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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5846100 | PMC |
| http://dx.doi.org/10.1016/j.jaci.2016.10.022 | DOI Listing |
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