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chronic granulomatous disease

 

Definition: a congenital defect in the killing of phagocytosed bacteria by polymorphonuclear leukocytes, which cannot increase their oxygen metabolism due to dysfunctional NADPH oxidase, either because of defective cytochrome [MIM*233710 and MIM*233690] or other specific factor deficiencies [MIM*233700 and MIM*306400]. As a result there is an increased susceptibility to severe infection by catalase-positive microorganisms; inheritance is usually autosomal recessive or X-linked.

Synonym(s): congenital dysphagocytosis, granulomatous disease

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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