Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 27876626.
- Also identified by DOI 10.1016/j.jaci.2016.10.022 and PMC identifier 5846100.
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Abstract
NLRC4-inflammasome hyperactivity causes infantile-onset Macrophage Activation Syndrome and enterocolitis with extraordinary serum IL-18 elevation (NLRC4-MAS). Herein, we report a critically ill infant with severe, refractory NLRC4-MAS who showed sustained response to treatment with experimental IL-18 inhibition.
Medical subject headings
- CARD Signaling Adaptor Proteins
- Calcium-Binding Proteins
- Enterocolitis
- Intercellular Signaling Peptides and Proteins
- Macrophage Activation Syndrome
- Mutation
- Paramyxoviridae
- Paramyxoviridae Infections