ABSTRACT
Case: Wiskott-Aldrich Syndrome (WAS) is a rare X-linked inborn error of immunity caused by mutations in the WAS gene. It is classically characterized by immunodeficiency, eczema, and micro-thrombocytopenia. It has been known since the 1960s that patients with WAS have an increased risk of lymphoproliferative disease though the exact incidence remains unknown in the American population. Limited case reports have discussed EBV-related lymphoproliferative disease in patients with WAS. We present a case of a 9-year-old boy with known WAS complicated by eczematous rash, thrombocytopenia, recurrent ear infections, and monoclonal gammopathy who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. Family had been offered treatment with hematopoietic stem cell transplant but declined multiple times in the past. Earlier in the year, he presented with possible MIS-C with negative SARS-CoV-2 PCR. He presented to our hospital with mastoiditis and lymphadenopathy. Physical examination showed severe eczema on hands and tender right mastoid. Laboratory evaluation showed thrombocytopenia, elevated IgG of 6290, IgA of 744, IgE of 827, low IgM of 41, and 14% response to pneumococcal titers. He was empirically treated with intravenous antibiotics. ENT performed right postauricular incision and drainage and the culture grew Hemophilus influenza. Throughout his hospital stay, his submandibular lymphadenopathy became more prominent despite treatment. Core needle biopsy of right submandibular lymph node was suggestive of EBV-associated lymphoid hyperplasia. EBV PCR and antibodies were both positive. CT chest, abdomen, and pelvis revealed multifocal pulmonary lymphadenopathy and a diffuse, bilateral nodularity as well as retroperitoneal and mesenteric lymphadenopathy. He was given four doses of weekly Rituximab, which successfully decreased EBV viremia below linear detectability. Immunoglobulin replacement therapy (IgRT) was initiated. Bronchoalveolar lavage and lung biopsy were performed and are results are currently pending. Discussion(s): We present a case of a 9-year-old boy with known WAS awaiting transplant who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. While lymphoproliferative disease is a known complication of WAS, EBV-related lymphoproliferative disease in WAS patients has only been reported as case reports and remains a rare but known complication of patient with WAS.Copyright © 2023 Elsevier Inc.
ABSTRACT
A comparative assessment of dissolved oxygen (DO) and biochemical oxygen demand (BOD) of river Ganga during prelockdown and lockdown periods was made through analysis of data generated from real-time water quality motoring systems. The concentration data for DO and BOD are examined for (i) prelockdown period (March 15-21, 2020) and (ii) lockdown period (March 22-April 15, 2020). The analysis results show 3%-20% decrease in DO concentration. The slight decrease in DO observed at all locations during the first week after lockdown which may be due to the increased levels of suspended solids and turbidity in the river water because of heavy rains. DO during fourth week of lockdown has shown a decreased value as compared to the prelockdown period at most of the locations. However, in West Bengal the DO has increased in lockdown. BOD value ranged between 1.13 mg/L and 5.56 mg/L during lockdown period, more or less similar to prelockdown range of 1.37-5.58 mg/L. This chapter further discusses the cause of water quality changes during the period of lockdown as compare to prelockdown period. © 2021 Elsevier Inc.
ABSTRACT
Introduction: A single tertiary care center has offered oral immunotherapy (OIT) as a clinical service since 2018 using commercial products and subsequently has offered FDA-approved Peanut (Arachis- hypogaea) Allergen Powder-dnfp (PNAP) since July 2020. Shared decision making regarding OIT was based on previous IgE testing, history, and family preference. Methods: A retrospective chart review was performed in REDCap database. Results: A total of 37 patients initiated OIT (21 commercial products and 16 PNAP). The mean age was 9.25 for commercial product and 7.25 for PNAP with more males than females participating. Co-morbid conditions included allergic rhinitis, asthma, and eczema. Mean peanut IgE prior to starting was 49.75 and Ara h2 was 32.73. Prior reactions to peanut included skin, GI and anaphylaxis. Three children failed an oral challenge prior to starting OIT. There were 5 children that had never ingested peanuts but completed OIT. Minor intermittent side effects including stomach upset and itchy mouth were reported during escalation of both products. Dose adjustment was required for 1 PNAP patient for complaint of dysphagia and pain. There were 4 patients who dropped out of OIT: refusal to eat (1), unrelated GI disease (1) and the COVID-19 pandemic (2). All patients upon reaching maintenance are daily dosing with peanuts or chocolate-covered peanut candy. Lifestyle changes reported since reaching maintenance include eating in restaurants previously avoided and ingesting foods with labels stating: “may contain peanuts,” and “foods processed in a facility with peanuts.” Conclusions: Peanut OIT is safe and well tolerated in our patient population.
ABSTRACT
An echocardiographic investigation is one of the key modalities of diagnosis in cardiology. There has been a rising presence of cardiological comorbidities in patients positive for COVID-19. Hence, it is becoming extremely essential to look into the correct safety precautions, healthcare professionals must take while conducting an echo investigation. The decision matrix formulated for conducting an echocardiographic evaluation is based on presence or absence of cardiological comorbidity vis-à-vis positive, suspected or negative for COVID-19. The safety measures have been constructed keeping in mind the current safety precautions by WHO, CDC and MoHFW, India.