hydrocephalus was absent. Due to their high sensitivity for paramagnetic substances like deoxyhemoglobin, the SWI and T2* weighted images revealed thin veins running deep between the thickened folia of the cerebellar lesion in great detail (). The 7T SWI minimal intensity projection (MIP) images depicted thin vessel branches as small as 250 µm, whereas the 1.5T SWI MIP images could only resolve larger vessels to a size of 450 µm. Compared to the 1.5T SWI images () the medial displacement and compression of the dentate nucleus by the tumor were much better registered on 7T SWI images (). The 1H-MRS at 1.5T demonstrated a reduction in NAA and a prominent lactate peak. Contrary to other previous reports, the Cho, Cr and the resulting Cho/Cr ratio were slightly elevated in the lesion and the myoinositol (MI) levels were not changed (, ). Thus far, neurosurgical therapy and histopathological examination have not been performed because the lesion exerted only mild compression of the IV ventricle without any hydrocephalus.\nA DNA analysis revealed a heterozygous mutation in exon 5 of the PTEN gene (chromosome 10 q23), and this supported the diagnosis of LDD (c. 388C > T; p. Arg130X). The patient received genetic counseling and is under neurological review.
Assessing a Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) with 7T MR Imaging
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A 58-yr-old man living in the southern area of Korea was admitted to the Chonnam National University Hospital complaining of painful swelling in both calves. The patient had a history of alcoholic liver cirrhosis. Two days prior to admission, the patient had eaten raw fish. He had neither history of trauma nor exposure to seawater.\nOn admission, both lower legs were erythematous and edematous, and hemorrhagic bullae were noted on the anterior aspect of the left lower leg. The patient's sclerae were icteric and the abdomen was distended. The patient's blood pressure was 100/40 mmHg, the temperature was 36.5℃, the pulse rate was 107 beats/min, and the respiration rate was 26/min.\nInitial laboratory results were as follows: white blood cell count, 4,200/µL; hemoglobin, 8.8 g/µL; platelet count, 55,000/µL; prothrombin time, 24.9 sec with an INR of 2.22; partial thromboplastin time, 66.1 sec; total serum protein, 5.8 g/dL; albumin, 2.5 g/dL; aspartate aminotransferase, 168 U/L; alanine aminotransferase, 56 U/L; alkaline phosphatase, 56 U/L; total bilirubin, 14.9 mg/dL; blood urea nitrogen, 69.0 mg/dL; creatinine, 1.1 mg/dL; creatinine kinase, 750 U/L; lactate dehydrogenase 767 U/L; erythrocyte sedimentation rate, 43 mm/hr; and C-reactive protein, 17.4 mg/dL.\nV. vulnificus septicemia was suspected based on the cutaneous manifestation of necrotizing fasciitis, the history of liver cirrhosis, and raw seafood consumption. Blood and a bulla aspirate were collected for bacteriologic cultures. The patient received 2,000 mg/day of intravenous ceftriaxone and 800 mg/day of ciprofloxacin, and fasciotomies were performed on the lower legs and dorsa of the feet. Despite treatment, erythematous swelling spread to the thigh and a second fasciotomy was performed on day 4 of hospitalization.\nCultures of blood, bulla aspirate, and tissue specimens obtained from the patient's calves yielded a gram-negative, nonfermentative, oxidase-positive bacilli. The isolate showed mucoid colony with β-hemolysis on blood agar plate after 48 hr incubation and H2S production on triple sugar iron agar. It was identified as Shewanella putrefaciens by the automatic identification systems using Vitek II (bioMerieux, Marcy-L'Etoile, France) and API 20NE (bioMerieux) with a probability of identification of 99.9%. Antimicrobial susceptibility testing with AST-N055 card (bioMerieux) showed susceptibility to all antibiotics except cefazolin. We performed a nucleic acid-based confirmatory test by using 16S rRNA gene sequencing analysis using the following primers: fD2 (5'-AGAGTTTGATCATGGCTCAG-3') and rP2 (5'-ACGGCTACCTTGTTACGACTT-3') (). The isolate gene sequence showed 99% nucleotide similarities with the sequences of S. algae strain ATCC 51192 (Gen Bank accession no. AB-205581). Therefore, the clinical isolate was ultimately identified as S. algae. Additional antimicrobial susceptibility test using disk diffusion method for the isolate showed susceptibility to colistin.\nIn spite of antibiotics and supportive care, bloody discharge from the fasciotomy sites persisted. On day 10 of hospitalization, massive hematochezia developed. Endoscopy showed esophageal and gastric varices with recent bleeding. On day 22 of hospitalization, the patient died with massive gastrointestinal bleeding and hepatic failure.
Primary Shewanella algae Bacteremia Mimicking Vibrio Septicemia
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A 28-year-old female, educated till 7th class, married and divorced 4 years back, brought to OPD by her sister, presented with apprehension, low mood, crying spells, worrying thoughts for 3 months. It started when her father expired 3 months back due to cardiac arrest. She could not bear the stress of her father loss and started developing low mood, crying spells. She also developed apprehension with palpitation at times of overthinking. According to her husband, they were not aware of her condition of problem in concentration and understanding before marriage, so they broke the marriage within a month. On examination, she has a swelling in the anterior portion of her neck. IQ was 46. TSH value was raised, 81 IUi/ml. Ultrasonography neck showed subacute thyroiditis. She was diagnosed with mixed anxiety and depression with moderate ID with hypothyroidism. She was treated with tablet sodium valproate 300 mg B.D., escitalopram 10 mg H.S, and tablet thyroxine 50 mcg.
Management of psychotic and mood disorders in intellectual disability: A case series
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A healthy 25-year-old female presented with a pruritic eruption on the right upper extremity of 6-year duration. There was no disease in childhood. The lesions appeared to follow lines of Blaschko, with progression to the shoulder and mid-scapula during 5 months of observation ( and ()). The patient described worsening of symptoms with sun exposure. Physical examination was significant for linear, scaly, clustered plaques involving the right proximal upper extremity, shoulder, and upper back. The remainder of her examination was normal. No acantholysis was noted. The differential diagnosis included inflammatory linear verrucous epidermal nevus (ILVEN), lichen striatus, linear keratosis follicularis (Darier disease), linear lichen planus, and porokeratotic eccrine ostial and dermal duct nevus (PEODDN). Syndromes such as craniosynostosis–anal anomalies–porokeratosis (CDAGS) were also considered in the differential diagnosis.\nA skin biopsy revealed distinctive cornoid lamellae comprising thin, columnar, closely stacked, parakeratotic cells extending through the stratum corneum with a minimal granular layer (). A diagnosis of linear porokeratosis was established based on clinical and histologic features. A therapeutic trial of isotretinoin 1.5 mg/kg for 24 weeks (6 months) and 1.7 mg/kg for 8 weeks (2 months) resulted in dramatic regression of hyperkeratotic changes, although mild local hyperpigmentation remained at the 2-month follow-up (). The patient provided informed consent to publish her unique case findings.
Linear porokeratosis presenting in adulthood: A diagnostic challenge: A case report
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A 57-years-old woman with a history of essential hypertension was brought into the emergency department (ED) with acute respiratory failure by an ambulance with a doctor. At home, she was found with a significantly decreased peripheral O2 saturation (SpO2) of 43% while breathing room air, saturation that increased to 86% after oxygen delivery (on a reservoir mask). On physical examination the patient presented tachypnea (34 breaths per minute), pale sweaty skin, hemodynamically stable, blood pressure 127/66 mmHg, a heart rate of 109 beats/min, a body temperature of 37.8 °C, Glasgow Coma Score (GCS) = 15/15. The patient complained of fever, dry cough, dyspnea on exertion and fatigue, that started 3 days before the presentation, claiming that, during the previous night, the dyspnea suddenly worsened and an anterior chest pain appeared. She had no other comorbidities except essential hypertension under treatment. Based on the history and clinical examination, SARS-CoV-2 infection was suspected. Prone position was initiated in order to improve ventilation, which the patient did not tolerate, so she returned to the seated position. She received an intravenous perfusion with 1 g acetaminophen, 8 mg dexamethasone, 1500 mg vitamin C, 40 mg pantoprazole and 500 mL normal saline (0.9%) solution. Blood was taken for lab tests and she was scheduled for a thoracic CT (computed tomography) scan. The pharyngeal and nasal swabs were taken for the RT-PCR test, which came positive after several hours.\nThe thoracic CT scan showed massive right pneumothorax (90 mm), lung partially collapsed, slightly left-displaced heart, multiple bilateral lung infiltrates with a ground-glass aspect that occupied about 65% of lung fields—CO-RADS classification 5, typical COVID-19 aspect. ()\nThe thoracostomy was performed and the drain tube was placed in the right fifth intercostal space, the medium axillary line, under local anesthesia. The patient’s condition partially improved and oxygen saturation increased by approximately 6% (SpO2 = 92–93%) 15–20 min after the drain tube was placed. Oxygen therapy on a reservoir mask with 15 L/min was continued, and remdesivir 200 mg as loading dose was initiated, then 100 mg at 24 h, lopinavir/ritonavir 200 mg/50 mg—2 pills every 12 h, enoxaparin 60 mg twice daily, dexamethasone 8 mg three times daily, pantoprazole 40 mg twice daily, vitamin C 1 g every 6 h, Ceftriaxone 1 g twice daily, her previous medicines for hypertension, acetaminophen on need and soluble regular insulin according to her glycemia level (given the constant high values observed during her hospitalization), vitamin D, B1, B6, zinc and alprazolam.\nLaboratory test results found to be pathological are shown in .\nAfter a few hours, a control chest X-ray was performed, confirming the correct placement of the drain tube with full expansion of the collapsed lung (). During the day, the patient’s breathing worsened (tachypnea 40 breaths/minute) with increased breathing effort, decreased oxygen saturation to 70%, hypoxemia (pO2 = 36 mmHg on arterial blood gases (ABG)) despite maximum oxygen delivery so she was admitted to the intensive care unit and non-invasive ventilation (NIV) was initiated. The patient tolerated non-invasive ventilation for a few hours, but then the condition of the patient worsened again, and the endotracheal intubation and invasive mechanical ventilation were decided.\nDuring hospitalization in the intensive care unit, the patient had a fluctuating evolution, and despite the treatment (medication, kinetotherapy, prone position, hydro-electrolyte rebalance solutions), the patient’s condition was deteriorating and required vasopressor support and mechanical ventilation. At 19 days after admission in the intensive care unit, the patient had cardiac arrest through asystole and did not respond to resuscitation maneuvers.
Case Report: Massive Spontaneous Pneumothorax—A Rare Form of Presentation for Severe COVID-19 Pneumonia
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A 53-year-old female with a history of rheumatoid arthritis and thyroid tumor was referred to our hospital. The patient’s chief complaint was abdominal pain for 1 day, and she had no history of abdominal surgery or trauma. Upon admission, she had right abdominal tenderness. Blood tests showed unremarkable results (white blood cell count, 4800/μL; red blood cell count, 4.78 × 106/μL; hemoglobin, 14.8 g/dL; hematocrit, 43.7%; platelets, 20.7 × 104/μL; C-reactive protein, 0.59 mg/dL; creatine kinase, 50 U/L; carcinoembryonic antigen, 2.0 ng/mL; and carbohydrate antigen 19–9, 12 U/mL). Contrast-enhanced computed tomography (CT) revealed intestinal torsion at the hepatic flexure (Fig. ). A colonoscopy was performed to evaluate the cause of the intestinal torsion, which revealed twisted mucosal folds in the transverse colon but no obvious neoplastic change on the mucosal surface (Fig. ). The scope could not be advanced past the torsion of the transverse colon, making it impossible to observe the superior colon.\nBased on these findings, the differential diagnosis included gastrointestinal stromal tumor, malignant lymphoma, sarcoma, carcinoid, and mesenteric tumors. Surgery was performed to release the transverse colonic volvulus and establish a definitive diagnosis. The lesion arose from the mesentery, causing a 180° clockwise rotation of the transverse colon around itself (Fig. ). The surgical diagnosis was transverse colonic volvulus due to a tumor of a mesenteric origin. Consequently, a right hemicolectomy with lymphadenectomy was performed.\nMacroscopically, the tumor was a clearly demarcated solid mass with a white–gray appearance (Fig. ). Histologically, the tumor consisted of dense collagenous fibers and a complicated proliferation of spindle-shaped cells, which generally appeared heterozygous and homogeneous, yet occasionally exhibited nuclear enlargement and mismatch in the center of the tumor (Fig. ). The tumor cells were positive for vimentin but negative for S100 protein, synaptophysin, cluster of differentiation 34, c-kit, and DOG-1, based on the immunohistochemical findings (Fig. ). These pathological findings led us to the final diagnosis of MF. The patient had an uneventful postoperative course and was eventually discharged in good condition 8 days after surgery. She is currently well and shows no signs of recurrence 6 months after the surgery.
Transverse colonic volvulus due to mesenteric fibromatosis: a case report
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A 16-year-old boy was referred to our department by his ophthalmologist. He was suffering from persistent epiphora for about one year, which had been diagnosed as chronic dacryocystitis due to right nasolacrimal duct obstruction, while he had undergone probing of the right nasolacrimal duct once, by his ophthalmologist. The patient also referred a mild pain in the area of median canthus, which was more intense during the night and relieved with aspirin uptake. In our department, clinical examination revealed only a small, firm mass, palpated in the middle angle of the right orbit. Nasal endoscopy revealed no intranasal pathology. There was normal ocular motility and no eyeball displacement.\nA Computed Tomography scan was performed, which showed a bone density mass within the right ethmoid air cells. The mass revealed sharp and well defined margins, extending laterally through the lamina papyracea, to the ipsilateral right orbit (Figure ). Subsequently, a Magnetic Resonance Imaging (MRI) scan was performed. Coronal, T1-weithted, post-contrast image, confirmed the presence of the mass filling the anterior portion of the right ethmoid air cells. The mass demonstrated patchy enhancement and protruded into the adjacent orbit. It should be noted that on MRI the mass depicted soft tissue intensity and enhanced following contrast administration (Figure ).\nThe mass was removed via an external ethmoidectomy approach. Intraoperatively it was noticed that the mass compressed the lacrimal sac, while no dacryocystorhinostomy was performed. Histopathological examination revealed that, the specimen mostly consisted of cancellous bone particles, some of which appeared to have distorted architecture, because of an interlacing network of variably sized, shaped and mineralized osteoid trabeculae. There was a rimming of osteoblasts surrounding the trabeculae. The intertrabecular spaces were occupied by loose connective tissue (Figure ). Focally dense sclerotic bone was recognized surrounding the above structures (Figure ). Diagnosis of ethmoid bone Osteoid Osteoma was set.\nOne year after the operation the patient was free of symptoms, while no recurrence occurred.
Osteoid osteoma of the ethmoid bone associated with dacryocystitis
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A 29-year-old man (height = 180 cm, weight = 60 kg) was admitted to our hospital due to sudden onset of palpitation, chest tightness, mild fever, and night sweats, accompanied with bilateral double lower extremities adynamia and paralysis for 5 days, but no obvious syncope and edema. Examination on admission revealed a normal heart rate of 96 beats/minute (bpm) and blood pressure of 120/80 mmHg. On cardiac auscultation, 3/6 grade systolic murmur (Levine Scale) was heard between the third and fourth ribs at the left margin of sternum. The sensory below the sternum was dysfunctional. Muscle strength on both legs was at 1/6 levels (Lovett Scale), and tendon reflex diminished. Electrocardiography showed a sinus rhythm with pulmonary P-wave. An X-ray image of the chest showed discrete and scattered miliary nodules over both lungs, and cardiac silhouette was enlarged. Magnetic resonance imaging (MRI) (Fig. ) showed an erosive space-occupying lesion located between the first and third thoracic vertebrae, which resulted in stenosis of the spinal canal and thinning of the spinal cord. Transthoracic echocardiography (TTE) (Fig. ) showed a huge mobile mass (72 × 58 mm) in the RA and myxoma was considered. The left ventricular ejection fraction was 60%. The remaining physical examination findings were unremarkable, and laboratory tests were normal, except for the accelerating erythrocyte sedimentation rate. There was no family history of heart disease, including tumors and other cardiovascular problems. The preliminary diagnosis was thoracic vertebra tumor and cardiac myxoma. To treat the incomplete paraplegia caused by thoracic vertebra tumor erosion, the operation including resection of thoracic vertebral lesions, decompression, and internal fixation with nail-rod must be carried out immediately. A multiple disciplinary team consisting of cardiologist, orthopedist, sonologist, and anesthesiologists, was created. Fatal complications of cardiac myxoma, such as intracardiac obstruction and pulmonary embolism, were assessed. Informed consent of critical illness was signed by the patient's legal representative.\nThe patient did not receive premedication before anesthesia. On arrival in the operating room, peripheral venous access was established on the dorsum of the hand, and continuous electrocardiogram and pulse oximetry monitoring were instituted. Invasive arterial blood pressure (IBP) was measured in the left radial artery, and the central venous pressure (CVP) was measured in the right femoral vein. Anesthesia induction was carried out with intravenous injection of midazolam 4 mg, penehyclidine hydrochloride 1 mg, etomidate 16 mg, and sufentanil 50 μg. Cisatracurium 12 mg was used to facilitate tracheal intubation. Maintenance of compound anesthesia consisted of 2% sevoflurane in an air–oxygen mixture, and remifentanil was continuously injected intravenously at 0.2 μg/kg/min. Intermittent bolus dose of 5 mg cisatracurium was administered for muscle relaxation. After anesthesia induction, hemodynamic parameters were stable at the supine position: heart rate of 80 bpm, IBP of 110/65 mmHg, and CVP of 8 mmHg. However, after the patient was turned to the prone position on the level of the standard operating table (lying on a pair of bolsters, unpressurized abdomen), the patient's heart rate increased gradually to 130 bpm, IBP dropped to 70/45 mmHg, and CVP increased to 22 mmHg. Moreover, the jugular vein was filled observably. Emergency treatment was carried out immediately against the symptoms of right heart failure. Fluid infusion was restricted and vasoactive drugs were used to maintain appropriate arterial blood pressure. Dopamine at 5 to 10 μg/kg/min and norepinephrine at 0.03 to 0.05 μg/kg/min were continuously injected intravenously, keeping the mean arterial pressure above 70 mmHg during the operation. During the 3 hours of surgery, blood volume supplementation totally consisted of sodium chloride 500 mL, hydroxyethyl starch 500 mL, fresh-frozen plasma 600 mL, and suspended red blood cells 2 U. Total blood volume loss was 600 mL, and urine output was 500 mL. After the operation, anesthetic drugs were stopped, and the patient was returned to the supine position. At this moment, his hemodynamic status was stabilized gradually with a heart rate of 84 bpm, IBP of 130/88 mmHg, and CVP of 10 mmHg. Dopamine and norepinephrine intravenous titration was suspended. When the patient recovered spontaneous respiration and responded to verbal commands, tracheal extubation was performed. After ventilated with 100% oxygen via a facemask in 5 min, the patient was transferred to the post-anesthesia care unit. Evaluation of spinal function was performed on the second postoperative. The muscle strength of the left leg was 4/6 levels, and the right leg was 3/6 levels; meanwhile, the superficial sensibility was considered dysesthesia. Furthermore, hemodynamic parameters were stable at the ward.
Hemodynamic management of a patient with a huge right atrium myxoma during thoracic vertebral surgery
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A 6 years old boy with dyspnea, orthopnea, generalized pitting edema and fever, was admitted to the pediatric intensive care unit, Shanghai Children’s Hospital. Three years before this episode, he presented to our hospital with acute onset of edema, hypoalbuminemia, heavy proteinuria and hyperlipidemia. The diagnosis of IgA nephropathy (Grade II) was made by percutaneous renal biopsy and in accordance with Lee’s classification []. Initially he responded well to steroid therapy, but became steroid resistant after 2 years treatment. Immunosuppressive agents administered during this time period, included cyclophosphamide, mycophenolate mofetil and tacrolimus. He had no history of trauma, tuberculosis or radiation therapy.\nPhysical examination revealed tachypnea, orthopnea, anasarca and ascites. The heart sounds were muffled and hepatomegaly was also noted. Blood pressure ranged from 90/60 mmHg (systolic/diastolic blood pressure) to 130/70 mmHg. Blood cell count showed that white blood cells (WBC) was 14.49 × 109/L, neutrophils 79%, hemoglobin 11.5 g/dl, C-reactive protein 130 mg/L. Biochemistry analysis revealed total protein of 36 g/L, albumin 10 g/L, alanine aminotransferase (ALT) 10 U/L, aspartate aminotransferase (AST) 29 U/L, triglycerides 2.05 mmol/L, cholesterol 10.13 mmol/L. Serum electrolytes (Na+, K+, Ca2+ and Cl−) were normal. Heavy proteinuria and hematuria were found on urinalysis (urinary protein: creatinine ratio 30.38). Serum creatinine was normal and estimated glomerular filtration rate (eGFR, calculated with Schwartz formula) was 147 ml/min/1.73m2. Blood and urine cultures were sterile. T-spot for tuberculosis was negative. There was no ultrasound evidence of thrombosis in the superior vena cava or subclavian vein. Ultrasound also demonstrated that both kidneys were enlarged with a loss of cortico-medullary differentiation. Thoracic computed tomography (CT) found no evidence of congenital malformation or malignancies. Echocardiography revealed pericardial fluid of 4.6 cm at maximal thickness, suggesting a large volume hydropericardial effusion. The massive pericardial and pleural effusions were additionally confirmed by thoracic CT (Fig. ). Notably, milky fluid was obtained from the pericardial space by pericardiocentesis (Fig. ). Chyle test was positive. Cell counts and biochemistry in the pericardial effusion revealed WBC 405 × 106/L, lymphocytes count 92%, red blood cells 63 × 106/L, AST 8 U/L, lactate dehydrogenase (LDH) 58 U/L, Glucose 7.22 mmol/L, total protein 7 g/L, albumin 4 g/L, adenylate deaminase (ADH) 2.6 U/L, triglycerides 2.55 mmol/L, cholesterol 0.79 mmol/L. These findings confirmed the diagnosis of chylopericardial effusion.\nContinuous renal replacement (CRRT) therapy was performed to alleviate the fluid overload. Meanwhile, pericardial drainage was performed and the diet was modified to low fat but rich in middle chain triglycerides and high protein. No side effects were encountered with this diet modification. The symptoms of cardiac tamponade subsided promptly, while the edema receded gradually over the subsequent 2 weeks. The indwelling pericardial catheter was removed when no fluid was drained after 3 weeks treatment and as shown in Fig. , the chylopericardial effusion was removed successfully. Pericardial effusion has not recurred at 1 year of follow up, on follow up echocardiography.
Chylopericardium in a child with IgA nephropathy: a case report
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A 61-year-old female veteran with history of percutaneous lead SCS implant in 10/2009 for neurogenic claudication with good relief of symptoms with subsequent SCS revision with generator relocation due to discomfort in 8/2015 presented in 8/2017 to pain management for new symptoms. She complained of intermittent shock-like sensations traveling from her midline lower thoracic insertion incision region of the lead extending up the spine a few inches occurring multiple times a day, 3-4 days a week for previous three months. The sensation would occur positionally while leaning back. She denied any back injury and felt the SCS was providing relief for her chronic symptoms. Interrogation of the SCS was normal. She was referred to neurosurgery for explantation and replacement with a magnetic resonance imaging (MRI) compatible device with a goal of eliminating the shock-like sensations, continue appropriate pain coverage and allow for future MRI scans.\nSurgery was performed two months later via midline lower thoracic incision and right flank incision under monitored anesthesia care (MAC). Her previous two leads which were positioned at the T9/T10 level were both removed and two more epidural leads were placed percutaneously through the L1/L2 epidural space into the T8/T9 level (Figure , ).\nUpon waking from surgery, she complained of severe left lower extremity sharp, spasmodic pain concentrated in the medial thigh going into the groin and buttock. Additionally, shooting pains down the extremity, patchy numbness proximally becoming circumferential numbness below the left knee, and distal greater than proximal weakness with very minimal movement of toes all were suddenly present. There was no contralateral pain, numbness, or weakness.\nThe anesthesia team cleared the patient of anesthesia complications. An urgent non-contrast MRI of the thoracic spine showed a syrinx in the left hemicord at the level of T11 extending to T12 appearing to be posttraumatic as there was a focus of increased T2 signal with a small amount of blood (Figure -; Figure -).\nThe patient was immediately started on dexamethasone, gabapentin, opioids, diazepam and ketamine infusion for analgesia and admitted to the neurosurgical inpatient service.\nRepeat MRI scan postoperative day 3 showed slight decrease in cord edema and smaller diameter of the longitudinal spread of the T2 intramedullary signal change correlating with slight improvement of symptoms. Her strength and distal numbness showed some improvement, groin pain resolved, however she noticed medial left knee pain associated with bladder fullness but with no difficulty voiding. She was taken off ketamine at this point and steroids started to be weaned. She was able to ambulate with walker and start physical therapy. Additionally, her SCS was reprogrammed with the added benefit of covering her pre-existing bilateral lower extremity pain. The intermittent shock sensations did not return after surgery. She was discharged to a rehabilitation facility and had a three-week post-operative follow-up visit with neurosurgery. Given her left lower extremity symptoms potentially exacerbated by the SCS, the SCS was reprogrammed to cover only her right lower extremity (RLE). She had her outpatient pain management clinic follow-up two months post-operatively and was frustrated that although she regained some strength, she was still having difficulty at work as she is a bus driver. She also felt that the pain level hadn’t improved and numbness remained the same. Repeat MRI of the thoracic spine without contrast on this day showed mild diminishment of syrinx size.
Spinal Cord Stimulator Explant and Revision Complicated by Syrinx Formation: A Case Report and Literature Review
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In March 2007 a 14 month old girl with congenital complete heart block underwent implantation of an endocardial pacemaker into the right ventricle. She had remained asymptomatic throughout the first year of life.\nSix weeks after her pacemaker insertion, she presented with pyrexia, lethargy, diarrhoea, vomiting and poor fluid intake. She was diagnosed with otitis media and sent home on amoxicillin/clavulanic acid. She was seen again two days later as she remained febrile. She was pale and lethargic with a liver edge extending to 1.5cm below the costal margin, and a grade 2/6 systolic murmur. She was sent home and advised to continue her oral antibiotics.\nTwo weeks after initial presentation she became acutely unwell, presenting in shock, with a grade 3/6 systolic murmur. She received a fluid bolus and commenced on intravenous ceftriaxone. CXR showed an enlarged heart with pacemaker and lead in situ ().\nInvestigations showed a C-reactive protein of 275 mg/L, white cell count of 35.7×109/L, neutrophils 17.2×109/L, platelets 111×109/L, and haemoglobin of 7.6g/dl. Infectious endocarditis was suspected. There were no external signs of pacemaker pocket infection. An echocardiogram showed “a mobile mass attached to the pacing wire and tricuspid valve, moderate tricuspid regurgitation and a patent foramen ovale” ().\nGentamycin was added to the antibiotic regime. Blood cultures showed a pure growth of gram negative rods later confirmed to be Haemophilus parainfluenzae, sensitive to 3rd generation cephalosporins and gentamycin. One week later she underwent open heart surgery under bypass for the complete removal of the infected cardiac pacemaker system ().\nCulture of the vegetation failed to isolate any organisms. A week later she had a permanent epicardial pacemaker system inserted (requiring sternotomy). She was discharged from the cardiothoracic unit two weeks after admission. A follow-up 2D echocardiogram showed moderate tricuspid regurgitation and a small vegetation that was still present near the apical ventricular septum. Clinically she has remained well and haemodynamically stable.
Pacemaker endocarditis due to Haemophilus parainfluenza: case report and literature review
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This study was approved by the Osaka Medical College Clinical Research Review Board, and the patient with her parents gave written informed consent for publication.\n13-year-old girl with no gravida and her first menses at 11 years of age had a regular menstrual cycle with 4 to 5 days of bleeding each month. She had no remarkable medical history or family history. She had felt gradual abdominal distention over the past several months. Her body mass index was 18 kg/m2.\nTransabdominal ultrasonography revealed a solid mass with a diameter of 10 cm located behind the uterus (Fig. ). The findings of a blood examination, including tumor markers, were within normal limits. Magnetic resonance imaging (MRI) revealed the mass to be within the uterine posterior wall measuring 11 cm at presentation. There was no denaturation in the uterine tumor and no suggestions of malignancy (Fig. ). Laparoscopic myomectomy was scheduled because of her abdominal distention.\nSurgery was performed with peumoperitoneum under general anesthesia. She underwent multiport (4 ports) laparoscopic myomectomy with parallel procedures. The uterus itself was the size of a newborn's head with normal tubes and ovaries (Fig. A). There was no adhesion. A dilute pitressin was injected into the myometrium. The serosal surface of the leiomyoma was incised in a monopolar direction, and dissection was performed with care taken to avoid entering the uterine cavity (Fig. B). After myomectomy, the leiomyoma was cut into smaller pieces inside a plastic bag (MemoBag, Teleflex, Japan) and retrieved from abdominal cavity. The myometrium was closed a using a simple interrupted suture and a baseball suture with 2-0 PDS (Ethicon, Johnson & Johnson, Somerville, NJ, USA) (Fig. C). The total weight of the leiomyoma removed was 660 g, and the surgery lasted for 5 hours and 17 minutes with 1050 ml of blood lost (864 ml restored by Cell Saver; Haemonetics Co., Braintree, MA, USA).\nThe postoperative course was uneventful. Pathologically, the tumor was diagnosed as a uterine leiomyoma. Eighteen months have passed since the treatment, and no recurrence has been noted.
Uterine leiomyoma in a 13-year-old adolescent successfully treated with laparoscopic myomectomy
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A medically free 60-year-old male patient presented to our endocrinology clinic with pain and swelling in the anterior aspect of his neck of 1-week duration. Three weeks prior to his presentation, he had an upper respiratory tract infection. Clinically the patient was euthyroid.\nOn examination, his pulse rate was 105 beats per minute and regular and his blood pressure was 145/85mm Hg. Thyroid examination showed a tender small goiter. He had no retrosternal goiter, bruits, or cervical lymphadenopathy. The rest of the examination was unremarkable. Laboratory values showed an elevated serum free T4 and free T3 and a suppressed thyroid-stimulating hormone (TSH) []. Erythrocyte sedimentation rate (ESR) was 80mm in the first hour. His white cell count was 8.54 × 109/L with a normal differential. The blood film showed occasional hypersegmented neutrophils.\nInitial clinical diagnosis was SAT. To confirm the diagnosis, a 99mTc scan was completed, which showed a focus of intense uptake in the region of the inferior pole of the right thyroid lobe with markedly suppressed uptake of the remainder of the thyroid []. The imaging finding was consistent with a toxic adenoma. Accordingly, the patient was started on carbimazole and propranolol.\nAlthough thyroid ultrasound was ordered on presentation, it was not completed until 8 days later because the patient missed his ultrasound appointment. It revealed multiple small hypoechoic nodules; the largest one in the superior pole of the left lobe measured 5mm × 10mm. More importantly, there was no nodule in the inferior pole of the right lobe corresponding to the hot focus seen on his initial thyroid scan. As a result, the diagnosis of toxic thyroid adenoma was questioned. Therefore, carbimazole was discontinued and propranolol was tapered and discontinued.\nRepeat Tc scan was performed when the patient’s serum TSH normalized []. It showed heterogeneous tracer uptake with a warm nodule in the inferior pole of the left thyroid lobe []. The hot focus in the inferior right lobe (which was presumed to be an autonomous adenoma) was no longer seen. A repeat thyroid ultrasound did not reveal significant changes. The final diagnosis was SAT in a patient with multinodular goiter with no scintigraphic evidence of autonomous adenoma.
Esophageal 99mTc-pertechnetate uptake mimicking an autonomous thyroid adenoma in a patient with subacute thyroiditis: a case report
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An 80-year-old Asian woman underwent cataract surgery in the right eye (OD). Preoperatively, best-corrected visual acuity (BCVA) was 20/70 OD with spherical equivalent of −6.50 diopters. Axial length measured with an optical biometer (IOLMaster, Carl Zeiss Meditec AG, Jena, Germany) was 26.08 mm OD. During phacoemulsification, posterior capsular rupture occurred. After aspiration of the lens fragments, anterior vitrectomy and scleral fixation of intraocular lens (IOL) was performed. Immediately after finishing the scleral fixation, a sudden elevation of dark-brown choroidal mass with rapid progression was detected. The main and side-port incisions were promptly closed with an intracameral instillation of 2% pilocarpine hydrochloride. A small amount of viscoelastic was left in the intracameral space to prevent vitreous incarceration. Intraocular pressure (IOP) was 37 mmHg OD immediately after surgery. After administration of intravenous 15% mannitol 200 mL, IOP decreased to 22 mmHg. After 1 day, BCVA was hand motion (HM), and IOP was 15 mmHg OD. Ultrasonography revealed SCH with central apposition (), and anterior segment examination showed a massive SCH touching the IOL (). Vitreous incarceration to wound was never found, although vitreous prolapse to intracameral space was observed. After 2 weeks, BCVA was HM, and IOP was 11 mmHg OD. Ultrasonography showed decrease in SCH with resolution of central apposition. At postoperative 6 weeks, BCVA improved to 20/120 and IOP was 11 mmHg. Ultrasonography showed a complete resorption of the SCH (), and the fundus was flat (). At postoperative 1 year, BCVA improved to 20/50 and IOP was 12 mmHg. Fundus was flat without any signs of retinal detachment.\nWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. Due to the retrospective nature and report of a single case, ethics approval was not requested. This study conformed to the tenets of the Declaration of Helsinki in respect of the testing of the device on humans. A copy of the written consent is available for review by the Editor of this journal.
Spontaneous resolution of massive expulsive suprachoroidal hemorrhage with good long-term visual outcome: a case report
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A 71-year-old female presented in our clinic with a large left facial mass requesting surgical excision for a cosmetically better facial appearance. She had been aware of the slow-growing, painless swelling for over the past fifteen years. She had sought medical advice ten years before, but she had been unwilling to undergo surgery due to the fear of the postoperative morbidity. Clinical examination revealed a mobile, soft, and non-tender mass measuring 10 × 7 cm in size over the region of the left parotid and upper lateral neck area (Figure ). The surface of the mass was smooth and the overlying skin was normal without any signs of discoloration or tumor-adhesion. The facial nerve function was intact and the remainder of the head and neck examination was unremarkable.\nThe high-resolution CT scan showed an adipose tissue signal density mass arising from the left deep-lobe parotid gland that protruded inferiorly to the upper lateral neck (Figures ). A classic parotidectomy incision was made and the main trunk of facial nerve identified at the stylomastoid foramen. From the main trunk of the facial nerve, each branch in continuity is dissected away from the overlying superficial parotid lobe. Then a glistening yellow mass was identified in the deep lobe, closely associated with the lower branches of the facial nerve (Figure ). The tumor was well encapsulated and easily and totally enucleated after mobilization of the overlying facial nerve branches. Then the raised superficial parotid lobe was repositioned to cover the facial nerves before wound closure (Figure ). The surgical specimen was well encapsulated measuring 9 × 6 cm (Figure ). The histopathology examination revealed fibrolipoma, a histological variant of lipoma. The patient had an uneventful recovery, with a satisfying facial contour and intact facial nerve function (assessed as House-Brackmann Grade 1) (Figure ). Neither tumor recurrence nor Frey's syndrome was observed 9 months after surgery.
Giant lipoma arising from deep lobe of the parotid gland
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A 9-year-old boy was referred to our hospital with a 20-day history of cough, stridor, and shortness of breath. He had showed a poor clinical response to conventional therapy with antibiotics and systemic steroids at a local hospital. He was previously healthy with no relevant family history. A computed tomography (CT) scan performed at a local hospital revealed a homogeneous mass obstructing the trachea. During preparation for rigid bronchoscopy (RB) at the local hospital, he was found to be intolerant to general anesthesia and his dyspnea exacerbated. He was transferred to our hospital immediately. On admission to our hospital, the patient appeared dyspneic and had a characteristic biphasic stridor, with a percutaneous oxygen saturation of 93% under mask oxygen inhalation, body temperature of 37.7°C, pulse rate of 162 beats/min, respiration rate of 34 breaths/min, and blood pressure of 127/74 mmHg.\nOn day 2, contrast-enhanced multidetector CT revealed a mass lesion with a fat density that was nearly totally obstructing the tracheal lumen (). The multidisciplinary consensus was to proceed with bronchoscopy to obtain a definitive diagnosis to guide management and determine the prognosis. After being fully informed about the surgical options and possible complications related to different surgical modalities, the patient's parents elected to have the mass removed by an electrocautery snare with FB. A rigid bronchoscope (Karl Storz, Germany), argon plasma coagulation (APC) (ERBE VIO200D+APC2), cryotherapy (ERBOKRYO CA), and Holmium: YAG laser (Dahua DHL-1, China) were available if necessary. The parents also agreed that if the treatment failed, surgical resection would be carried out immediately. Written informed consent for the interventional procedures was obtained from the patient's parents.\nThe mass resection was performed on day 3. Pulmonologists, otolaryngologists, thoracic surgeons, anesthesiologists, and pediatric intensive care unit doctors were all in the operating room to ensure the success of the treatment and the safety of the patient. General anesthesia was induced with 8% sevoflurane. After the intravenous administration of 2 mg of midazolam, a laryngeal mask airway (LMA) was inserted to maintain an open airway. Intraoperatively, anesthesia was maintained with 3% sevoflurane. Based on the depth of anesthesia, 10 mg of pentazocine and 50 mg of propofol were intravenously administered intraoperatively. A swivel adapter was used to connect the proximal end of the laryngeal mask to the T-piece anesthesia system. A flexible fiberoptic bronchoscope (Olympus 260; external diameter: 4.9 mm, working channel: 2 mm) operated by an experienced pulmonologist was inserted via the swivel adapter. A large mass covered by smooth mucosa with a stalk was obstructing approximately 85% of the trachea in the mid-tracheal region (). A flexible 1.9-mm electrocautery snare (Olympus CD-6C-1) was then introduced through the working channel of the bronchoscope. After placing the loop of the snare around the base of the mass (), the electrocautery snare was used in blend mode at 30 W to cut and coagulate the base of the mass (). The mass was resected without evidence of bleeding or perforation (). The inspired oxygen concentration was maintained at 25% to avoid potential combustion during the interventional procedure. Due to the broad base of the mass, part of the remaining tissue at the root was removed with biopsy forceps to reduce the amount of residual tissue. Postoperatively, the patient recovered completely and was discharged home on day 8. The pathologic examination suggested that the mass was a tracheal hamartoma predominantly constituted of mature fat cells (). The patient's condition remained stable for 1 year following the interventional therapy. The patient returned to the hospital for repeat chest CT examination and bronchoscopy at 1 year postoperatively (). There was a slight protrusion at the position of the incision ().
Case Report: Resection of Giant Endotracheal Hamartoma by Electrosurgical Snaring via Fiberoptic Bronchoscopy in a 9-Year-Old Boy
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A 54 year-old man was referred to the outpatient clinic having been injured in his right eye by the hook of a clothes hanger while falling. Swelling and bruising on the right eyelid were observed but there were no lacerations. He complained of image tilting in the right eye compared to the image inthe left eye. His corrected visual acuity was 6 / 6 in both eyes. Findings on intraocular examination were unremarkable. Non-dilatating fundusphotos (VX-10; Kowa Optimed, Tokyo, Japan) showed a 12-degree excyclotorsion in the right eye and an 8-degree excyclotorsion in the left eye. Image J (National Institutes of Health, Bethesda, MD, USA) was used to measure the angle between the line that crosses the center of the optic disc and parallels the fovea and the line that extends from the optic disc center to the fovea []. Non-enhanced orbit computed tomography was performed to rule out eyeball rupture, intraocular foreign body, or orbit wall fracture as well as any other abnormalities. During surgery, a 15-mm horizontal conjunctival laceration over the superior rectus muscle was found. The tendon of the superior oblique muscle protruded inferio-nasally through the lacerated conjunctiva (). The cut margin of the ruptured tendon was irregular and the width was about 4 mm. We could not find the distal end of the tendon. It was assumed that the location of the rupture was in the middle of the tendon under the superior rectus muscle. The superior rectus muscle seemed to be intact. To correct torsional diplopia, we attached the anterior and posterior borders of the superior oblique tendon 5 and 9 mm apart from the nasal margin of the superior rectus muscle insertion (). One week postoperatively, the patient showed vertical diplopia and 4-prism dioptor right hypertropia by the alternative prism and cover test in the primary position. Double Maddox rod testing revealed 2° of excyclotorsion in the right eye. However, the patient did not complain of torsional diplopia. In the fundus photographs, the excyclotorsion of the right eye improved from 12° to 7° (). Underaction of the right superior oblique muscle was not noted. Three months postoperatively, right hypertropia gradually increased to 20-prism diopters and the patient complained of vertical diplopia. A 7 mm resection of the right inferior rectus was performed. Two months postoperatively, the patient remained comfortable and stable.
Traumatic Rupture of the Superior Oblique Muscle Tendon
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In June 2009, a 60 year old male patient was referred with anemia, thrombocytopenia, loss of weight and fever. His white blood cell count was 7 × 109/l, with 64.5% neutrophils, 24.6% lymphocytes, 4.2% monocytes, 1% eosinophiles, 1% basophils and 4.6% blast. Bone marrow was hypercellular with 19% blast cells. Dysplastic changes in bone marrow included cytoplasmic hypogranulation of neutrophils, erythroblasts and micromegakaryocytes. The red blood cell count was 3.38 × 106/cmm with 8.3 g/dl hemoglobin level along with platelet count of 49 × 109/l, and LDH value of 571 U/l. Physical examination of the patient showed splenomegaly. The patient was treated with Zyloric (300 mg as a daily dose) and Hydroxyurea (500 mg as a daily dose) for 1 month and later continued on Hydroxyurea (500 mg as a daily dose) for 3 month. The patient died 3 months after diagnosis.\nKaryotyping was done after the initiation of the treatment which showed a mosaic and biclonal karyotype with 45, XY, -4, der(6)t(4;6)(?;?), der(8)t(4;8)(?;?), t(13;16)(?;?)/45, XY, der(7)t(7;13)(?;?),-13 (Figure ), which was further studied by molecular cytogenetics (Figure , , ). Dual-color-FISH using WCP and CEP probes specific for chromosomes 4, 6, 7, 8, 13 and 16 confirmed the translocation seen in GTG-banding. Application of subtelomeric probes for 7pter and 7qter revealed two signals of subtelomeric 7qter on both homologous chromosomes 7 and one signal of subtelomeric 7pter on intact chromosome 7. Thus, subtelomeric region 7pter was deleted on the derivative chromosome 7 (Figure ). Applying an RB1-specific probe showed one signal only on the normal chromosome 13 in both clones. The analysis using MCB probes specific for individual chromosome involved in translocation, determined the breakpoint location and the final karyotype was found to be with 45, XY, -4, der(6)t(4;6)(p15.1;p21.3), der(8)t(4;8)(q31.2;q22), t(13;16)(q21.3;p11.2)[]/45, XY, der(7)t(7;13)(p22.2~22.3;q21.3),-13 [].\nImmunophenotyping of peripheral blood characterized the neutrophiles which showed abnormal side scatter pattern, as well as abnormal intensity staining patterns for CD11b(63%), CD11c(59.3%), CD10(17.4%), CD13(44%), CD15(59.3%), CD16(46%) and CD33(20.4%). The majority of monocytes were HLADr+ (4.2%). Lymphocyte subsets percentages were low. The patient was diagnosed as having common MDS, refractory anemia with excess of blasts (RAEB) in the French-American-British (FAB) classification, or RAEB-II in the World Health Organization (WHO) classification [].
Biclonal myelodysplastic syndrome involving six chromosomes and monoallelic loss of RB1 - A rare case
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A 43-year-old Australian female presented to the emergency department complaining of shortness of breath, urinary frequency and heavy menstrual bleeding on a background of known multi-fibroid uterus. She denied any bowel symptoms. The patient had been diagnosed with a large multi-fibroid uterus whilst living in the USA, however, due to a lack of health insurance and high medical costs, surgical treatment had not been sought. She had received two blood transfusions in 2018 due to anaemia. Due to the coronavirus disease of 2019 pandemic she decided to return home to Australia and upon her return presented to the emergency department seeking treatment. Her body mass index was 36 and she had iron deficiency anaemia secondary to heavy menstrual bleeding. She had no past surgical history. She had never been pregnant and desired preservation of her fertility. She was on oral iron supplementation.\nOn examination, her vital signs were within normal limits. Her uterus was palpable to just below the xiphisternum and her abdomen was mildly tender. A full blood count was performed which showed haemoglobin of 91 g/l with a mean corpuscular volume of 73 fL. Renal function was normal. A pelvic ultrasound revealed a uterus that was grossly enlarged by multiple fibroids, extending above the level of the umbilicus and into the right hypochondrium. The largest fibroid was a broad-based right fundal exophytic subserosal fibroid measuring 147 × 114 × 180 mm. There was a large posterior intramural fibroid measuring 124 × 111 × 103 mm, in addition to multiple other fibroids. The patient was further assessed with magnetic resonance imaging (MRI; ), which re-demonstrated marked uterine enlargement secondary to the fibroids, some of which demonstrated areas T2 hyperintensity reflecting cystic degeneration. The largest exophytic fibroid at the right lateral uterine fundus (dotted outline) extended superiorly to abut the inferior right hepatic lobe, compressing the right kidney and displacing bowel loops to the left upper quadrant. The largest intrauterine fibroid compressed the endometrial cavity and displaced it rightward. No malignant or aggressive features were present. These imaging findings were consistent with an ultrasound and MRI performed in the USA ~18 months prior.\nThe patient was commenced on tranexamic acid and Provera to manage her bleeding while awaiting surgery. A hysterectomy was advised, however due to her desire for children this recommendation was declined by the patient. To investigate her fertility anti-mullerian hormone, follicle stimulating hormone and luteinizing hormone levels were performed (all within normal parameters). The patient elected to undergo a myomectomy with the understanding the there was a risk of proceeding to hysterectomy intraoperatively. An iron transfusion was organized preoperatively.\nAn abdominal uterine myomectomy was performed. A midline laparotomy was made and extended above the umbilicus due to the size of the uterus. Three uterine incisions were made (fundal, posterior wall and anterior wall) and a total of 29 fibroids were removed ranging from 20 cm to 1 cm in diameter. The cavity was breached with the posterior incision and closed with 2-0 polydioxanone suture; otherwise, the uterine wounds were closed with multi-layered 1.0 vicryl sutures. The uterus remained large (20 weeks gestation) at the end of the procedure. On further inspection no large fibroids were noted and diffuse adenomyosis was diagnosed. Estimated blood loss from the procedure was 1.5 l.\nHaemoglobin was 74 g/l post-operatively and one unit of packed red bloods cells was transfused. The patient had an uneventful recovery and was discharged home on day five post-operatively. Histopathology showed benign leiomyomas with no atypical features. The patient was reviewed in clinic 6 weeks after her surgery. She noted one period post-operatively and described this as normal. She expressed interest in a sperm donor program and was referred to a fertility service for further management.
Successful uterine-sparing surgical management in a patient with a large multi-fibroid uterus
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A Hindu male patient aged 22 years diagnosed with Wilson's disease attended our psychiatry out patient department (OPD) with chief complains of wandering behavior from home multiple times since past 6 months, inappropriate giggling, muttering to self, poor self-care, social withdrawal, and emotional blunting. The patient had occasional anger outbursts with irritability and beating parents whenever he was asked to take proper self-care or eat food. Over and above these psychiatric symptoms, the patient had severe extra pyramidal symptoms like drooling of saliva, rigidity in muscles of bilateral upper limbs, festinate gait, and masking of face. There was history of discontinuation to the prescribed doses of penicillamine 8 months ago, i.e., 2 months before the initiation of the current symptoms. Following this, psychotic symptoms were the first to develop, followed by fever, severe anemia, and extrapyramidal symptoms (without antipsychotic medication) about 1 month ago.\nInvestigations of haematology in current exacerbation depicted Hb of 10.9 g/dl, platelet count of 1,06,000/mm3, WBC of 2840/mm3, serum bilirubin of 1.5 mg/dl, serum ceruloplasmin of 3.60 mg/dl (20-60) mg/dl, and serum copper of 68 mcg/dl (80-155), 24-hour urine copper excretion of 95 (20-50) mcg/dl. On ophthalmologic examination, bilateral Kayser–Fleischer rings were visible, MRI findings showed hypo echoic regions in basal ganglia and the ultrasonography of abdomen showed hepato-spleenomegaly.\nPatient was currently on zinc acetate 250 mg/d, penicillamine 600 mg/d, olanzapine 5 mg/d, and trihexyphenydyl 4 mg/d. In the current episode, the neurologist had tried to treat the psychotic symptoms with 6 mg of risperidone with 4 mg of trihexyphenydyl; however, the patient had an episode of severe neck dystonia after which he was shifted to 10 mg of olanzapine, which was reduced to 5 mg after disturbances experienced by patient due to the extrapyramidal symptoms.\nThe patient was diagnosed with Wilson's disease 4 years ago after confirmation by laboratory investigations and ophthalmological examination by a neurologist and treatment with penicillamine. The first symptoms to develop were drooling of saliva with rigidity of limbs and withdrawn behavior and wandering aimlessly. The patient was managed by neurophysician with penicillamine 500 mg/d in divided doses and control over both neurological and psychiatric symptoms was obtained by it. No psychiatric liaison was required.\nThe dilemma was that, presently, there was exacerbation of psychiatric symptoms; however, poor control by antipsychotic medications and worsening of extrapyramidal symptoms on olanzapine even at low dose of 5 mg/day with 4 mg of trihexyphenydyl. It was decided to treat the current psychiatric manifestations with electro convulsive therapy (ECT) and discontinue all antipsychotics. He was referred to the medical side and a high-risk consent from the family members was obtained to start ECT in the patient. A course of 6 ECT was planned and administered in the patient. The patient showed 25-30-sec seizure response in all ECT sessions which were delivered as per the calculated dose of ECT, administered thrice weekly on alternate days. No prolonged seizure or post-ictal confusion as mentioned in previous case reports was observed. Later, the patient was given 4 ECTS, twice weekly, and 4 ECT once a week, as maintenance ECTs and, with complete resolution of psychiatric manifestations, ECTs were discontinued without starting any antipsychotic medication. The patient was continued on penicillamine and zinc for control of neurologic and other manifestations of Wilson's disease.\nIt has been reported that, in Wilson's disease, the psychiatric manifestations may precede neurological signs in the early stages of Wilson's disease. This patient had consulted us after being a defaulter in the treatment of Wilson's disease from a neurologist. The current scenario had more of psychiatric problems than neurologic. The visible extrapyramidal symptoms were worsened with antipsychotics. However, by discontinuing the antipsychotic treatment, there was an exacerbation of the neuropsychiatric symptoms. With references to case reports from previous data, it was decided to start a course of 6 ECTs in the patient, which is a routine course of therapy followed in our hospital for psychotic patients. We also went a step ahead to continue with the maintenance ECTs in this patient and preferred not to stabilize him on any oral antipsychotic drug due to poor outcomes in the past 6 months. The patient was followed-up regularly and, after omission of ECTs and continuing regular treatment from neurologist, the patient showed no active symptoms either from psychiatric side or any other domain of Wilson's disease. ECT in control of acute psychosis of Wilson's disease is an encouraging option, especially in patients who have extrapyramidal symptoms along with psychosis as neuropsychiatric symptoms of Wilson's disease. This adds to the long list of managing psychosis of non-psychiatric origin by ECT: intractable epilepsy, multiple sclerosis, parkinson's disease, dementia, neuroleptic malignant syndrome, space occupying lesions, movement disorder, normal pressure hydrocephalus, and encourages us to increase the use of ECT and refrain from any hesitation and stigma related to this time tested therapy.[] Treating psychosis of organic origin with ECTs is a safer and better option whenever there is poor response to antipsychotics.
Electro Convulsive Therapy in Psychiatric Manifestations in Wilson's Disease
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A 59-year-old woman with a severely atrophied right mandible was treated with the sandwich osteotomy technique filled with autologous bone graft harvested by a cortical bone collector from the ramus.\nThe requirements of the Helsinki Declaration were observed, and the patient gave informed consent for all surgical procedures. After local infiltration of anaesthesia (mepivacaina plus adrenaline 1:200,000), buccally and lingually to the defect area, a single vertical incision was initiated at the distal margin of the mesial tooth (43) to the defect. The second incision was carried out distally about 3 cm far from the first (Fig. ). The soft tissues were elevated from the bone through the tunnelling mechanism cranially, mesially and distally in a subperiosteal plane. The elevator of Zucchelli (Stoma®—Storz am Mark GmbH, Emmingen-Liptingen, Germany) was used for the subperiosteal dissection.\nHowever, the entire periosteum could be preserved to ensure adequate vascularisation of the future bone cranial segment.\nA horizontal osteotomy of the edentulous mandibular bone was then made with a piezoelectric device (Mectron Medical, GE, Italy). The tip MT1-10 was used to perform the osteotomy. The segmental mandibular sandwich osteotomy (SMSO) was finished by two (mesial and distal) slightly divergent vertical osteotomies (Fig. ). The horizontal osteotomy was located at least 2 mm below the ridge bone and approximately 2 mm above the mandibular canal. The vertical mesial osteotomy was made 2 mm distal to the last tooth and 2 mm above the mental foramen. Also, the mesial vertical muco-periosteal incision is necessary to place the incision 2 mm distant from the mental foramen. The bone fragment remains anchored to the lingual and crestal periostea. The entire bone fragment was displaced cranially, and the desirable position was obtained.\nThe length of the segments was matched to the deficient, resorbed alveolar ridge. The segment was displaced crestally to the desired three-dimensional place and fixed with 0.8 mm thickness, pure titanium, L-Plate with 2.4 mm titanium matrix mandible cortex screws—self-tapping tip (Synthes GmbH Eimattstrasse, Oberdorf, Switzerland; Fig. ). The gap was filled completely with autologous bone chips harvested from the mandibular ramus by a cortical bone collector (Safescraper Twist, Meta, Italy). No barrier membranes were used to protect the grafts. The vertical incisions were closing with interruptive suturing of the flaps with a resorbable material (Polysorb 3-0, Covidien LLC, MA, USA). In this way, the suture will not fall on the osteotomy line of the jaw; the result will be a better predictability of soft and hard tissue healing (Fig. ). Orthopantomography (OPG) was performed immediately after the procedure (Fig. ).\nThe sandwich technique for bone augmentation of the atrophic mandible was first described by Schettler and Holtermann, with promising results. Since then, variations in this surgical procedure have been proposed by several investigators [–].\nAll these authors have proposed the same approach about the flap: paracrestal incision. In order to preserve the blood supply, it is of fundamental importance that the least number possible of the vessels of the soft tissue be damaged. Based on this concept, we believe that changing the flap design can obtain the improvement of the healing of the wound. The sandwich osteotomy with the tunnel technique meets these requests. This is because the incisions were only two and vertical in the buccal side. Another advantage, especially with respect to implants, is that vascularisation is maintained in the bone ridge throughout augmentation intervention; thus, the interface at the implant shoulder in terms of hard-to-soft tissue to implant interface is kept as true to the original as possible.\nThis technique should be applied in patients with at least 5 mm of minimal crestal amount of bone above the nerve to perform the sandwich osteotomy successfully.\nWe prefer to obtain not more than 5 mm of the vertical movement for the sandwich graft. Efforts to displace the segment greater than 5 mm not only risk the potential for vascular embarrassment by detaching periosteal blood supply but also can excessively rotate the segment palatally, compromising aesthetic gingival projection.\nWe observed no signs of impaired sensibility after the sandwich osteotomy technique. Jensen found transient paraesthesia in all patients, lasting up to 6 weeks [].
Segmental sandwich osteotomy and tunnel technique for three-dimensional reconstruction of the jaw atrophy: a case report
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The case was a 5-year-old-girl, who was admitted to the hospital with a 2-year history of intermittent convulsions. She was born to healthy, unrelated parents. Her mother had slight vaginal bleeding for one week at the three months pregnancy without any therapy. She appeared normal at birth at 40 + 1 weeks of gestation and had normal Apgar scores. Birth weight, length, and head circumference were within normal ranges. Family history was unremarkable for neurologic diseases. Her psychomotor development was delayed. Her parents reported that she sat at eight months, and walked with support until 18 months. She suffered from bruxism and characteristic stereotyped hand movements such as clapping and tapping started at the age of 2 years. At the age of 3 years, she developed seizures characterized by staring eyes, hands rubbing and hypokinesis of the head (10 times per day), severely associated with lip and face cyanosis (1 time per day). Her parents reported that her EEG in a specific hospital showed abnormality at three years of age, and the seizures were not adequately controlled after one year of Chinese medicine therapy. Thus, they gave up drug therapy. In the last year, another abnormal behavior was observed. She regularly tapped her lips using the right hand, and bed using the left hand in awake and stopped until sleep. In the last month, the frequency of seizure increased significantly, and seizure symptoms developed more serious. So her parents took her to our hospital for comprehensive examination. She was diagnosed with RTT from her history and clinical findings. A cerebral magnetic resonance image was normal. Mutational analysis of MECP2 revealed a mutation in this affected girl. Twenty-four-hour video-EEG recordings revealed a slowing advantage rhythm (5-6 Hz) of occipital region and extensive slow wave activity in the background activity. Besides, multifocal discharges were noted during awake, especially in the Rolandic region, significantly aggravated to evolution toward electrical status epilepticus during sleep (ESES). Several partial seizures and constantly-tapping her lips using right hand were recorded during EEG recordings, which was the same as that described by the parents. To our surprise, tapping- lips in light and slow rhythm could trigger extensive low amplitude fast wave rhythm (Fig. ), and relatively stronger and quicker rhythm could result in multifocal bilateral discharges (Fig. ). Corresponding discharges were disappeared when the movement was intervened by parents. The patient chose left hand to rhythmically tap lips when the right hand was restricted, but there were no synchronic discharges accompanied by the action. Moreover, the synchronous phenomenon of action and discharge was not observed when the right hand was tapping cheek or abdomen. More interestingly, the discharges were not precipitated by tapping- lips using observer’s hand at the certain tempo and intensity. The coexistence phenomenon persisted in different EEG caps, instruments, and rooms, which ruled out the possibility of an artifact. By the phone call following-up, the total frequency of epilepsy seizures was markedly less after one year of treatment with valproate, but hand stereotypies did not respond to the antiepileptic drug.
Tapping-lips aggravated interictal bilateral discharges in EEG in the patients with Rett syndrome: a case report
Summarize this medical case report, ensuring to cover the initial presentation
A 49-year-old woman with B-rapidly accelerated fibrosarcoma (BRAF) mutant metastatic melanoma was treated initially with multiple surgical resections. She subsequently presented with symptomatic, unresectable brain metastases and limited extracranial disease in the subcutaneous tissues and peritoneum. She received oral steroids and whole brain radiotherapy, followed by pembrolizumab (2 mg/kg intravenously, planned for three weekly cycles). A baseline CT scan demonstrated likely hepatic steatosis, but no malignant liver infiltration.\nEight days after the first infusion of pembrolizumab, she presented to the emergency department with jaundice. Liver function tests were markedly deranged (bilirubin 90 μmol/L, alkaline phosphatase (ALP) 237 U/L, gamma-glutamyl transpeptidase 2094 U/L, AST 961 U/L and ALT 1536 U/L, with a normal prothrombin time). An ultrasound scan suggested hepatic steatosis, but did not demonstrate any focal lesions, biliary dilatations or vascular abnormality. A full liver screen excluded infectious and metabolic aetiologies (including hepatitis B, hepatitis C, cytomegalovirus (CMV), Epstein-Barr virus and adenovirus infection, α1-antitrypsin deficiency, Wilson’s disease and haemochromatosis) and an autoantibody screen (including antinuclear, antimitochondrial, antismooth muscle, and anti-liver kidney microsomal antibodies) was unremarkable. Serum immunoglobulins revealed mild hypogammaglobulinaemia (5.59 g/L), with normal IgA and IgM levels. There were no recent medication changes except for the introduction of pembrolizumab.\nDespite immediate treatment with prednisolone (~1 mg/kg once daily orally) for presumed autoimmune-type hepatitis secondary to pembrolizumab, the patient’s serum bilirubin and ALP continued to worsen (see for liver function tests over time, and a summary of treatment types and their durations) and she proceeded to a diagnostic liver biopsy. This demonstrated diffuse steatosis with steatohepatitis and only a single small bile duct evident on H&E staining despite portal tracts being well represented and the sample measuring 14 mm in length (). Cytokeratin 7 (CK7) immunohistochemistry also failed to demonstrate bile ducts and showed only a very minimal and focal intermediate hepatobiliary phenotype (). Copper-associated protein (a feature of prolonged cholestasis) was not identified, and typical autoimmune hepatitis-like features were absent. The findings were felt to be consistent with a relatively acute-onset vanishing bile duct syndrome. The patient was commenced on ursodeoxycholic acid (UDCA) after the diagnosis was made.\nGiven no improvement in serum ALP or bilirubin with corticosteroids and UDCA, MMF was added at a dose of 1g twice daily orally. Although ESMO guidelines recommend alternative escalation to 2mg/kg dose of (methyl)prednisolone for immune-related hepatitis, this was not felt appropriate given the appearances of the liver biopsy. After 56 days, MMF was then stopped owing to a lack of improvement in liver function and profound neutropaenia. The patient also developed insulin-dependent diabetes mellitus, likely steroid induced, as well as a remarkably high serum cholesterol (40.7 mmol/L) that was unresponsive to statin treatment. Fat-soluble vitamin depletion was observed with an undetectable serum vitamin D level (presenting with profound symptomatic hypocalcaemia with a corrected serum calcium of 1.4 mmol/L), a serum vitamin K level of 0.09 μg/L and a protein induced by vitamin K absence/antagonist-II of >10 AU/mL (accompanied by a rising international normalised ratio (INR)). The low serum calcium and the elevated INR were considered to be a consequence of cholestasis and responded completely to oral vitamin D and K supplementation. The patient suffered a wedge fracture at T11, thought to be a consequence of prolonged steroid use and/or vitamin D depletion.\nEight weeks after receipt of the single pembrolizumab infusion, the patient was offered BRAF-targeted treatment. Since both dabrafenib and trametinib are metabolised by the liver and excreted in the bile, dabrafenib was initially introduced at 75 mg once daily (25% standard dose), and increased after demonstration of tolerance to 75 mg twice daily after 2 weeks. A partial response in the extracranial disease, but a mixed response in the intracranial disease was observed. Trametinib was then added at a dose of 1 mg once daily, with further partial response in the extracranial disease, and mixed response in the intracranial disease. Both drugs were escalated to full treatment doses (dabrafenib 150 mg twice daily and trametinib 2 mg once daily). These doses were well tolerated, and the hepatic dysfunction slowly began to improve with time and ongoing steroid/UDCA treatment. The patient ultimately died from progressive intracranial disease almost 8 months after presenting with vanishing bile duct syndrome, and 6 months after starting BRAF-targeted treatment.
Severe steroid-resistant anti-PD1 T-cell checkpoint inhibitor-induced hepatotoxicity driven by biliary injury
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A 33-year-old woman with the dizziness as a chief complain. Magnetic resonance imaging (MRI) found a sellar lesion with a diameter of 18 mm invading into the bilateral cavernous sinus []. The physician suspected it to be a pituitary adenoma. A normal pituitary gland was seen on top of the tumor. The posterior gland was found superoposterior to the tumor. Despite the tumor’s lack of impingement to the optic apparatus, she was recommended to have regular visits but were lost to follow-up later. Six years later, she noticed bilateral visual disturbance and visited the clinic. MRI showed an extensive growth of the tumor.\nOn admission to Kagoshima University Hospital, the patient had clear consciousness. Her vision was 0.3 in the right and 0.7 on the left. Bitemporal hemianopia was found on perimetry. Her serum sodium (Na) level was 127 meq/L. MRI showed a large tumor with a maximal diameter of 56 mm, involving the sellar, suprasellar, retroclival, and bilateral cavernous sinus regions and strongly compressing the optic nerve and optic chiasm []. The tumor was slightly hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI compared to white matter. The bilateral internal carotid arteries were completely encased. A postgadolinium scan showed a heterogeneous enhancement. The relatively well- enhanced thick bundle suggested that the pituitary gland was located in the upper left part of the tumor. A computed tomography (CT) scan showed that the tumor was slightly hyperdense []. Neither bleeding nor calcification was seen. The sellar floor was remarkably thickened.\nThe assessments of the anterior pituitary hormonal function showed hyperprolactinemia (53.9 ng/mL) and growth hormone deficiency, but the other four hormonal axes showed normal secretory functions. There was no manifestation of diabetes insipidus.\nDecompression surgery of the optic chiasm was conducted through an endoscopic transsphenoidal approach. The removal of the thickened sella and thinned fibrous tissue presumed to be a remnant of the dura mater revealed the tumor, which was basically fibrous and easy to bleed. Tumor removal was continued until the suprasellar arachnoid membrane and tough tissue consistent with a pituitary gland appeared, leaving the tumor in the cavernous sinuses. Postoperative MRI demonstrated the sufficient decompression of the optic chiasm and an intact pituitary gland []. Postoperatively, a significant improvement of visual field deficit and normalization of vision (1.2 in both) was noted. The anterior pituitary function did not worsen. The blood prolactin level normalized (11.2 ng/mL).\nPathologically, the tumor was composed of a proliferation of round to oval neoplastic cells with scant to moderate cytoplasm and chromatin-rich short rod-like nuclei against a background of intercellular neuropil-like fibrillary matrix []. Cellular pleomorphism was mild. There were a few ganglion cells accompanied by elongated cells. These cells were partly packed, showing a lobular arrangement separated by a fibrovascular stroma []. The neoplastic cells and fibrillary matrix were immunohistochemically positive for neuronal markers, including synaptophysin [], neurofilament protein [], neuron-specific enolase, and NeuN [] chromogranin A []. The neoplastic cells were also positive for arginine vasopressin (AVP) []. The S-100 protein [] and vimentin were positive, mainly in spindle cells surrounding the fibrovascular stroma. They were negative for any anterior pituitary hormones, epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP), or thyroid transcription factor-1 (TTF-1). These pathologic findings were suggestive of sellar neuroblastoma. The MIB-1 index was 6%.\nA postoperative whole-body CT scan revealed a right ovarian cyst, which was later pathologically proven to be a simple cyst; otherwise, no other abnormalities were found. She underwent 50-Gy of intensity-modulated radiation therapy to the residual tumor due to hospital’s standard operating procedure, which has controlled its growth for the 3 years []. The serum Na levels remained low postoperatively at 126–130 meq/L, which did not cause any disturbance in consciousness. She was diagnosed with the syndrome of inappropriate secretion of antidiuretic hormone (syndrome of inappropriate antidiuretic hormone secretion [SIADH]) at 12 months after surgery. The laboratory results were as follows: serum Na: 130 meq/L, osmolarity: 259 osmo/kg, AVP: 18 pg/mL, urinary Na: 96 meq/L, and urinary osmolarity: 507 osmo/kg. The patient had no hypoadrenocorticism, kidney dysfunction, and cardiac failure. She is currently working in a factory without neurological impairment or hormonal replacement.
A sellar neuroblastoma showing rapid growth and causing syndrome of inappropriate secretion of antidiuretic hormone: A case report
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A 64-year-old man, a Maasai from Ngorongoro District, Arusha Region, northern Tanzania, presented to our facility in February 2020 with chief complaints of chest pain and cough for the past 3 years. He further reported that, at times, he experienced a sore throat, blood-stained sputum, pain that was greater on the right side of the chest, and intermittent low-grade fevers. The patient also reported significant unintentional weight loss and generalized body weakness. He had been consuming local herbs with occasional relief and was unable to afford modern hospital care expenses. Despite using traditional medicines, he had no notable improvements. He had no significant past medical history, denied tuberculosis contact, and consumed local brew. He denied any formal education and was a farmer and livestock keeper. Upon examination, he was cachexic with mild conjunctival pallor and saturating at 97% on room air. His axillary temperature was 36.4 °C; his pulse rate was 95 beats per minute; and his blood pressure was 100/70 mmHg. His chest examination revealed reduced air entry on the right side. His abdomen was flat and moved with respiration with traditional marks on the upper quadrants, with no tenderness, and with a liver span of 10 cm. The finding of his neurologic examination was unremarkable. His hemoglobin level was 12.8 g/dl with an erythrocyte sedimentation rate (ESR) of 110 mm/hour. His creatinine level was 83 μmol/L, serum urea was 4.14 mmol/L, aspartate aminotransferase was 17.52 U/L, alanine aminotransferase was 23.78 U/L, and serum electrolytes were within normal range.\nA CT scan of the patient’s thorax showed a massive cyst in the right lower lung measuring 19 × 11 cm with well-defined margins. A working diagnosis of bronchogenic cyst was reached with the possibility of hydatid cyst. Ultrasonography excluded the presence of the disease in the abdominopelvic region.\nWe performed a right-sided sixth-interspace thoracotomy. Intraoperatively, we found a large hydatid cyst occupying the middle and lower lobes. During blunt dissection, the cyst ruptured, but the middle and lower lobes were successfully removed (Fig. ). Approximately 30 minutes after surgery, the patient died in the intensive care unit (ICU) of sudden cardiac arrest. The probable cause of death we speculate to be anaphylaxis triggered by leakage from the ruptured cyst. The specimen taken intraoperatively was analyzed and confirmed to be a hydatid cyst of the lung (Fig. ).
Giant isolated hydatid lung cyst: two case reports
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A female patient aged 17 years reported to Faculty of Dentistry, SEGI University for a routine dental check-up. The patient was of Chinese ethnicity and was accompanied by her mother. Patient's medical history revealed underlying Williams syndrome. Detailed history, extra-oral examination, intra-oral examination, and radiographic evaluations were performed. Extra-oral examination revealed the long face, broad forehead, wide cheeks, underdeveloped mandible, pouting lips, and widely spaced eyes. The characteristic “elfin facies” seen in Williams syndrome was noticed []. Intra-oral examination revealed Angle's class III malocclusion []. The mandibular right lateral incisor was screwdriver shaped [Figure and ]. Plaque and calculus deposits, especially in the mandibular anterior region, deep pits and fissures and dental caries, was noticed in maxillary left and right second molars []. Intra-oral periapical radiographs were taken which showed generalized widening of the periodontal ligament space [Figure –]. Pulp vitality tests revealed that the teeth were vital. No history of trauma was reported. A treatment plan involving oral prophylaxis, pit and fissure sealants, and restorations for carious teeth were planned for the patient. Due to the various systemic complications associated with Williams syndrome, the patient was referred to the physician for medical opinion and consent for the above mentioned dental treatment.\nPatient's physician provided the medical consent for dental treatment, along with the medical history of the patient. Williams syndrome was diagnosed by FISH test 7q11.2 deletion. The patient had a history of epilepsy for which she was taking anticonvulsants. However, anti-convulsants were stopped since 2 years in the patient. Ultrasound examination of the kidney and the neural artery Doppler studies were also normal in the patient. The patient had a history of transient hypertension, but her blood pressure was normal without any medication. The patient had some learning difficulties but was otherwise very independent, cheerful and friendly with good self-esteem.\nThe treatment needed for the patient was explained, and informed consent was obtained from the patient. Oral prophylaxis, composite restorations in maxillary right and left second molars, and pit and fissure sealant in mandibular right and left first molars were performed in the patient. The patient was advised for regular follow-up and an orthodontic consultation for malocclusion.
Dental management of patient with Williams Syndrome - A case report
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A 52-year-old female was admitted with complaints of quadriparesis and slurring of speech for 2 days. She was a known hypertensive and was on treatment with beta-blockers. Her history was not suggestive of any medications or conditions which could have caused severe hypokalemia.\nAt presentation, she was normotensive with a heart rate of 60/min and electrocardiography changes suggestive of hypokalemia with U-waves []. Laboratory results showed severe hypokalemia (1.5 meq/L) and arterial blood gases revealed picture of metabolic acidosis with pH 7.22 and serum bicarbonate 15.2 mmol/L. Laboratory data showed serum chloride 111.3 meq/L, magnesium 2.65 meq/L, albumin 3.80, globulin 3.90, and an anion gap of 14. Further workup revealed urine pH 5.7, a normal 24 h urinary potassium concentration of 40.36 mmol/day, raised 24 h urinary calcium of 487 mg/day, and normal serum cortisol.\nHypokalemia was corrected by potassium supplementation with monitoring of serum potassium 12 hourly. Thyroid profile showed hypothyroidism with elevated thyroid-stimulating hormone and thyroid antibodies level of 28.9 meq/L, suggesting an autoimmune thyroid disorder.\nAn autoimmune antibody profile was sent to investigate the cause of distal renal tubular acidosis (DRTA) and it showed positive antinuclear antibody (ANA) of 1:80 and strong positive for SS-A, Ro-52 and mild positive for Ds-DNA. Further investigation into patient's history and clinical examination revealed complaints of dry eyes and mouth in the past and parotid swellings bilaterally. Biopsy of parotid could not be done due to refusal of consent by the patient. Based on the ANA profile, clinical history, parotid involvement, metabolic acidosis with severe hypokalemia, and autoimmune thyroiditis, a diagnosis of Sjogren's syndrome causing DRTA with autoimmune thyroiditis was made. The patient responded well to the treatment with correction of potassium and supplementation of intravenous bicarbonate, showing marked improvement of weakness and speech.
Secondary Sjogren's Syndrome Presenting with Distal Tubular Acidosis and Quadriparesis
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A 69-year-old man (height, 170 cm; weight, 80 kg) was scheduled for repeated arthroscopic lavage of the right knee for the treatment of unimproved septic arthritis in spite of the previous procedure that had been carried out 2 weeks previously. At the evaluation done 2 weeks ago, it was found that he had peripheral arterial occlusive disease in the lower extremities, but there was no evidence of deep vein thrombosis. He was being treated for hypertension and type 2 diabetes. During the first arthroscopic lavage under general anesthesia, new onset atrial fibrillation with a rapid ventricular response was noted. The transthoracic echocardiography done at that time showed left atrial enlargement, normal left ventricular function with an ejection fraction of 61.3% and there was no evidence of intracardiac thrombus. Flecainide and aspirin were prescribed for the atrial fibrillation.\nBefore the second operation, the preoperative electrocardiogram showed normal sinus rhythm. Intramuscular injection of glycopyrrolate 0.2 mg was given as premedication. Anesthesia was induced with propofol and rocuronium and the airway was secured with a cuffed 7.5 mm endotracheal tube. The electrocardiogram, pulse oxygen saturation, capnogram and esophageal temperature were monitored. The invasive arterial pressure was also monitored because the vital signs were unstable during the previous operation due to atrial fibrillation and the septic condition. Anesthesia was maintained with sevoflurane and 50% nitrous oxide in oxygen. Arterial blood gas analysis that was done 10 minutes after induction of anesthesia showed pH 7.38, PaCO2 45.1 mmHg, PaO2 85.7 mmHg, HCO3- 26.0 mEq/L and SaO2 95.5% (FiO2: 0.5). The end tidal CO2 was 24 mmHg. The tidal volume was increased to 750 ml from 650 ml. Twenty minutes after changing the ventilator setting, arterial blood gas analysis showed pH 7.31, PaCO2 47.0 mmHg, PaO2 75.1 mmHg, HCO3- 23.6 mEq/L and SaO2 92.8% (FiO2: 0.5).\nPhenylephrine 100 µg was injected intravenously at 30 minutes after induction because the blood pressure started to drop to 90/60 mmHg and the heart rate increased to 100 beats per minute. Forty minutes after anesthetic induction when the surgical procedure was almost completed, the arterial blood pressure dropped to 65/45 mmHg with a heart rate of 48 beats per minute. Epinephrine 30 µg and then an additional 50 µg of peinephrine and atropine sulfate 0.5 mg were injected intravenously. However, the vital signs deteriorated and the pulsatile activity disappeared in the arterial line. Cardiopulmonary resuscitation (CPR) was immediately started and the surgery was completed. The nitrous oxide and sevoflurane were turned off. Epinephrine 1 mg and atropine sulfate 0.5 mg were administered intravenously. The vital signs were not improved in spite of intermittent administration of epinephrine. The arterial blood gas analysis showed pH 7.37, PaCO2 27.8 mmHg, PaO2 63.9 mmHg, HCO3- 15.8 mEq/L and SaO2 90.8% (FiO2: 1.0).\nEight minutes after starting CPR, external cardiac massage was stopped because the vital signs were restored. Arterial blood gas analysis showed pH 6.96, PaCO2 63.9 mmHg, PaO2 221.4 mmHg, HCO3- 13,9 mEq/L and SaO2 98.5% (FiO2: 1.0). Thereafter, with the vital signs fluctuating severely, the CPR was intermittently continued with the administration of epinephrine and sodium bicarbonate on the basis of the hemodynamic status and the results of the arterial blood gas analysis. Ten minutes after initiation of CPR, an emergency transthoracic echocardiogram was performed with the impression of pulmonary embolism and it showed a massive amount of thrombus in the right atrium and a dilated hypokinetic right ventricle with a D shaped left ventricle (). Under the preliminary diagnosis of massive pulmonary embolism, the decision was made to proceed with fibrinolysis while the CPR was ongoing. Thirty-seven minutes after the initiation of CPR, 20 mg of alteplase, which is a r-tPA (Actylase®, Boehringer Ingelheim, Ingelheim, Germany), was administered intravenously and 100 mg was continuously infused over 90 minutes. The echocardiogram after injection of r-tPA showed improvement of the D shaped left ventricle, but multiple, mobile thrombi in the right atrium were still present (). The hemodynamic performance of the systemic and pulmonary circulation was stabilized. There was no sign of bleeding at the operation site. The external cardiac massage was stopped 26 minutes after injecting the recombinant tissue-type plasminogen activator. The patient, who was in an intubated state, was transferred to the intensive care unit. As the patient was transferred to the intensive care unit, the blood pressure increased to 110/60 mmHg and the heart rate was 100 beats per minute, with mechanical ventilation. Heparin was given 12 hours after injecting the r-tPA. The PT was INR was 1.65 and the aPTT was 50-70 seconds. Eighteen hours later, the patient's consciousness became alert and the endotracheal tube was extubated.\nThe thrombi had completely disappeared and the dilated hypokinetic right ventricle had returned to normal, as was noted on the echocardiogram performed 3 days after surgery (). The heparin infusion was stopped 5 days after surgery and patient recovered completely without any hemorrhagic episodes. He was transferred from the intensive care unit to the medical ward.
Successful thrombolytic therapy with recombinant tissue-type plasminogen activator for massive pulmonary embolism -A case report-
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Mrs. 41-year-old, P2A1, underwent a laparotomy total hysterectomy due to adenomyosis. She suffered from abnormal uterine bleeding and dysmenorrhea VAS 3 for 4 months before admission. She felt palpable mass on the lower abdomen and had history of hospitalization for blood transfusion due to anemia of blood loss (Hemoglobin level 6.8 g/dl). On ultrasound examination, we revealed enlarged and globular uterus, diffuse adenomyosis with the diameter of 52 mm. During hysterectomy, the Obstetrics and Gynecologist (OB-GYN) performed bilateral salpingectomy to reduce the risk of having ovarian cancer.\nP (patients): Women underwent hysterectomy for benign condition or permanent contraception surgery I (intervention): Bilateral prophylactic salpingectomy C (comparison): No prophylactic salpingectomy O (objective): Ovarian cancer incidence.\nAmong women underwent hysterectomy for benign condition or permanent contraception surgery, can bilateral prophylactic salpingectomy reduce the incidence of ovarian cancer?
Prophylactic salpingectomy and ovarian cancer: An evidence-based analysis
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A 37 year-old United Arab Emaerite woman gravida 4, para 3, was referred to our emergency department, as a case of missed miscarriage following 14 weeks amenorrhea. The patient's obstetric history included three Cesarean sections last one 9 years back. The first Cesarean section was done at 7 month gestation due to placental abruption, the second was done for placenta previa and the third was done electively. The patient presented with history of lower abdominal pain and bleeding per vagina for the last 12 days. Vaginal examination revealed an enlarged uterus of 8 weeks size that is slightly tender on movement. The cervical os was closed with no vaginal bleeding. Trans-abdominal ultrasound in transverse scan (Figure ) shows enlarged uterus, empty uterine cavity and cervical canal and a gestational sac (4.57 × 4.36 cm) situated in the lower part of the anterior uterine wall at the site of the Cesarean section scar. In a longitudinal scan (Figure ) the sac is seen protruding through the anterior uterine wall and pushing towards the bladder with a thin layer of myometrium (0.8 cm thick) separating them. A non viable embryonic echo was observed inside the sac measuring 3.5 cm at Crown Rump Length (CRL) corresponding to 10 weeks plus three day gestation. No fluid was seen in the cul-de-sac. With these ultrasound criteria present an ectopic implantation in the previous Cesarean section scar was considered. The mother was counselled regarding the treatment options, but due the possibility of prolonged follow up period with medical treatment she preferred to go for surgical intervention.\nA laparotomy was performed with Pfannenstiel incision under general anesthesia. The bladder was adherent to the lower part of the uterus so the peritoneum was incised and the bladder was dissected down to the cervix. The gestational sac was seen bulging and thinning out the uterine wall anteriorly at the scar site (Figure ). Most of the scar was in fact disrupted except for a thin layer of fibro-muscular tissue. When this layer was gently incised the sac bulged out with its bluish color (Figure ). Approximately 4-5 cm intact gestational sac was delivered followed by a placental tissue. The implantation of the ectopic pregnancy caused dehiscence of the previous Cesarean section scar. A trial to identify the cervical canal by retrograde probing was not easy as there was a thin layer of uterine tissue separating the implantation site from the uterine cavity. When this layer was overcome a communication was created between the implantation site and the uterine cavity. The dehiscent scar was then repaired, haemostasis was assured and bilateral tubal ligation was done according the request of the patient and her husband. The estimated blood loss was less than 500 ml and there was no need for blood transfusion. The patient had an uneventful postoperative recovery and was discharged from the hospital on postoperative day 5.
Ectopic intramural pregnancy developing at the site of a cesarean section scar: a case report
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The patient is an 82-year-old Hispanic male with a past medical history of hyperlipidemia, hypertension, diabetes mellitus, and chronic kidney disease stage IV who presented to the emergency department complaining of bilateral upper extremity and lower extremity weakness and paresthesias for two weeks preceding admission. The patient had multiple falls recently after the onset of these symptoms. His symptoms had gotten to the point where he could not use his walker or even get up from a chair to walk. He denied headaches, diplopia, fevers, chest pain, shortness of breath, nausea, vomiting, dizziness, bowel incontinence, or bladder incontinence. He also denied any recent travel or consumption of undercooked meats. Vital signs were as follows: temperature of 36.8 °C, heart rate of 58 beats per minute sinus rhythm, respiratory rate of 14 breaths/min, blood pressure of 152/65, saturating 97% on room air. His physical examination was most significant for motor weakness of the bilateral upper and lower extremity, +3/5 on the right versus +4/5 on the left. Grip strength was tested in the upper extremity while dorsiflexion and plantarflexion were tested in the lower extremity. Pronator drift exam was negative.\nHis laboratory studies were not significant for any acute findings. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were mildly elevated and a complete blood count with differential (CBC with diff) and coagulation profile were within normal limits (Table ). Serology for cysticercosis IgG resulted as negative. CT scan of the head without contrast was negative for acute intracranial abnormalities. There was an old infarct detected in the left internal capsule region. Also noted were chronic right frontal cortical calcifications and anterior right temporal lobe calcifications that were thought to be related to an old infection or hemorrhage. CT angiogram of the head and neck revealed 60% stenosis at the left common carotid artery (CCA) origin and proximal left internal carotid artery (ICA). MR imaging of the cervical spine (c-spine) with and without contrast showed multilevel cervical spondylosis with severe C3-C4 and moderate to severe C6-C7 thecal sac stenosis. Subtle increased cord signals at C3-C4 were concerning for compressive myelopathy. A neurosurgical consultation was obtained and C3-C4 anterior spinal fusion with intervertebral cage placement was performed. MRI of the brain with and without contrast showed multiple subcentimeter parenchymal and extra-axial enhancing lesions in the frontal lobes and along the right sylvian fissure with associated calcifications concerning for active neurocysticercosis (Figure ). The largest lesion along the right insula measured 7 mm with signal characteristics of a small abscess. An Infectious Disease consult was obtained and the patient was started on albendazole and praziquantal by mouth for ten days in total with dexamethasone. His hospital course was uncomplicated with no seizure activity reported. He worked well with physical therapy postoperatively and his bilateral upper and lower extremity weakness improved.
An Asymptomatic Case of Radiologically Active Neurocysticercosis
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We describe a 23-year-old male patient who presented with right-sided painless neck swelling since one month. There was no history of trauma, fever, and systemic illness. The patient had no complains of weakness, dysphagia, and hoarseness of voice. On local examination, a single ovoid swelling was present in the right carotid triangle, anterior to the sternocleidomastoid, measuring 3 × 2 cms. Swelling did not move on deglutition or tongue protrusion. There was no loss of muscle power in all muscles, no signs of wasting, or neurological deficits.\nUSG neck showed a hypoechoic lesion seen at bifurcation of the carotid artery. Moderate central and peripheral vascularity was seen within the lesion. Findings were suggestive of a carotid body tumor/nerve sheath tumor.\nCECT neck revealed a well-defined, well-encapsulated, peripherally enhancing soft tissue mass lesion of size 3.4 × 1.8 cm (). The mass lesion was noted in the right carotid sheath between the right common carotid artery and internal jugular vein, posterolateral to the right lobe of thyroid. The lesion was seen below the carotid bifurcation. Features were suggestive of a vagal schwannoma.\nThe tumor was approached by an anterior approach along the medial border of sternocleidomastoid. Platysma and fascia were dissected to reach the tumor. There was a medium-sized globular smooth mass, well-encapsulated seen engulfing the vagus nerve (Figures –). Extracapsular tumor excision was done in toto by conventional technique. Vagus was severed in the process. Vagus was reanastomosed, using the sural nerve graft ().\nPostoperatively, the patient had mild dysphagia which resolved in two days. The patient reported a minimal change in voice. There were no signs of aspiration. Postoperative videolaryngoscopic assessment revealed right vocal cord palsy. The patient's voice improved with speech therapy.
Head and Neck Schwannomas: A Surgical Challenge—A Series of 5 Cases
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A 24-year-old Tanzanian woman, primigravida at the gestational age of 26 weeks, was referred from another nearby facility with a one-month history of persistent abdominal pain associated with long standing recurrent vaginal bleeding during the antenatal period. She had been admitted repeatedly at a local community hospital due to abdominal pain. She denied any history that would suggest previous pelvic inflammatory disease, intrauterine contraceptive device use or pelvic surgery. During her last admission, abruptio placenta was suspected, and the patient was unsuccessfully induced twice with oxytocin without knowing the status of the fetuses. She was subsequently transferred to our hospital for further evaluation and treatment.\nOn arrival, physical examination revealed a conscious woman in severe pain, pale, afebrile. Her abdominal examination was notable for distended abdomen consisted with uterine fundus of 30 cm, generalized tenderness was elicited on palpation and fetal heartbeats could not be detected by fetoscope. Vaginal examination revealed a closed, uneffaced, firm and posterior cervix, and the presenting part was not found. Transabdominal ultrasound revealed live intrauterine diamniotic–dichorionic twins, estimated gestational age was 26 weeks and there were enlarged cystic placentas suggestive of retro-placental clots.\nLaboratory investigation revealed hemoglobin level of 7.0g/dl, the blood group A, and Rhesus positive.\nThe diagnosis of concealed abruptio placenta with live twin pregnancy was reached, and the patient was counseled and an emergency abdominal delivery was performed.\nAt laparotomy, the findings were: A thickened peritoneum with area of hematoma resembling a cystic mass, and a normal-sized intact uterus separate from the pregnant sac which was engulfed by thickened omentum. Both fallopian tubes and ovaries were grossly normal. Live female twins were extracted from the gestational sacs. Two placentas were seen, one attached to the ceacum and ascending colon and the other firmly adhered to the posterior aspect of the omentum, the transverse and the sigmoid colon. The placenta attached to the ceacum and ascending colon developed profuse bleeding during the delivery of the babies, requiring ligation of placental blood vessels to control bleeding. Both placentas were left in situ with their umbilical cords cut to 2cm long. Estimated blood loss was 2000ml; the patient was subsequently transfused 4 units of whole blood. The first twin weighed 700 g and the other 800g, each with Apgar score of 5 at first minutes and 6 at fifth minute. Grossly, both fetuses had no congenital abnormalities and were admitted to the neonatal intensive care unit due to prematurity []. Unfortunately, both babies died within the first week of life.\nPostoperatively, the patient was covered with broad spectrum antibiotics and admitted to the intensive care unit. Three days later she developed septicemia which was successful treated with meropenem. The patient improved and was discharged on postoperative day 14, with plan for outpatient follow-up in one month.\nFourteen days after discharge, the patient was readmitted with peritonitis which necessitated repeat laparotomy and attempt to remove the placentas provoked massive hemorrhage which was controlled by intra-abdominal packing and the patient was transfused with 3 units of blood. The abdominal packs were removed after 48 hours with no active bleeding from placental site. Eight days after removing the packing, the patient developed fascial dehiscence and was taken to operating room for exploration, where both placentas were easily detached without provoking bleeding. Her postoperative recovery thereafter was unremarkable, and she was discharged to attend outpatient clinic. After three visits of 11 months, she was discharged from the clinic in a good condition.
Managing a Live Advanced Abdominal Twin Pregnancy
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This was a 38-year-old male with a history of injury to the right eye with a stick a week earlier. The patient was diagnosed to have a corneal tear elsewhere and underwent a corneal tear repair. At the visit to our clinic, the presenting vision was counting fingers close to face. Anterior segment showed no view due to exudates and B scan confirmed exudates in the vitreous cavity. The patient underwent a prompt endoscopic vitrectomy with intraocular antibiotic injection. At the last follow up 8 month post-operative, the endophthalmitis had resolved with a final best corrected visual acuity of 20/100. The patient developed secondary glaucoma which was controlled with topical anti-glaucoma medications.
Can Prompt Endoscopic Vitrectomy in Post-Trauma Endophthalmitis with Corneal Edema Avoid Unnecessary Keratoplasties?
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A 42-year-old man was admitted with headache and diplopia of 2 days duration in December 2014. The feature of headache was persistent and electric shock-like peri-orbital pain on the right side, without nausea and vomit. He was engaged in fungus mushroom cultivation for 3 years. His medical history was insignificant. Neurological examination showed the right abducens nerve palsy. T2-weighted MRI revealed an heterogenous intrasellar mass (Fig. a). Contrast enhanced T1-weighted MRI showed the mass was of slight rim enhancement (Fig. b). Levels of endocrinological workup were all within normal limits. Before operation, headache could not be controlled by the drug of mannitol and NSAIDs except oxcarbazepin.\nWith a preoperative diagnosis of pituitary neoplasm, endoscopic trans-sphenoidal surgery was planned. After the diminished sphenoidal sinus was entered, some milk-like pus and thickened mucosa were excised. When the paper-thin sellar floor bone and dura were opened, more milk-like pus and a mass of ash black mixed and necrotic material were found and removed. The pituitary gland and the diaphragma sellae were found to be intact after the removal of the lesion. The specimens were negative for aerobic and anaerobic bacterial cultures. Histopathological examination of the necrotic material consisted of numerous hyphae, according with characteristic of aspergillus species (Fig. ). Plasma (1 → 3)-beta-d glucan determination was done and showed a elevated level (203.5 Pg/mL, normal, 0–100.5 Pg/mL). Itraconazole was given on a dosage of 200 mg twice a day orally for 6 weeks.\nPostoperatively he get a transient diabetes insipidus for 5 days. He had relief from headache and improvement in function of the right abducens nerve. No recurrence was observed on contrast enhanced MRI scans during 6-month follow-up.
Secondary headache due to aspergillus sellar abscess simulating a pituitary neoplasm: case report and review of literature
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A 64-year-old female with non-insulin-dependent diabetes, non-ischaemic cardiomyopathy, and previous history of hypertension and stroke was found to have non-sustained ventricular tachycardia on Holter monitoring, LBBB (QRSd 170 ms), LVEF 35%, and NYHA class III symptoms underwent attempted CRT. A Medtronic Attain Ability model 4196 LV lead was placed in a high lateral CS branch but a high pacing capture threshold was found. The implanter was unable to pass the lead into a lower (small) lateral branch and the middle cardiac vein had PNS distally with no capture when pulled more proximally. This LV lead was discarded and a St Jude QuickSite model 1056T LV lead was placed in the middle cardiac vein but a capture threshold >5 V was found distally and again no capture proximally. It was not possible to pass this lead into the high lateral branch. This lead was also discarded and no further attempts at LV lead implantation were made on this occasion. A Pacesetter St Jude Tendril model 1788T atrial lead was placed in the right atrial appendage and a Pacesetter St Jude Durata model 7120 was placed in the RV apex.\nThe patient returned to our institution 9 months after the original attempt at CRT for a further attempt at LV lead placement. A Quartet LV lead was successfully implanted in a postero-lateral branch of the CS (vector D1–M2; capture threshold 3.1 V at 0.5 ms pulse width; impedance 1344 Ω). The lead was attached to a Promote Q model CD3221-36 generator and placed in the old pre-pectoral pocket. Pacing parameters had improved at pre-discharge pacing check (threshold 1.75 V at 0.5 ms pulse width, impedance 510 Ω) and were better still at 6-week pacing check (threshold 1.25 V at 0.5 ms, impedance 540 Ω).
Use of a quadripolar left ventricular lead to achieve successful implantation in patients with previous failed attempts at cardiac resynchronization therapy
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A 47 year old female was admitted to hospital with severe progressive dyspnea for over one year, associated with pre-syncope and intermittent chest discomfort on minimal exertion. She was a WHO class 4 on presentation to hospital.\nHer past history was relevant for having a renal transplant at age 25. However, 6 years prior to this admission, she developed gastric lymphoma, necessitating stopping her immunosuppressive therapy. Ultimately, her transplanted kidney failed and she has an AV fistula inserted for dialysis.\nOn examination, her oxygen saturation was 98% on room air. Her respiratory rate was 14 breaths per minute. She had a loud P2 but no right ventricular (RV) heave or extra heart sounds. Her JVP was not distended, and there was no ascites, abdominal organomegaly or peripheral edema. She did have a large tortuous left brachial AV fistula (Figure ).\nGiven the suspicion for PH, a transthoracic echocardiogram (TTE) was completed showing a severely elevated RV systolic pressure at 101 mmHg. There was no intracardiac shunt on bubble study and the left ventricular (LV) ejection fraction was 62%.\nA right heart catheterization done on the morning of dialysis confirmed severe PH with a mean pulmonary arterial pressure (mPAP) of 85 mmHg (PH defined as >25 mmHg at rest). She had an elevated pulmonary vascular resistance (PVR) at 674 dyne.sec.cm-5. (PH: >240 dyne.sec.cm-5). Her pulmonary capillary wedge pressure (PCWP) was 14 mmHg (non-cardiac PH defined as <15 mmHg). Her cardiac output (CO) was elevated at 8.3 L/min (N: 4–7 L/min). Her LV diastolic pressure was 8 mmHg.\nWork up for PH was normal and included a CT chest, pulmonary function tests (normal except for low DLCO), V/Q scan, HIV, connective tissue disease (ANA, ENA, RF), liver profile, TSH, polysomnogram, abdominal ultrasound with Doppler flow and TTE with bubble study. She was deemed a WHO class 4. As she could not walk a few meters without assistance and had presyncope, we could not perform a baseline 6 MWD. Her brain naturetic peptide (NTproBNP) was elevated at 35,620 ng/L (normal: less than 125 ng/L).\nOur patient had severe pulmonary hypertension with normal LV function, along with an elevated CO. We measured flow through the fistula by sodium-dilution technique, and calculated the flow to be a minimum of 2 L/min. We proposed that the chronic high flow through her AV fistula likely resulted in pulmonary artery vascular remodeling and the development of PH.\nThe high CO and increased PVR were managed simultaneously by banding the fistula, decreasing its flow to 959 ml/min and prescribing oral Sildenafil 20 mg, three times per day. It was intended to start her on intravenous Epoprostenol but she improved within one week from a WHO class 4 to a WHO class 2.\nResults of a repeat right heart catheterization 4 months later correlated with her improved exercise ability. Her mPAP decreased to 47 mmHg, her PVR decreased to 422 dyne.sec.cm-5, her PCWP decreased to 10 mmHg and her CO was lower at 7 L/min. Her fistula was then compressed with a sphygmomanometer (140 mmHg for 15 minutes). The total CO decreased to 5.3 L/min but her mPAP and PVR remained unchanged, confirming a fixed vasculopathy. Follow up 6 MWD was 540 meters (90% predicted) and her NTproBNP decreased to 10 305 ng/L. Follow up TTE showed only mild RV dilatation, with mild tricuspid regurgitation and a reduced RV systolic pressure at 69 mmHg.
Pulmonary hypertension: Tortuous route to diagnosis
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An 82-year-old Caucasian man was referred to our unit from another local hospital in order to undergo capsule endoscopy. He presented with unexplained anemia, and previous upper and lower endoscopic examinations had failed to reveal any pathological findings. His medical history included arterial hypertension.\nThe patient swallowed the capsule in the presence of a physician without any evident swallowing difficulties. He then returned to the original hospital where the capsule endoscopy procedure was carried out. Our patient did not show any overt symptoms during the next day. The following day, the data recorder system was sent to our hospital to download the video.\nWhen we reviewed the capsule endoscopy images we realized that the capsule was located in the bronchial system and had remained there for the entire duration of the study (Figure ). We immediately contacted our colleagues from the other hospital who reported that our patient remained asymptomatic. An emergency chest X-ray confirmed the presence of the capsule in the left side of the bronchopulmonary tree (Figure ). We proposed a bronchoscopy but our colleagues preferred a wait-and-see policy since our patient was asymptomatic apart from a minimal cough, with pulsed oxygen saturation of 96% in room air. Although the X-ray pictures were clear, another upper GI endoscopy was performed to check if the capsule was in the esophagus; the procedure results were negative. Two days later a repeat chest X-ray showed the capsule in the right bronchus (Figure ). After two days the capsule was retrieved in the feces. Surprisingly, our patient remained asymptomatic during the entire admission period; he made an uneventful recovery and we decided not to repeat the capsule endoscopy procedure.
Asymptomatic bronchial aspiration and prolonged retention of a capsule endoscope: a case report
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A 43-year-old Caucasian gentleman with past medical history of homozygous hereditary hemochromatosis and pyruvate kinase deficiency leading to a splenectomy at 4 years of age was admitted to the Buffalo Mercy Hospital with a three-hour history of fever, chills, and generalized weakness. The patient denied any recent travel or sick contacts. The only recent change at home was the addition of a new puppy that infrequently bit and scratched him. The patient also reported that one week ago he had eaten home-cooked clams. Lastly, he swam in Lake Erie on many occasions over the past month. During that period of time, local health officials had closed off that particular beach several times because of elevated coliform levels.\nOn examination, he had a temperature of 39.5°C, heart rate 120 beats/min, blood pressure 80/50 mmHg, and respiratory rate 18 breaths/min. The rest of his dental, chest, abdomen, skin, and neurological exams including meningeal signs were unremarkable. A complete blood count revealed leukocytosis with bandemia (WBC 17.1, bands 28%) and macrocytic anemia (Hgb 8.3 g/dL, MCV: 129.5 dL) while a complete metabolic panel showed renal failure (BUN 18 mg/dL, creatinine 3.55 mg/dL) and liver failure (ALT 2500 U/I, AST 5000 U/I, total bilirubin 14.5 mg/dL, indirect bilirubin 10 mg/dL, and ALP 262 U/I). Additional laboratory findings included lactic acidosis (179.1 mg/dL) with ABG showing high anion gap metabolic acidosis. Electrocardiogram showed only sinus tachycardia. Transthoracic echocardiogram was unremarkable. Computed tomograms of head, chest, and abdomen were unremarkable. Two blood cultures and urine culture were performed. The patient was diagnosed with septic shock.\nAggressive volume resuscitation measures were started along with empiric coverage utilizing intravenous ceftriaxone and vancomycin. The patient developed respiratory failure soon after. Because of worsening renal failure, he also later underwent hemodialysis. On the second day of admission, the blood cultures returned and showed gram negative rods while the urine culture was negative. Total iron and ferritin were performed and returned 196 μg/dL and 1350.2 mg/mL respectively. Disseminated intravascular coagulopathy developed (PTT 74.2 seconds, PT 37.1 seconds, INR 3.2 U/I, fibrinogen 40 mg/dL, fibrinogen degradation products 60 μg/mL, and platelets 55) and the multiorgan failure continued to worsen. As the patient's condition continued to deteriorate, levofloxacin and tobramycin were added to the ongoing antibiotic therapy. Using VITEK2 for microbial identification and antibiotic susceptibility testing, the final blood culture identified the gram negative rods as Plesiomonas shigelloides. The organism was found to be susceptible to a multitude of different antibiotics including the patient's regimen of ceftriaxone, levofloxacin, and tobramycin (). Despite aggressive attempts at resuscitation, the patient's condition further worsened and he passed away on the third day of hospitalization.
Plesiomonas shigelloides Septic Shock Leading to Death of Postsplenectomy Patient with Pyruvate Kinase Deficiency and Hemochromatosis