Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
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BACKGROUNDPediatric SARS-CoV-2 infection can be complicated by a dangerous hyperinflammatory condition termed multisystem inflammatory syndrome in children (MIS-C). The clinical and immunologic spectrum of MIS-C and its relationship to other inflammatory conditions of childhood have not been studied in detail.METHODSWe retrospectively studied confirmed cases of MIS-C at our institution from March to June 2020. The clinical characteristics, laboratory studies, and treatment response were collected. Data were compared with historic cohorts of Kawasaki disease (KD) and macrophage activation syndrome (MAS).RESULTSTwenty-eight patients fulfilled the case definition of MIS-C. Median age at presentation was 9 years (range: 1 month to 17 years); 50% of patients had preexisting conditions. All patients had laboratory confirmation of SARS-CoV-2 infection. Seventeen patients (61%) required intensive care, including 7 patients (25%) who required inotrope support. Seven patients (25%) met criteria for complete or incomplete KD, and coronary abnormalities were found in 6 cases. Lymphopenia, thrombocytopenia, and elevation in inflammatory markers, D-dimer, B-type natriuretic peptide, IL-6, and IL-10 levels were common but not ubiquitous. Cytopenias distinguished MIS-C from KD and the degree of hyperferritinemia and pattern of cytokine production differed between MIS-C and MAS. Immunomodulatory therapy given to patients with MIS-C included intravenous immune globulin (IVIG) (71%), corticosteroids (61%), and anakinra (18%). Clinical and laboratory improvement were observed in all cases, including 6 cases that did not require immunomodulatory therapy. No mortality was recorded in this cohort.CONCLUSIONMIS-C encompasses a broad phenotypic spectrum with clinical and laboratory features distinct from KD and MAS.FUNDINGThis work was supported by the National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases; the National Institute of Allergy and Infectious Diseases; Rheumatology Research Foundation Investigator Awards and Medical Education Award; Boston Children's Hospital Faculty Career Development Awards; the McCance Family Foundation; and the Samara Jan Turkel Center.
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PMID:Distinct clinical and immunological features of SARS-CoV-2-induced multisystem inflammatory syndrome in children. 3273 Feb 26

Several cases of a severe multi-system inflammatory syndrome have been reported in children during the current COVID-19 pandemic. Clinical manifestations may resemble Kawasaki disease (KD) which is the most common childhood vasculitis. A 7-year-old-girl presented with fever, rash and abdominal pain. Examination showed maculopapular rash over lower limbs, back, right ear, trunk and abdomen; erythema and swelling over bilateral upper eyelids; conjunctival injection; reddened lips and erythema over palms and soles. She had lymphopenia, thrombocytopenia, with elevated erythrocyte sedimentation rate, C-reactive protein (CRP), pro-brain natriuretic peptide and interleukin-6 (IL-6). Real-time polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was negative. Serology, however, could not be performed due to unavailability. Macrophage activation syndrome was ruled out with normal ferritin and triglyceride with raised fibrinogen level. Echocardiography showed normal coronary diameters. Child received meropenem, intravenous immunoglobulin, aspirin and methylprednisolone. She improved with resolution of fever, decrease in CRP, increase in platelet and lymphocyte count. Index child had features similar to those reported from UK and Italy: features of KD, abdominal pain, lymphopenia, thrombocytopenia, elevated IL-6 and myocardial dysfunction with no significant respiratory involvement. Pediatricians should be aware of such uncommon presentation in children to initiate early treatment with immunomodulatory therapy.
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PMID:Multi-System Inflammatory Syndrome in a Child Mimicking Kawasaki Disease. 3277 86