A bleeding colonic ulcer from invasive Aspergillus infection in an immunocompromised patient: a case report.

INTRODUCTION: Invasive Aspergillus commonly involves the lungs, but can also affect other organs such as the skin, adrenal glands, central nervous system, liver, spleen and the gastrointestinal tract. Gastrointestinal aspergillosis is rare and is most often discovered in immunocompromised patients. There is only one other case report to our knowledge that describes the diagnosis being discovered on histopathological analysis of endoscopic biopsies of necrotic ulcers. CASE PRESENTATION: A 36-year-old Hispanic woman presented with septic shock secondary to extensive Fournier gangrene that required multiple surgical debridement of the perineal and retroperitoneal area. Her vital signs on admission were a temperature of 39.4°C and blood pressure of 85/56 mmHg, pulse rate of 108/min and respiratory rate of 25. An examination of the perineum/genital area revealed bilateral gluteal and perilabial edema, erythema and focal areas of necrotic tissue with purulent discharge. Other surgeries included small bowel resections with ileoileal anastomosis that later developed an anastomotic leak that required and diverting end ileostomy. Eleven weeks after admission, our patient developed hematochezia from the colostomy associated with a decrease in hemoglobin and hematocrit to 6.4 g/dL and 20.2% respectively. Colonoscopy through the ostomy revealed blood throughout the colon and a 3 cm necrotic ulcer with an adherent clot in the transverse colon. Biopsies were taken from the edge of the ulcer. Histopathological analysis of the specimen with Grocott's methenamine silver stain revealed septated hyphae with the 45-degree-angle branching that is morphologically consistent with Aspergillus species. Our patient was treated with intravenous voriconazole for 30 days with a prolonged hospitalization but no recurrent bleeding. CONCLUSIONS: Gastrointestinal aspergillosis is an unusual presentation of invasive Aspergillus associated with a high mortality rate. Characteristic features of gastrointestinal aspergillosis include invasion of the mesenteric arteries, intravascular thrombosis and subsequent tissue ischemia. Clinical manifestations of invasive Aspergillus of the gastrointestinal tract can include fever, abdominal pain, ileus, peritonitis, bloody diarrhea or hematochezia. In an autopsy series of patients with invasive Aspergillus, 37 of 107 patients had Aspergillus involvement of the gastrointestinal system; the most common pathological findings included ulcers and abscesses. Although rare, invasive aspergillosis may present with gastrointestinal bleeding associated with necrotic ulcers on endoscopic examination.

Rapid or normal gastric emptying as new supportive criteria for diagnosing cyclic vomiting syndrome in adults.

BACKGROUND: Cyclic vomiting syndrome (CVS) in adults is a disorder characterized by recurrent and stereotypic episodes of severe nausea, vomiting and abdominal pain separated by symptom-free intervals. Our goal was to investigate gastric emptying (GE) in CVS patients. MATERIAL AND METHODS: This was a retrospective study of 30 adult patients who met Rome III diagnostic criteria for CVS. Rapid GE was defined using two different predefined criteria as either <50% isotope retention or <65% isotope retention at 1st hour and/or <20% at 2nd hour. RESULTS: Of the 30 patients (25 had 4-hr GE) diagnosed with CVS, 22 were females and 8 males with a mean age of 39 years. Overall, 20 (80%) of the 25 CVS patients met the predefined criteria of <50% retention for rapid GE in the first hour. Fifteen (60%) met the 2-hour criteria for rapid emptying of <20% retention. Five (16.6%) patients of the 25 had a normal GE with a mean retention at the first hour of 65% (52-78%). Nine (36%) also met another predefined criteria of <35% retention for rapid GE in the first hour. Sixteen (64%) met criteria for normal GE. CONCLUSIONS: (1) In adult CVS patients, GE is either rapid or normal, clearly distinguishing this entity from gastroparesis. (2) Cyclic vomiting syndrome is an important new etiology to explain the finding of rapid GE on a radionuclide test. (3) We suggest that rapid gastric emptying should be added as supportive criteria for diagnosing CVS in adults.

Chorea, Hyperglycemia, Basal Ganglia Syndrome (C-H-BG) in an uncontrolled diabetic patient with normal glucose levels on presentation.

PATIENT: Female, 66 FINAL DIAGNOSIS: Chorea • hyperglycemia • Basal Ganglia Syndrome (C-H-BG) Symptoms: Hemibalism • hemichorea MEDICATION: - Clinical Procedure: - Specialty: Endocrinology and Metabolic. OBJECTIVE: Challenging differential diagnosis. BACKGROUND: Hemichorea-hemiballism (HCHB) is a spectrum of involuntary, continuous non-patterned movement involving 1 side of the body. Possible causes of HCHB include hemorrhagic or ischemic stroke, neoplasm, systemic lupus erythematosus, HHNK, Wilson's disease, and thyrotoxicosis. This case illustrates the need to be aware of hyperglycemia as a cause of hemiballism/hemichorea, which is now referred to in the medical literature as C-H-BG (chorea, hyperglycemia, basal ganglia) syndrome. CASE REPORT: A 66-year-old Hispanic woman presented to our care with hemiballism/hemichorea of the right arm and leg of 1 week duration. She had been admitted 3 months prior with toxic metabolic encephalopathy secondary to hyperosmolar hyperglycemic non-ketotic syndrome with a blood glucose level of 984 mg/dL. Her blood glucose level was normal but hemoglobin A1C was 12.2%. A brain MRI revealed an asymmetric T1 hyperintensity of the left putamen. This specific finding was compatible with hyperglycemia-induced hemichorea hemiballism syndrome. The hemiballism/hemichorea slowly improved over the course of the hospitalization with strict glycemic control. At the 3-month follow-up visit she had no involuntary movements of her extremities, and she had well controlled blood glucose levels and a hemoglobin A1C of 9.0. CONCLUSIONS: In a patient with normal glycemic levels but a history of uncontrolled diabetes, C-H-BG syndrome should be on the top of the differential list when the characteristic MRI findings of a hyperintensity in the basal ganglia are observed. This is a rare disease that deserves attention because it is reversible with correction of hyperglycemia. Thus, prompt recognition and treatment is essential to avoid adverse outcomes.

Hepatitis C- and HIV-induced porphyria cutanea tarda.

PATIENT: Male, 47 FINAL DIAGNOSIS: Porphyria cutanea tarda Symptoms: Chills • cough dry • thumb swelling MEDICATION: - Clinical Procedure: - Specialty: Metabolic Disorders and Diabetics. OBJECTIVE: Challenging differential diagnosis. BACKGROUND: Porphyria cutanea tarda (PCT) is the most common type of the porphyria. It occurs due to the deficiency of enzyme uroporphyrinogen decarboxylase (UROD), which is the fifth enzyme in the biosynthesis of heme and catalyzes the conversion of uroporphyrinogen to coproporphyrinogen. The risk factors for PCT include hereditary hemochromatosis, hepatitis C infection, ethanol abuse, estrogen use, HIV, smoking, chlorinated polycyclic aromatic hydrocarbons, and hemodialysis. CASE REPORT: A 47-year-old Hispanic man presented with right thumb swelling, redness, and pain for approximately 1 week. Past medical history included HIV/AIDS, hepatitis C infection, alcohol abuse, heroin abuse, and CMV retinitis. Skin examination revealed blistering and hypo/hyper pigmented lesions over the dorsal aspects of the hands and other sun-exposed areas. Serum porphyrins were discovered to be elevated. The quantitative urine porphyrins revealed elevation of uroporphyrins, heptacarboxyl-porphyrins, hexacarboxy-porphyrins, pentacarboxyl-porphyrins and coproporphyrin. Genetic mutation of UROD was not detected. Due to the classic cutaneous lesions, laboratory findings, and associated risk factors, we were able to confirm our suspicion of the sporadic (type 1) form of PCT. CONCLUSIONS: A strong correlation has been demonstrated between the sporadic (type 1) form of PCT and hepatitis C virus (HCV) infection in multiple studies. The mechanism through which HCV infection may cause or trigger PCT is unknown. PCT has been described for many years, but still eludes the differential diagnosis in a patient with cutaneous findings. The uniqueness of our case is the possibility that combined risk factors have an effect on PCT.

Pyridostigmine-induced high grade SA-block in a patient with myasthenia gravis.

PATIENT: Female, 70 FINAL DIAGNOSIS: SA block induced by pyridostigmine Symptoms: Asymptomatic Medication: Pyridostigmine Clinical Procedure: Pacemaker insertion Specialty: Electrophysiology. OBJECTIVE: Unusual clinical course. BACKGROUND: Myasthenia gravis requires a long-term treatment with a parasympathomimetic agent, which may result in bradycardia and asystole. Pharmacologic treatment with a reversible inhibitor of inosine monophosphate dehydrogenase (IMPDH) and Methylprednisolone is seen to improve the muscular symptoms but may reinforce potential bradyarrhythmias. This potential side effect can be treated with the levo isomer of atropine, Hyoscyamine, or Glycopyrollate in an intact conduction system. CASE REPORT: A 70-year old Caucasian female patient with a family history of myasthenia gravis presented with mild weakness of the bilateral facial muscles, moderate dysarthria, dysphagia, diplopia predominantly on the right side and difficulty tracking ocular movements bilaterally. The treatment with pharmacological agents was initiated. Subsequently she developed asymptomatic bradycardia and SA-block. An improvement on Hyoscyamine failed to appear. A dual chamber pacemaker was placed. CONCLUSIONS: In symptomatic or asymptomatic bradycardia with significant high grade SA-block in patients with myasthenia gravis the insertion of a permanent pacemaker can be the definitive solution.

Serum amyloid A renal amyloidosis in a chronic subcutaneous ("skin popping") heroin user.

BACKGROUND: Systemic AA amyloidosis is a long-term complication of several chronic inflammatory disorders. Organ damage results from the extracellular deposition of proteolytic fragments of the acute-phase reactant serum amyloid A (SAA) as amyloid fibrils. Drug users that inject drug by a subcutaneous route ("skin popping") have a higher chance of developing secondary amyloidosis. The kidneys, liver, and spleen are the main target organs of AA amyloid deposits. More than 90% of patients with renal amyloidosis will present with proteinuria, nephrotic syndrome, or renal function. CASE PRESENTATION: A 37 year-old female presented to the hospital with a one-week history of pain and redness in her right axilla. Her relevant medical history included multiple skin abscesses secondary to "skin popping", heroin abuse for 18 years, and hepatitis C. The physical examination revealed "skin popping" lesions, bilateral costovertebral angle tenderness, and bilateral knee swelling. The laboratory workup was significant for renal insufficiency with a serum creatinine of 5 mg/dL and 14.8 grams of urine protein per 1 gram of urine creatinine. The renal biopsy findings were consistent with a diagnosis of renal amyloidosis due to serum amyloid A deposition and acute tubulointerstitial nephritis. CONCLUSIONS: AA renal amyloidosis among heroin addicts seems to be associated with chronic suppurative skin infection secondary to "skin popping". It is postulated that the chronic immunologic stimulation by one or more exogenous antigens or multiple acute inflammatory episodes is an important factor in the pathogenesis of amyloidosis in these patients. Therefore, AA renal amyloidosis should always be considered in chronic heroin users presenting with proteinuria and renal impairment.