Life-threatening NLRC4-associated hyperinflammation successfully treated with Interleukin-18 inhibition

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-1...

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Bibliografiske detaljer
Udgivet i:J Allergy Clin Immunol
Main Authors: Canna, Scott W., Girard, Charlotte, Malle, Louise, de Jesus, Adriana, Romberg, Neil, Kelsen, Judith, Surrey, Lea F., Russo, Pierre, Sleight, Andrew, Schiffrin, Eduardo, Gabay, Cem, Goldbach-Mansky, Raphaela, Behrens, Edward M.
Format: Artigo
Sprog:Inglês
Udgivet: 2016
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5846100/
https://ncbi.nlm.nih.gov/pubmed/27876626
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jaci.2016.10.022
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