Journal
JOURNAL OF CLINICAL IMMUNOLOGY
Volume 41, Issue 7, Pages 1457-1462Publisher
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10875-021-01078-4
Keywords
Down syndrome; COVID-19; MIS-C; inborn errors of immunity
Categories
Funding
- National Institute of Allergy and Infectious Diseases [R01AI150300, R01AI150300-01S1]
- T32 training grant [T32HD075735]
Ask authors/readers for more resources
Research has found that children with Down syndrome may develop severe, atypical cases of multisystem inflammatory syndrome (MIS-C), characterized by specific immune features.
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 Fc gamma RI. 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available