Amik Sodhi

Therapeutic plasmapheresis for hypertriglyceridemia-associated acute pancreatitis: case series and review of the literature

Background: Severe hypertriglyceridemia (HTG) is the third leading cause of acute pancreatitis (AP) in the United States. The current standard of care includes management of HTG using pharmacological therapy. More recently, plasmapheresis has been proposed as a therapeutic tool for decreasing triglyceride (TG) levels, especially in critically ill patients. Few studies are available to ascertain overall benefits of plasmapheresis over traditional management. Objective: To analyze the outcomes of patients treated with plasmapheresis for severe HTG-associated pancreatitis. Methods: We conducted a retrospective chart review of three patients with severe HTG- associated (TGs greater than 1000 mg/dl; 11.29 mmol/l) AP at the Methodist University Hospital. All the patients underwent plasmapheresis as part of their treatment. Results: The average TG level before plasmapheresis was 3532 mg/dl (range: 2524–4562 mg/dl; 39.9 mmol/l; range: 28.5–51.6 mmol/l). All patients made a full recovery, with a significant improvement in TG levels after plasmapheresis. The mean number of sessions was 1.3 (range 1–2), and mean TG level after plasmapheresis was 1051 mg/dl (range: 509–1771 mg/dl; 11.9 mmol/l; range: 5.8–20 mmol/l). After the first session, the average reduction of TG level was 2481 mg/dl (range 753–3750 mg/dl; 28 mmol/l; range: 8.5–42.4 mmol/l) or approximately 70%. None of the patients developed complications related to plasmapheresis. Conclusions: Plasmapheresis can be an effective and rapid treatment option in patients with severe HTG and complications. However, further research, including randomized controlled studies, is necessary.

Vitamin D Supplementation: Not So Simple in Sarcoidosis

Introduction Americans are increasingly receiving vitamin D supplementation, often based on low‐measured 25‐hydroxy‐vitamin D (25‐OH‐vit D). In sarcoidosis, there is often increased metabolism of 25‐OH‐vit D to 1,25‐dihydroxy‐vitamin D (1,25‐OH‐vit D), so 25‐OH‐vit D may remain low, despite high levels of 1,25‐OH‐vit D. In such cases, vitamin D supplementation may lead to hypercalcemia. Methods We randomly selected 196 patients with sarcoidosis who received at least 1 prescription of vitamin D between 2005 and 2011 and 196 control patients. Primary outcome was the incidence of hypercalcemia during the 2 years following the vitamin D prescription. A secondary outcome was the proportion of patients who had received vitamin D prescriptions and who had adequate blood work performed before the prescription. Results The 25‐OH‐vit D and 1,25‐OH‐vit D levels were measured in only 70% and 23%, respectively, of those receiving supplementation. Hypercalcemia was noted more frequently in the group that received vitamin D (42.3%) as compared with the nonsupplemented group (18.3%), P < 0.0001. Patients who received a vitamin D prescription developed moderate and severe hypercalcemia more frequently (12.8%) as compared to the group that did not receive vitamin D (3.6%), P = 0.001. In multivariate analysis, having a prescription for vitamin D increased the risk of developing hypercalcemia to approximately 2‐fold. The risk of developing hypercalcemia (odds ratio = 4.1) was increased with renal failure. Conclusions Our study demonstrates that a substantial proportion of patients with sarcoidosis who receive vitamin D are not getting appropriate pretesting. This increases their risk for developing hypercalcemia.

Concurrent Myelomatous Pleural Effusion and Extramedullary Mediastinal Involvement as an Initial Manifestation of Multiple Myeloma

Patient: Female, 72 Final Diagnosis: Myelomatous pleural effusion Symptoms: Dyspnea Medication: — Clinical Procedure: Thoracentesis Specialty: Pulmonology Objective: Rare disease Background: Myelomatous pleural effusion (MPE) is a rare occurrence in patients with multiple myeloma (MM). Fewer than 20 cases of MPE have been reported as an initial manifestation of MM. Extramedullary plasmacytoma (EMP) occurs in fewer than 5% patients with MM, and mediastinal EMP is even rarer, with only about 80 cases reported in the literature. We present a case study involving a patient with concurrent MPE and mediastinal EMP as an initial manifestation of MM. Case Report: The patient was a 74-year-old nonsmoking female with a 3-month history of exertional dyspnea and back pain. On exam, the patient was afebrile (temperature 37.2°C), blood pressure was 160/74 mm Hg, heart rate was 92 bpm, respiratory rate was 22/min, and oxygen saturation was 87% on room air. Patient was in mild distress and had decreased breath sounds over right lung fields about halfway up with dullness to percussion. Computed tomography of the chest showed a moderate-sized right pleural effusion and an anterior mediastinal mass. Thoracentesis showed a lymphocyte-predominant exudate. Cytology showed numerous plasma cells including immature forms. Stains for CD138 were positive, confirming plasma cell origin of cells. The anterior mediastinal mass was also biopsied and showed diffuse infiltrate of lymphocytes with plasma cell features that were also positive for CD138. Systemic protein electrophoresis showed a monoclonal immunoglobulin G kappa spike, and bone marrow biopsy was consistent with MM. Conclusions: MPE and EMP are extremely rare manifestations in MM. In addition, it is extremely rare for these to be the presenting features of MM. We report concurrently occurring MPE and EMP in a patient as her initial manifestation of MM.

Atrio-esophageal fistula: A case series and literature review

Case series Patient: Male, 72 • Male, 29 • Male, 75 Final Diagnosis: Atrio-esophageal fistula Symptoms: Altered mental state • chest pain • fever • melena Medication: — Clinical Procedure: — Specialty: Critical Care Medicine Objective: Rare disease Background: Percutaneous catheter radiofrequency ablation (RFA) and cryoablation of the left atrium and pulmonary vein ostia have become successful therapeutic modalities in the management of atrial fibrillation. Atrio-esophageal fistula is a rare complication. Awareness of complication risk is imperative because without prompt diagnosis and urgent surgical intervention, the outcome is often fatal. We present 3 cases of atrio-esophageal fistula following percutaneous catheter radiofrequency ablation (RFA). Case Reports: Case 1: A 72-year old white male presented 27 days after percutaneous RFA for atrial fibrillation with fever, altered mental status, and melena. Esophagogastroduodenoscopy (EGD) revealed a 1-cm defect in the mid-esophagus. Upon thoracotomy, severe hemorrhage ensued from a concomitant injury to the left atrium. Multiple attempts to repair the left atrial perforation were unsuccessful and the patient died. Case 2: A 71-year old white male presented 29 days after percutaneous RFA for atrial fibrillation with fever and tonic-clonic seizure. Recognition of possible atrio-esophageal fistula was considered and confirmed on thoracotomy. Surgical fixation of the left atria and esophagus were performed. The patient survived and was discharged to a skilled care facility. Case 3: A 75-year old white male presented 24 days after percutaneous RFA for atrial fibrillation with chest pain. An echocardiogram revealed a large pericardial effusion and pericardiocentesis was performed. Despite aggressive measures, the patient died. The autopsy demonstrated a communicating esophageal fistula with the right pulmonary vein. Conclusions: Clinicians tending to patients who have recently undergone atrial ablation need to be aware of atrio-esophageal fistula as a rare but highly fatal complication.

Role of Transbronchial Needle Aspiration (Conventional and EBUS Guided) in the Diagnosis of Histoplasmosis in Patients Presenting with Mediastinal Lymphadenopathy.

Objectives The superior performance of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in the diagnosis and staging of malignancy has been demonstrated, with some investigators suggesting the same for sarcoidosis. The role of EBUS-TBNA in the diagnosis of histoplasmosis is not clear, however. In this study we estimate the diagnostic yield of conventional TBNA (cTBNA) and EBUS-TBNA for the diagnosis of histoplasmosis in patients with mediastinal lymphadenopathy. Methods Retrospective chart review was conducted on 452 consecutive patients who underwent cTBNA or EBUS-TBNA for mediastinal lymphadenopathy from January 1, 2005 to December 31, 2014 at Methodist Le Bonheur Healthcare–affiliated hospitals in Memphis, Tennessee. Data collection included demographic information, reason for the procedure, size of the lymph nodes, procedures performed, and the final diagnosis. Results Among 452 cases reviewed, 146 underwent cTBNA and 306 underwent EBUS-TBNA. Final diagnoses include malignancy (41.5%), sarcoidosis (11.2%), and histoplasmosis (8.1%). Among 146 patients who underwent cTBNA, a final diagnosis was obtained by this modality in 58 patients (39.7%). The diagnostic rate for cTBNA for malignancy was 68% (40/59), 30% (4/13) for sarcoidosis, and 43% (6/14) for histoplasmosis. In 306 patients who underwent EBUS-TBNA, 188 had a final diagnosis (61.4%) obtained by this modality. For EBUS-TBNA, the diagnostic rates were 79.5% (101/127) for malignancy, 74% (28/38) for sarcoidosis, and 78% (18/23) for histoplasmosis. Conclusions EBUS-TBNA had a higher yield than cTBNA for the diagnosis of histoplasmosis. Clinicians practicing in areas with a high prevalence of histoplasmosis and sarcoidosis should use EBUS-TBNA, whenever available, for this reason.

Chronic Granulomatous Disease Presenting as Aspergillus Fumigatus Pneumonia in a Previously Healthy Young Woman.

Patient: Female, 23 Final Diagnosis: Chronic granulomatous disease Symptoms: Fever • shortness of breath Medication: — Clinical Procedure: Bronchoscopy Specialty: Pulmonology Objective: Unusual clinical course Background: Chronic Granulomatous Disease (CGD) is a rare immunodeficiency disease caused by a genetic defect in the NADPH (nicotinamide adenine dinucleotide phosphate) oxidase enzyme, resulting in increased susceptibility to bacterial and fungal infections. The inheritance can be X-linked or autosomal recessive. Patients usually present with repeated infections early in life. We present an unusual case of a 23-year-old patient diagnosed with CGD. Case Report: A 23-year-old white woman with no previous history of recurrent infections presented with complaints of fever, shortness of breath, and diffuse myalgia. She had been treated twice for similar complaints recently, but without resolution. She was febrile, tachypneic, tachycardic, and hypoxic at presentation. Physical examination revealed diffuse inspiratory rales. Laboratory results showed leukocytosis. Her initial chest X-ray and CT chest showed reticular nodular interstitial lung disease pattern. Despite being on broad-spectrum antibiotics for 5 days, she continued to require supplemental oxygen and continued to be tachypneic, with minimal activity. Initial diagnostic tests, including bronchoscopy with biopsy and lavage, did not reveal a diagnosis. She then underwent a video-assisted thoracoscopic surgery (VATS) lung biopsy. The biopsy slides showed suppurative granulomatous inflammation affecting greater than 50% of the parenchymal lung surface. Fungal hyphae consistent with Aspergillus were present in those granulomas. A diagnosis of CGD was made and she was started on Voriconazole. She improved with treatment. Her neutrophil burst test showed negative burst on stimulation, indicating phagocytic dysfunction consistent with CGD. Autosomal recessive CGD was confirmed by genetic testing. Conclusions: CGD can present in adulthood without any previous symptoms and signs. Clinicians should consider this disease in patients presenting with recurrent or non-resolving infections. Timely treatment and prophylaxis has been shown to reduce serious infections as well as mortality in these patients.

Pleural Small Cell Lung Carcinoma: An Unusual Culprit in Pleural Effusion

Patient: Male, 77 Final Diagnosis: Pleural small cell carcinoma Symptoms: Chest pain • shortness of breath Medication: — Clinical Procedure: Thoracocentesis Specialty: Pulmonology Objective: Rare disease Background: Small cell lung carcinoma (SCLC) usually presents as lung or mediastinal lesions. It is very rare for SCLC to present primarily as an isolated pleural effusion with no lung or mediastinal lesions. Case Report: We report the case of a 77-year-old white male with a 60-pack year history of smoking, chronic obstructive pulmonary disease (stage IV), and asbestos exposure who presented with shortness of breath and left lateral chest pain for 7 days. On physical examination, he was very short of breath, with a prolonged expiratory phase on chest auscultation. Laboratory results were normal except for leukocytosis and chest radiograph revealing left-sided pleural effusion. Computerized tomography (CT) scanning of the chest with IV contrast showed left-sided pleural effusion without any lung or mediastinal lesions. Thoracentesis was performed and fluid was sent for analysis. Repeat CT chest/abdomen/pelvis, done immediately following thoracocentesis, did not show any masses or lymphadenopathy. Fluid analysis, including cytology and immunostain pattern, was consistent with small cell carcinoma. Conclusions: Small cell lung cancer presenting as an isolated pleural effusion is extremely rare. It requires close attention to cytology and immunohistochemistry of pleural fluid samples. It also has implications for management and should be managed as limited-stage SCLC.

Wait!!! No Steroids for this Asthma…

Patient: Female, 31 Final Diagnosis: Hyperinfection syndrome due to Strongyloides stercoralis Symptoms: Abdominal pain • shortness of breath Medication: Prednisone Clinical Procedure: Bronchoscopy with BAL Specialty: Pulmonology Objective: Unusual clinical course Background: Strongyloides stercoralis (SS) is a parasite seen in certain parts of the USA and in people from other endemic areas. In these patients steroids might precipitate or exacerbate asthma. Apart from worsening of asthma, serious complications like hyperinfection syndrome and even death can occur in these patients if treated with steroids. Treatment is either ivermectin or albendazole based on severity of the disease. Clinicians have to be very careful when prescribing steroids in patients presenting with an exacerbation of asthma from areas endemic for Strongyloides stercoralis. Case Report: A young woman with history of asthma presented with complaints of nausea, vomiting, abdominal pain, wheezing, and dry cough. Physical examination revealed diffuse expiratory wheezing and mild diffuse abdominal pain without rebound or guarding. Laboratory results showed leukocytosis with eosinophilia. Stool studies showed Strongyloides stercoralis. Imaging revealed ground-glass opacities in the right upper and lower lobe along with an infiltrate in the lingular lobe on the left side. Bronchoscopy showed Strongyloides stercoralis. The patient was diagnosed with hyperinfection syndrome due to Strongyloides stercoralis most probably exacerbated by prednisone given for her asthma. Steroids were then discontinued and the patient was started on ivermectin. The patient improved with treatment. Repeat stool examination was negative for Strongyloides stercoralis. Conclusions: Clinicians have to be very careful when prescribing steroids in patients presenting with an exacerbation of asthma who are from areas endemic for Strongyloides stercoralis and should test for it (preferably with serology test) before starting treatment.

Solitary Fibrous Tumor of the Pleura: A Rare Cause of Pleural Mass

Patient: Male, 63 Final Diagnosis: Solitary fibrous tumor of the pleura Symptoms: Chronic cough Medication: — Clinical Procedure: CT guided biopsy • surgical resection Specialty: Pulmonology Objective: Rare disease Background: A solitary fibrous tumor of the pleura is a rare but usually benign mesenchymal tumor arising from the pleura. Patients are often asymptomatic, resulting in the majority of tumors being detected incidentally on chest imaging. We present a case of a large solitary pleural tumor and review the typical radiographic and pathologic findings associated with this finding. Case Report: A 63-year-old white man with chronic obstructive pulmonary disease (COPD) was found to have a large pleural mass on chest radiography during a pre-operative assessment. The tumor was biopsied and findings were consistent with solitary fibrous tumor of the pleura. Conclusions: SFTPs are generally considered benign tumors although there is a risk of malignant transformation and recurrence. Imaging studies play an important role in identifying the tumor and planes of resection, and histologic diagnosis is critical in differentiating SFTP from other type of pleural masses. Surgical resection is main therapy of choice.