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


Picard C., von Bernuth H., Ghandil P., Chrabieh M., Levy O., Arkwright P. D. , ...Daha Fazla

MEDICINE, cilt.89, ss.403-425, 2010 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 89 Konu: 6
  • Basım Tarihi: 2010
  • Doi Numarası: 10.1097/md.0b013e3181fd8ec3
  • Dergi Adı: MEDICINE
  • Sayfa Sayıları: ss.403-425

Özet

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.