Journal
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
Volume 27, Issue 1, Pages 89-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.hoc.2012.11.002
Keywords
Chronic granulomatous disease; Gene defects; NADPH oxidase; Immune defect; Inflammatory bowel disease
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Funding
- Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health
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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.
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