A Perfect Storm: Abdominal Pain and Ileus Explained by Acute Intermittent Porphyria Caused by Prehospitalization and Intrahospitalization Factors.

Acute intermittent porphyria (AIP) is a rare autosomal dominant inherited disease, predominantly seen in female patients, caused by mutations in the hydroxymethylbilane synthase gene. When impaired, elevated heme biosynthesis precursor levels accumulate in the liver, resulting in neurological symptoms, psychiatric disturbances, darkened urine color, abdominal pain, nausea, vomiting, and ileus. We present a 22-year-old Hispanic female with diffuse abdominal pain and no bowel movements for 8 days. She reported recent antibiotic and oral contraceptive pill use. Computerized tomography of her abdomen revealed a dilated small bowel and marked colonic distension. A colonoscopy found mild nonspecific inflammation in the rectosigmoid and terminal ileum. Her abdominal pain persisted despite interventions and improvements in appetite, bowel movements, abdominal imaging, and treatment of an identified Clostridium difficile infection. A random urine porphobilinogen was then obtained and found to be elevated. Fractionation of plasma and urine porphyrins was suggestive of AIP. Her symptoms improved with 3 days of intravenous (IV) hematin and IV dextrose. This is a unique case of a rare disease due to her clinical presentation with ileus, unremarkable past medical history, family history, and the prehospitalization and intrahospitalization factors that likely exacerbated the patient AIP.

Fine Needle Biopsies of Solid Pancreatic Lesions: Tissue Acquisition Technique and Needle Design Do Not Impact Specimen Adequacy.

BACKGROUND AND AIMS: Data on adequacy of EUS guided biopsies using different tissue acquisition techniques and fine needle aspiration needle designs have been inconclusive. Data on newer fine needle biopsy (FNB) needles are scarce. This study compared the performance of 3 acquisition techniques and 2 fine needle biopsy designs in solid pancreatic lesions. METHODS: Single-center, randomized, pilot clinical trial (Trial registration number NCT03264092). Patients undergoing EUS biopsy of pancreatic lesions were randomized to 1 of 3 acquisition techniques (dry suction, wet suction, slow pull) and 1 of 2 22G FNB needle designs. The primary outcome was specimen cellularity. Secondary outcomes included blood contamination and number of passes needed for diagnosis. RESULTS: A total of 52 (35.3%), 49 (33.3%) and 46 (31.3%) specimens were obtained with slow pull, dry suction and wet suction, respectively. A total of 56 (38%) and 91 (62%) specimens were obtained with each needle, respectively. No difference in cellularity scores was identified by technique (3.28 vs 3.55 vs 2.94; p = 0.081) or needle type (3.45 vs 3.15; p = 0.19). The same was true for blood contamination and diagnostic pass. A diagnosis was reached after 3 passes in 51 patients (93%). Histological diagnosis was possible in 45 specimens (82%). No severe adverse events occurred. CONCLUSIONS: Cellularity of pancreatic specimens obtained with FNB needles via EUS was not influenced by technique and needle design. Three passes were enough to obtain a histological diagnosis in most patients. Larger clinical trials are required to validate the results of this study.

Treatment of Primary Mediastinal B-Cell Lymphoma With R-CEOP (Rituximab, Cyclophosphamide, Etoposide, Vincristine, and Prednisone).

Primary mediastinal B-cell lymphoma (PMBCL) is a rare subtype of non-Hodgkin's lymphoma. It typically has an aggressive behavior with potential clinical emergencies including cardiac tamponade, thrombosis of major neck vessels, airway obstruction, and tumor lysis syndrome. In this case report, a 38-year-old Caucasian male presented with shortness of breath, a two-month history of 40-pound weight loss, and a left-sided chest wall mass. CT imaging showed a mediastinal mass, measuring 13 × 14.6 × 8.6 cm3, with invasion and partial occlusion of the brachiocephalic veins and upper superior vena cava causing superior vena cava syndrome, and encasement of multiple coronary artery segments. CT-guided biopsy showed high-grade B-cell lymphoma. Cytology biomarkers were positive for CD20, CD45, and PAX5. A trans-thoracic echocardiogram (TTE) was obtained prior to chemotherapy initiation to establish a baseline for cardiac function, which showed an ejection fraction (EF) of 45-50%, right ventricle volume overload and dilation, and pulmonary hypertension. R-CEOP (rituximab, cyclophosphamide, etoposide, vincristine, and prednisone) chemotherapy regimen was initiated and a follow-up echocardiogram after three cycles, revealed a significant improvement in EF; the patient subsequently received three additional cycles of R-EPOCH. Current regimens in the United States utilize dose-adjusted R-EPOCH and R-CHOP, but they must be used cautiously in patients with compromised cardiac function, due to the cardiotoxic side effects of the chemotherapy agent, doxorubicin. This case illustrates that anthracycline-free regimens should be considered in patients with reduced cardiac function, with this case showing the utilization of an anthracycline-free regimen (R-CEOP) for the first three cycles, followed by a transition to R-EPOCH.

From Chemical Burn to Below Knee Amputation: Amputation Secondary to Application of Over-the-Counter Topical Analgesic With Menthol and Methyl Salicylate.

Topical analgesics and topical rubefacients are widely used to control acute as well as chronic pain every day. Due to their availability without a prescription, consumers often overlook any potentially harmful effects and consider them largely benign. Here, we present a rare case of chemical burn triggered by a typical over-the-counter (OTC) analgesic containing menthol and methyl salicylate resulting in chemical burn, complicated by necrotizing infection treated by below the knee amputation.

Acute aleukemic mast cell leukemia: Report of a case and review of the literature.

Mast Cell Leukemia (MCL) is the rarest form of systemic mastocytosis, a rare group of neoplastic disease that result from clonal proliferation of mast cells and their accumulation in one or more organ systems. The diagnosis of MCL is made by meeting the World Health Organization (WHO) 2017 criteria. MCL is further subclassified as leukemic or aleukemic based on presence or absence of circulating mast cells in the peripheral blood and acute versus chronic based on presence or absence of findings indicative of impaired organ function due to mast cell infiltration. A 64-year-old Hispanic male presented with myalgia, diarrhea, urticarial rash, back pain, and fulminant disseminated intravascular coagulation. Bone marrow examination, supplemented by immunohistochemistry results, fulfilled the WHO criteria for the diagnosis of MCL. To the best of our knowledge, this is the first documented case of aleukemic acute MCL in a Hispanic patient.

IV Lipid Emulsion Infusion in the Treatment of Severe Diphenhydramine Overdose.

BACKGROUND Diphenhydramine is a commonly available over-the-counter antihistamine; however, there are few documented cases of treatment when ingested in toxic quantities, where it can cause a sodium channel blockade leading to wide-complex tachycardia, seizures, and death. Conventional treatment includes sodium bicarbonate infusion, but few cases have documented the addition of lipid emulsion therapy. CASE REPORT A 24-year-old African American female ingested 18 g (360 pills of 50 mg) over-the-counter diphenhydramine. She presented comatose, with hemodynamic instability and hypotension, intubated with pupil dilation to 6 to 7 mm, and initial electrocardiography findings showing a type 1 AV block with a QT/QTc of 360/402 ms which progressed into sinus tachycardia with widened QRS intervals of 134 ms and prolonged QT/QTc intervals of up to 638/759 ms. Treatment using sodium bicarbonate and magnesium was initiated; however, the intraventricular conduction delay persisted. Infusion of 20% intravenous lipid emulsion was administered; following this, the patient developed narrow complex QRS with sinus rhythm and shortened the QT/QTc interval to 448/516 ms. She recovered quickly and was transferred to inpatient psychiatric unit for further evaluation, and discharged 1 month later. CONCLUSIONS Lipid emulsion therapy has been utilized in treatment of various medication overdoses, but there are few documented cases in the treatment of diphenhydramine overdose. With the amount of diphenhydramine ingested by the patient in this case report, the use of combined conventional and lipid emulsion therapy was utilized in the stabilization and management of the patient, and should be considered in scenarios where conventional treatments have not improved the clinical outcome.

Acute on chronic anaemia with a haemoglobin of 18 g/L (1.8 g/dL) and haematocrit of 7.2.

Anaemia can present with symptoms of fatigue, shortness of breath, weakness, malaise, tachycardia and skin pallor. If left untreated, this can progress to life-threatening complications such as arrhythmias, cardiac hypertrophy and myocardial infarction. In this report, a 43-year-old woman, who was ambulatory with no exertional dysponea, presented with weakness, fatigue, bilateral lower extremity oedema and intermittent right sided chest pain for several months. This patient was subsequently found to have a haemoglobin of 18 g/L (1.8 g/dL) and haematocrit of 7.2%. She was admitted to the hospital and treated with seven units of blood. CT scan showed a 9.6 cm uterine fibroid in addition to a 5.9×5.4 cm mass near the right kidney, which was later diagnosed as metastatic carcinoid tumour. This case deserves attention due to the importance of looking for multiple causes of blood loss and the effects of low haemoglobin levels.

Facile generation of cell microarrays using vacuum degassing and coverslip sweeping.

A simple method to generate cell microarrays with high-percentage well occupancy and well-defined cell confinement is presented. This method uses a synergistic combination of vacuum degassing and coverslip sweeping. The vacuum degassing step dislodges air bubbles from the microwells, which in turn enables the cells to enter the microwells, while the physical sweeping step using a glass coverslip removes the excess cells outside the microwells. This low-cost preparation method provides a simple solution to generating cell microarrays that can be performed in basic research laboratories and point-of-care settings for routine cell-based screening assays.