Chronic granulomatous disease (CGD), one of the phagocytic cell defects, is the primary immunodeficiency caused by dysfunction of the NADPH oxidase complex in neutrophils. The clinical, demographic and laborator
CHRONIC GRANULOMATOUS DISEASE (CGD): ISOLATED DEFECT OF THE NADPH-OXIDASE ACTIVITY IN NEUTROPHILSPediatric Research publishes original papers, invited reviews, and commentaries on the etiologies of diseases of children and disorders of development, extending from molecular biology to epidemiology. Use of ...
Chronic granulomatous disease(CGD) is a rare inheritedphagocytic disordercaused by defective oxidative burst inneutrophilsandmonocytes. This disease is caused by mutations in any of the four genes that comprise theNADPH oxidasecomplex inphagocytic cells. Thecatalytic subunitof the NADPH oxidase ...
The presence of functional, donor-derived neutrophils was assessed by flow cytometry using two different fluorescent dyes that measure reactive oxygen species generated by the phagocyte NADPH oxidase. No evidence of paternal-derived functional neutrophils above a level of 0.15% was observed. Peripheral ...
Female carriers have manifested some symptoms, such as dermatitis, stomatitis, and discoid lupus-like disease [161–165]. CGD-like infections can sometimes present in female carriers, and these patients demonstrate both normal and CGD-neutrophils with functional mosaicism[166]. In one report of 15...
Sabbione F, Gabelloni ML, Ernst G, Gori MS, Salamone G, Oleastro M, Trevani A et al (2014) Neutrophils suppress γδ T-cell function. Eur J Immunol 44:819–830 Segal AW (1987) Absence of both cytochrome b-245 subunits from neutrophils in X-linked chronic granulomatous disease. Natur...
Chronic granulomatous disease DHR: Dihydrorhodamine-1,2,3 GAM: Galactomannan NAPDH: Nicotinamide adenine dinucleotide phosphate NBT: Nitroblue tetrazolium NCF1: Neutrophil cytosolic factor 1 PMA: Phorbol 12-myristate 13-acetate PMN: Polymorphonuclear neutrophils ROS: Reactive oxygen species Re...
In Vivo Interferon-γ Therapy Augments the In Vitro Ability of Chronic Granulomatous Disease Neutrophils to Damage Aspergillus Hyphae During the recently completed double-blind, placebo-controlled, randomized trial of recombinant interferon-γ (rIFN-γ)therapy in chronic granulomatous dis... JH Rex,JE...
Neutrophils of twins and brother killed only 23%, 32% and 16% Staph. aureus after 2 hours (control 83%). Mother and father were normal. All had normal neutrophil MPO staining. Phagocytic neutrophil 02 consumption expressed in nanomoles/min/12脳106 cells was DJ (.00), SJ (.01), and ...
DECREASED TUMOR CYTOLYSIS OF NEUTROPHILS (PMN) FROM PATIENTS WITH CHRONIC GRANULOMATOUS DISEASE (CGD)Pediatric Research publishes original papers, invited reviews, and commentaries on the etiologies of diseases of children and disorders of development, extending from molecular biology to epidemiology. Use ...