Inheritance Pattern and Clinical Aspects of 93 Iranian Patients with Chronic Granulomatous Disease

Background Chronic granulomatous disease (CGD) is a rare immunodeficiency due to a genetic defect in one of the NADPH-oxidase components. We studied CGD inheritance forms (autosomal recessive (AR) or X-linked (XL)) and AR-CGD subtypes in Iran. Methods Clinical and functional investigations were cond...

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Veröffentlicht in:	Journal of clinical immunology 2011-10, Vol.31 (5), p.792-801
Hauptverfasser:	Fattahi, Fatemeh, Badalzadeh, Mohsen, Sedighipour, Leyla, Movahedi, Masoud, Fazlollahi, Mohammad Reza, Mansouri, Seyed Davood, Khotaei, Ghamar Taj, Bemanian, Mohammad Hassan, Behmanesh, Fatemeh, Hamidieh, Amir Ali, Bazargan, Nasrin, Mamishi, Setareh, Zandieh, Fariborz, Chavoshzadeh, Zahra, Mohammadzadeh, Iraj, Mahdaviani, Seyed Alireza, Tabatabaei, Seyed Ahmad, Kalantari, Najmeddin, Tajik, Shaghayegh, Maddah, Marzieh, Pourpak, Zahra, Moin, Mostafa
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Age of Onset Biomedical and Life Sciences Biomedicine Child Child, Preschool Consanguinity DNA Mutational Analysis Female Genes, Recessive - genetics Genes, X-Linked - genetics Granulomatous Disease, Chronic - epidemiology Granulomatous Disease, Chronic - genetics Granulomatous Disease, Chronic - physiopathology Humans Immunology Infant Infectious Diseases Internal Medicine Iran Lymphatic Diseases Male Medical Microbiology Middle Aged NADPH Oxidases - genetics Respiratory Tract Infections Risk Factors
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Zusammenfassung:	Background Chronic granulomatous disease (CGD) is a rare immunodeficiency due to a genetic defect in one of the NADPH-oxidase components. We studied CGD inheritance forms (autosomal recessive (AR) or X-linked (XL)) and AR-CGD subtypes in Iran. Methods Clinical and functional investigations were conducted in 93 Iranian CGD patients from 75 families. Results Most of the patients were AR-CGD (87.1%). This was related to consanguineous marriages ( p = 0.001). The age of onset of symptoms and diagnosis were lower in XL-CGD compared with AR-CGD ( p
ISSN:	0271-9142 1573-2592
DOI:	10.1007/s10875-011-9567-x