Chronic granulomatous disease

Hematol Oncol Clin North Am. 2013 Feb;27(1):89-99, viii. doi: 10.1016/j.hoc.2012.11.002. Epub 2012 Nov 22.

Abstract

Chronic granulomatous disease (CGD) is a paradigm for nonlymphoid primary immune defects, and has guided elucidation of oxygen metabolism in the phagocyte, vasculature, and brain. It has been in the forefront of the development of antimicrobial prophylaxis before the advent of advanced HIV and before its routine use in neutropenia. It has been an attractive target for gene therapy and bone marrow transplantation for nonmalignant diseases. Therefore, CGD is worthy of attention for its historical interest and because it is a disease for which expert management is imperative.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Animals
  • Granulomatous Disease, Chronic* / complications
  • Granulomatous Disease, Chronic* / diagnosis
  • Granulomatous Disease, Chronic* / etiology
  • Granulomatous Disease, Chronic* / therapy
  • Humans
  • Mutation
  • NADPH Oxidases / genetics
  • NADPH Oxidases / metabolism
  • Neutrophils / immunology
  • Neutrophils / metabolism

Substances

  • NADPH Oxidases