Background The clinical manifestations and natural history of severe acute respiratory distress syndrome coronavirus 2 (SARS-Cov-2) related Multisystem Inflammatory Syndrome in Children (MIS-C) are poorly defined. Using a systematic review of individual cases and case series and collating elements of the clinical course, the objective of this study was to provide a detailed clinical description and natural history of MIS-C. Methods Case reports and series of MIS-C were recovered from repeated MEDLINE searches, a single EMBASE search and table of contents reviews of major general medicine and pediatric journals performed between June 3 and July 23, 2020. Fever, evidence of inflammation, and evidence of organ dysfunction were required for inclusion. Results MEDLINE and EMBASE searches produced 129 articles and 10 articles were identified from journal contents or article bibliographies; 16 reports describing 505 children with MIS-C comprise this review. Thirty- two children (14.7%) had negative results for SARS-Cov-2 by nucleic acid and/or antibody testing. The weighted median age was 9 years (6 months to 20 years). Clinical findings included fever (100%), gastrointestinal symptoms (88.0%), rash (59.2%), conjunctivitis (50.0%), chelitis/ “strawberry tongue” (55.7%) or extremity edema/erythema (47.5%). Median serum CRP, ferritin, fibrinogen and D dimer concentrations were above the normal range. Intravenous gammaglobulin (78.1%) and methylprednisolone/prednisone(57.6%) were the most common therapeutic interventions ; immunomodulation was used in 24.3% of cases. Myocardial dysfunction requiring ionotropic support (57.4%) plus extracorporeal membrane oxygenation (5.3%), respiratory distress requiring mechanical ventilation (26.1%), and acute kidney injury (11.9%) were the major complications; anticoagulation was used commonly (54.4%) but thrombotic events occurred rarely (3.5%). Seven (1.4%) children died. Conclusions MIS-C following SARS-Cov-2 infection frequently presents with gastrointestinal complaints and/or rash; conjunctivitis, chelitis and/or extremity changes also occur frequently. Serious complications occur frequently and respond to aggressive supportive therapy.
In children with pneumonia, serum CRP concentrations exceeding 40-60 mg/L weakly predict a bacterial etiology.
BackgroundWhile cystic fibrosis (CF) is the most common cause of bronchiectasis in childhood, non-CF bronchiectasis is associated with a wide variety of disorders. The objective of this study was to determine the relative prevalence and specific etiologies on non-CF bronchiectasis in childhood.MethodsEMBASE, Medline, OVID Cochrane Reviews, Directory of Open Access Journals, Open Science Directory, EPSCO information services, and OAlster were searched electronically and the bibliographies of selected studies were searched manually. The search was conducted independently by 2 authors. Study Selection: (1) any clinical trial, observational study or cross-sectional case series of 10 or more patients with a description of the conditions associated with bronchiectasis; (2) subjects aged 21 years or younger; (3) cystic fibrosis was excluded and; (4) the diagnosis was confirmed by computed tomography of the chest. Data Extraction: Patient number, age range, inclusion criteria, diagnostic criteria, patient source, and categorical and specific etiology.ResultsFrom 491 studies identified, 12 studies encompassing 989 children with non-CF bronchiectasis were selected. Sixty-three percent of the subjects had an underlying disorder. Infectious (17%), primary immunodeficiency (16%), aspiration (10%), ciliary dyskinesia (9%), congenital malformation (3%), and secondary immunodeficiency (3%) were the most common disease categories; 999 etiologies were identified. Severe pneumonia of bacterial or viral etiology and B cell defects were the most common disorders identified.ConclusionsThe majority of children with non-CF bronchiectasis have an underlying disorder. A focused history and laboratory investigated is recommended.Electronic supplementary materialThe online version of this article (doi:10.1186/s12887-014-0299-y) contains supplementary material, which is available to authorized users.
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