Article
[NLRC4 associated autoinflammatory diseases: A systematic review of the current literature].
La Revue de medecine interne - 1 Apr 2018
Rodrigues F, Hentgen V, Bachmeyer C, Kone-Paut I, Belot A, Grateau G, Sarrabay G, Georgin-Lavialle S
Abstract excerpt
The auto-inflammatory diseases linked to NLRC4 mutations are recently described entities. Transmission is autosomal dominant in 80 % of cases; cases of somatic mutation have already been reported. The disease may display two very different clinical phenotypes: the phenotype 1 (30 %), severe, is dominated by a multisystemic inflammation starting in the first year of life with symptoms of chronic inflammatory bowel...
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