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Xu, C. IRAK-4 Deficiency. Encyclopedia. Available online: https://encyclopedia.pub/entry/4243 (accessed on 21 September 2026).
Xu C. IRAK-4 Deficiency. Encyclopedia. Available at: https://encyclopedia.pub/entry/4243. Accessed September 21, 2026.
Xu, Camila. "IRAK-4 Deficiency" Encyclopedia, https://encyclopedia.pub/entry/4243 (accessed September 21, 2026).
Xu, C. (2020, December 23). IRAK-4 Deficiency. In Encyclopedia. https://encyclopedia.pub/entry/4243
Xu, Camila. "IRAK-4 Deficiency." Encyclopedia. Web. 23 December, 2020.
IRAK-4 Deficiency
Edit

IRAK-4 deficiency is an inherited disorder of the immune system (primary immunodeficiency). This immunodeficiency leads to recurrent infections by a subset of bacteria known as pyogenic bacteria but not by other infectious agents. (Infection with pyogenic bacteria causes the production of pus.)

genetic conditions

References

  1. Ku CL, von Bernuth H, Picard C, Zhang SY, Chang HH, Yang K, Chrabieh M,Issekutz AC, Cunningham CK, Gallin J, Holland SM, Roifman C, Ehl S, Smart J, TangM, Barrat FJ, Levy O, McDonald D, Day-Good NK, Miller R, Takada H, Hara T,Al-Hajjar S, Al-Ghonaium A, Speert D, Sanlaville D, Li X, Geissmann F, Vivier E, Maródi L, Garty BZ, Chapel H, Rodriguez-Gallego C, Bossuyt X, Abel L, Puel A,Casanova JL. Selective predisposition to bacterial infections in IRAK-4-deficientchildren: IRAK-4-dependent TLRs are otherwise redundant in protective immunity. JExp Med. 2007 Oct 1;204(10):2407-22.
  2. Maglione PJ, Simchoni N, Black S, Radigan L, Overbey JR, Bagiella E, BusselJB, Bossuyt X, Casanova JL, Meyts I, Cerutti A, Picard C, Cunningham-Rundles C.IRAK-4 and MyD88 deficiencies impair IgM responses against T-independentbacterial antigens. Blood. 2014 Dec 4;124(24):3561-71. doi:10.1182/blood-2014-07-587824.
  3. Medvedev AE, Lentschat A, Kuhns DB, Blanco JC, Salkowski C, Zhang S, Arditi M,Gallin JI, Vogel SN. Distinct mutations in IRAK-4 confer hyporesponsiveness tolipopolysaccharide and interleukin-1 in a patient with recurrent bacterialinfections. J Exp Med. 2003 Aug 18;198(4):521-31.
  4. Picard C, Casanova JL, Puel A. Infectious diseases in patients with IRAK-4,MyD88, NEMO, or IκBα deficiency. Clin Microbiol Rev. 2011 Jul;24(3):490-7. doi:10.1128/CMR.00001-11. Review.
  5. Picard C, Puel A, Bonnet M, Ku CL, Bustamante J, Yang K, Soudais C, Dupuis S, Feinberg J, Fieschi C, Elbim C, Hitchcock R, Lammas D, Davies G, Al-Ghonaium A,Al-Rayes H, Al-Jumaah S, Al-Hajjar S, Al-Mohsen IZ, Frayha HH, Rucker R, Hawn TR,Aderem A, Tufenkeji H, Haraguchi S, Day NK, Good RA, Gougerot-Pocidalo MA,Ozinsky A, Casanova JL. Pyogenic bacterial infections in humans with IRAK-4deficiency. Science. 2003 Mar 28;299(5615):2076-9.
  6. Picard C, von Bernuth H, Ghandil P, Chrabieh M, Levy O, Arkwright PD, McDonaldD, Geha RS, Takada H, Krause JC, Creech CB, Ku CL, Ehl S, Maródi L, Al-Muhsen S, Al-Hajjar S, Al-Ghonaium A, Day-Good NK, Holland SM, Gallin JI, Chapel H, Speert DP, Rodriguez-Gallego C, Colino E, Garty BZ, Roifman C, Hara T, Yoshikawa H,Nonoyama S, Domachowske J, Issekutz AC, Tang M, Smart J, Zitnik SE, Hoarau C,Kumararatne DS, Thrasher AJ, Davies EG, Bethune C, Sirvent N, de Ricaud D,Camcioglu Y, Vasconcelos J, Guedes M, Vitor AB, Rodrigo C, Almazán F, Méndez M,Aróstegui JI, Alsina L, Fortuny C, Reichenbach J, Verbsky JW, Bossuyt X,Doffinger R, Abel L, Puel A, Casanova JL. Clinical features and outcome ofpatients with IRAK-4 and MyD88 deficiency. Medicine (Baltimore). 2010Nov;89(6):403-25. doi: 10.1097/MD.0b013e3181fd8ec3.
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