Residual NADPH oxidase and survival in chronic granulomatous disease.

ABSTRACT:

Background

Failure to generate phagocyte-derived superoxide and related reactive oxygen intermediates (ROIs) is the major defect in chronic granulomatous disease, causing recurrent infections and granulomatous complications. Chronic granulomatous disease is caused by missense, nonsense, frameshift, splice, or deletion mutations in the genes for p22(phox), p40(phox), p47(phox), p67(phox) (autosomal chronic granulomatous disease), or gp91(phox) (X-linked chronic granulomatous disease), which result in variable production of neutrophil-derived ROIs. We hypothesized that residual ROI production might be linked to survival in patients with chronic granulomatous disease.

Methods

We assessed the risks of illness and death among 287 patients with chronic granulomatous disease from 2

SUBMITTER: Kuhns DB 

PROVIDER: S-EPMC3069846 | biostudies-literature | 2010 Dec

REPOSITORIES: biostudies-literature