title: Chronic granulomatous disease presenting with hypogammaglobulinemia.
abstract: Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder caused by inherited defects in the nicotinamide adenine dinucleotide phosphate oxidase complex. The neutrophils of patient with CGD can ingest bacteria normally, but the oxidative processes that lead to superoxide anion formation, hydrogen peroxide production, nonoxidative pathway activation, and bacterial killing are impaired. Serious infections result from microorganisms that produce catalase. Immunoglobulin levels of patients with CGD are usually normal or elevated. We describe a patient with CGD associated with hypogammaglobulinemia, an unusual co-occurrence.
authors: Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors: D
authors: D
authors: Hanoglu
authors: T T
authors: TT
authors: Ozgür
authors: D
authors: D
authors: Ayvaz
authors: M Y
authors: MY
authors: Köker
authors: O
authors: O
authors: Sanal
mesh_terms: D000361:Agammaglobulinemia / Q000209:etiology* / Q000628:therapy; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D001424:Bacterial Infections / Q000188:drug therapy / Q000209:etiology; D002181:Candidiasis, Vulvovaginal / Q000188:drug therapy / Q000209:etiology; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000175:diagnosis; D003241:Consanguinity; D004198:Disease Susceptibility; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D006801:Humans; D016867:Immunocompromised Host; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D018655:Lymphocyte Count; D016131:Lymphocyte Subsets / Q000473:pathology; D011628:Puberty, Delayed / Q000209:etiology; D012008:Recurrence; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D000935:Antifungal Agents; D016756:Immunoglobulins, Intravenous