Epstein-Barr virus associated smooth muscle tumors in post transplant pediatric patients two cases of rare locations, and review of the literature.

Epstein-Barr virus (EBV) may present few or no symptoms in immunocompetent individuals; however, in immunocompromised patients as in the case of AIDS and post-transplant patients, the virus occasionally stimulates neoplastic transformations. Epstein-Barr virus may play a role in the development of smooth muscle tumors (SMT). In the case of Epstein-Barr associated smooth muscle tumors (EBV+SMT), the virus is thought to be the leading factor to the tumorigenic pathway. We report two pediatric patients (6 and 13 years old) who underwent liver transplantation and developed EBV+SMT in the colon and orbit. These two cases represent rare locations for this kind of lesion.

Cardiovascular Disease Risk in Calcium Pyrophosphate Deposition Disease: A Nationwide Study of Veterans.

OBJECTIVE: Calcium pyrophosphate deposition (CPPD) disease represents a common crystalline arthritis with a range of manifestations. Our goal was to investigate risks for cardiovascular events in patients with CPPD. METHODS: We performed a retrospective matched cohort analysis in the Veterans Health Administration Corporate Data Warehouse, 2010-2014. CPPD was defined by ≥1 International Classification of Diseases, Ninth Revision codes for chondrocalcinosis or calcium metabolism disorder. CPPD patients were age- and sex-matched to approximately 4 patients without codes for CPPD; we excluded patients with a cardiovascular event during the 365 days prior to the index date. Demographic information, traditional cardiovascular risk factors, medications, and health care utilization were assessed at baseline. The primary outcome was a major adverse cardiovascular event (MACE: myocardial infarction, acute coronary syndrome, coronary revascularization, stroke, or death). Secondary outcomes included individual components of MACE. Cox proportional hazards models estimated fully adjusted hazard ratios (HRs) and 95% confidence intervals (95% CIs). RESULTS: We identified 23,124 CPPD patients matched to 86,629 non-CPPD patients with >250,000 person-years of follow-up. The study population was 96% male, mean age was 78 years, and 75% were White. The frequency of traditional cardiovascular risk factors was similar between the 2 cohorts. CPPD was not significantly associated with risk for MACE (HR 0.98 [95% CI 0.94-1.02]) in fully adjusted models, though risks of myocardial infarction, acute coronary syndrome, and stroke were significantly higher in the CPPD cohort compared to the non-CPPD cohort. CONCLUSION: CPPD did not confer an increased risk for MACE, a composite end point including all-cause mortality. Our results propose CPPD as a novel risk factor for MACE components, including myocardial infarction, acute coronary syndrome, and stroke.

SRP-positive necrotising myopathy: takes more than just the muscles.

Necrotising myopathy is an autoimmune disease that commonly affects muscles. Here we examine a case of a middle-aged women presenting with a chief report of shortness of breath, who subsequently developed muscle weakness. Her clinical course was complicated by respiratory failure and pulmonary hypertension likely due to the underlying pathology of signal recognition particle-positive necrotising myopathy. After further evaluation, her shortness of breath was thought to be secondary to muscle pathology rather than cardiopulmonary pathology. She was transferred to our institution for workup by rheumatology. At the time of admission, 6 months after initial presentation, her weakness progressed, so that she was unable to lift her arms and legs against gravity. Furthermore, neurological examination revealed mild facial and nuchal weakness, severe proximal weakness, more moderate distal weakness and global areflexia.

A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome.

BACKGROUND: Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. OBJECTIVES: To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. CASES: We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria. Both were treated with anakinra with remarkable response to therapy. CONCLUSION: Schnitzler syndrome is a rare and underdiagnosed disorder. High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias. A serum protein electrophoresis and immunofixation should be performed in these patients. The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy. A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms. Thus, patients warrant long-term follow-up. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.

Role of human metapneumovirus, influenza A virus and respiratory syncytial virus in causing WHO-defined severe pneumonia in children in a developing country.

OBJECTIVE: The role of respiratory viruses in causing severe, life threatening pneumonia in children in developing countries is not well established. Our study aims to determine the role of human metapneumovirus (HMPV), influenza A virus and respiratory syncytial virus (RSV) in children, aged 6 weeks to 2 years, hospitalized with WHO defined severe pneumonia (tachypnea plus any general danger sign or chest in-drawing) at a public sector hospital in Karachi, Pakistan. METHODS: This study was conducted from November 2010 to September 2011 at Abbassi Shaheed Hospital, a large public tertiary care hospital in Karachi, Pakistan. Children admitted with WHO-defined severe pneumonia were enrolled and throat swabs were obtained to detect respiratory viruses using real time RT-PCR. Chest x-rays of all subjects were obtained and independently interpreted by two radiologists to diagnose radiologic pneumonia. RESULTS: 169 children were enrolled. HMPV was detected in 24 (14.2%), influenza A virus in 9 (5.3%) and RSV in 30 (17.8%) children admitted with severe pneumonia. Of 9 patients with influenza A, 8 tested positive for H1N1. Viral etiology was found in 18% of radiologically confirmed pneumonia. HMPV infections peaked in February and April, influenza A was prevalent in January, June and November and RSV infections were most prevalent from June to September. CONCLUSION: HMPV, influenza A and RSV are common causes of WHO-defined severe pneumonia in hospitalized children in Karachi. Knowledge regarding the viral etiology of pediatric pneumonia and individual viral seasonality can help in the recommendation and implementation of appropriate management strategies.