Seven chronic granulomatous disease cases in a single-center experience and a review of the literature


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Kutlug S., Sensoy G., Birinci A., Saraymen B. , Köker M. Y. , Yildiran A.

ASIAN PACIFIC JOURNAL OF ALLERGY AND IMMUNOLOGY, cilt.36, ss.35-41, 2018 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 36 Konu: 1
  • Basım Tarihi: 2018
  • Doi Numarası: 10.12932/ap0859
  • Dergi Adı: ASIAN PACIFIC JOURNAL OF ALLERGY AND IMMUNOLOGY
  • Sayfa Sayıları: ss.35-41

Özet

Background: Chronic granuloniatous disease (CGD) is a rare primary immunodeficiency caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzyme system. this disease causes the disordered functioning of phagocytic cells. It is characterized by life-threatening and/or recurrent infections by bacteria and fungi. CGD has both an X-linked recessive (X-CGD) and autosomal recessive (AR-CGD) phenotypes. AR form have four subtypes including defects with one of these NADPH oxidase components (p22, p40, p47 and p67(Phox)).