Clinical Features and Outcome of Patients With IRAK-4 and MyD88 Deficiency

Date
2010
Author
Marodi, Laszlo
Nonoyama, Shigeaki
Domachowske, Joseph
Issekutz, Andrew C.
Tang, Mimi
Smart, Joanne
Zitnik, Simona Eva
Hoarau, Cyrille
Kumararatne, Dinakantha S.
Thrasher, Adrian J.
Davies, E. Graham
Bethune, Claire
Sirvent, Nicolas
de Ricaud, Dominique
Vasconcelos, Julia
Rodrigo, Carlos
Almazan, Francisco
Mendez, Maria
Ignacio Arostegui, Juan
Alsina, Laia
Fortuny, Claudia
Reichenbach, Janine
Verbsky, James W.
Bossuyt, Xavier
Doffinger, Rainer
Abel, Laurent
Puel, Anne
Casanova, Jean-Laurent
Camcioglu, Yildiz
Guedes, Margarida
Picard, Capucine
von Bernuth, Horst
Vitor, Artur Bonito
Ghandil, Pegah
Chrabieh, Maya
Levy, Ofer
Arkwright, Peter D.
McDonald, Douglas
Geha, Raif S.
Takada, Hidetoshi
Krause, Jens C.
Creech, C. Buddy
Ku, Cheng-Lung
Ehl, Stephan
Al-Muhsen, Saleh
Al-Hajjar, Sami
Al-Ghonaium, Abdulaziz
Day-Good, Noorbibi K.
Holland, Steven M.
Gallin, John I.
Chapel, Helen
Speert, David P.
Rodriguez-Gallego, Carlos
Colino, Elena
Garty, Ben-Zion
Roifman, Chaim
Hara, Toshiro
Yoshikawa, Hideto
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Abstract
Autosomal recessive interleukin-1 receptor-associated kinase (IRAK)-4 and myeloid differentiation factor (MyD) 88 deficiencies impair Toll-like receptor (TLR)-and interleukin-1 receptor-mediated immunity. We documented the clinical features and outcome of 48 patients with IRAK-4 deficiency and 12 patients with MyD88 deficiency, from 37 kindreds in 15 countries.
URI
http://hdl.handle.net/20.500.12627/62507
https://doi.org/10.1097/md.0b013e3181fd8ec3
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