NYMC Faculty Publications
Atypical Inflammatory Syndrome Triggered by SARS-CoV-2 in Infants with Down Syndrome
Author Type(s)
Faculty
DOI
10.1007/s10875-021-01078-4
Journal Title
Journal of Clinical Immunology
First Page
1457
Last Page
1462
Document Type
Case Report
Publication Date
10-2021
Department
Pediatrics
Abstract
While adults with Down syndrome (DS) are at increased risk of severe COVID-19 pneumonia, little is known about COVID-19 in children with DS. In children without DS, SARS-CoV-2 can rarely cause severe COVID-19 pneumonia, or an even rarer and more typically pediatric condition, multisystem inflammatory syndrome in children (MIS-C). Although the underlying mechanisms are still unknown, MIS-C is thought to be primarily immune-mediated. Here, we describe an atypical, severe form of MIS-C in two infant girls with DS who were hospitalized for over 4 months. Immunological evaluation revealed pronounced neutrophilia, B cell depletion, increased circulating IL-6 and IL-8, and elevated markers of immune activation ICAM1 and FcɣRI. Importantly, uninfected children with DS presented with similar but less stark immune features at steady state, possibly explaining risk of further uncontrolled inflammation following SARS-CoV-2 infection. Overall, a severe, atypical form of MIS-C may occur in children with DS.
Recommended Citation
Malle, L., Bastard, P., Martin-Nalda, A., Carpenter, T., Bush, D., Patel, R., Colobran, R., Soler-Palacin, P., Casanova, J., Gans, M., Rivière, J. G., & Bogunovic, D. (2021). Atypical Inflammatory Syndrome Triggered by SARS-CoV-2 in Infants with Down Syndrome. Journal of Clinical Immunology, 41 (7), 1457-1462. https://doi.org/10.1007/s10875-021-01078-4