Autosomal recessive interleukin-1 receptor-associated kinase 4 deficiency in fourth-degree relatives.

Cardenes, María; von Bernuth, Horst; García-Saavedra, Ayoze; Santiago, Esther; Puel, Anne; Ku, Cheng-Lung; Emile, Jean-François; Picard, Capucine et al. · J Pediatr · 2006

case_report · Level V

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Abstract

We report a kindred with autosomal recessive interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency in 3 fourth-degree relatives. A diagnosis of IRAK-4 deficiency should be considered in families with invasive bacterial disease, even if the individuals affected are only distantly related, which falsely suggests multigenic or dominant inheritance with low penetrance.

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