title: Pulmonary manifestations of chronic granulomatous disease.
abstract: Chronic granulomatous disease (CGD) is an inherited disorder, characterized by defects in superoxide-generating NADPH oxidase of phagocytes. The genetic defects in CGD induce failure to activate the respiratory burst in the phagocytes, leading to severe recurrent infections and unexplained prolonged inflammatory reactions that may produce granulomatous lesions. A noble advance in curative therapy for CGD is hematopoietic stem cell transplantation. Since the most common site of involvement in CGD is the lung, the pulmonologists (pediatrics or adult) may be among the first to recognize the pattern of infection, inflammation and granuloma formation, leading to diagnosis of CGD. Pulmonologists need to be aware of different lung manifestations of CGD.
authors: Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran. mahdavini@yahoo.com
authors: Seyed Alireza
authors: SA
authors: Mahdaviani
authors: Seyed Amir
authors: SA
authors: Mohajerani
authors: Nima
authors: N
authors: Rezaei
authors: Jean-Laurent
authors: JL
authors: Casanova
authors: Seyed Davood
authors: SD
authors: Mansouri
authors: Ali Akbar
authors: AA
authors: Velayati
mesh_terms: D000328:Adult; D000818:Animals; D002648:Child; D003937:Diagnosis, Differential; D006099:Granuloma / Q000209:etiology / Q000517:prevention & control; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008168:Lung / Q000473:pathology*; D009154:Mutation / Q000235:genetics; D019255:NADPH Oxidases / Q000235:genetics / Q000378:metabolism*; D010586:Phagocytes / Q000378:metabolism*
chemical_list: D019255:NADPH Oxidases