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Br Med Bull
2016[Jun]; 118
(1
): 50-63
PMID26983962
show ga
INTRODUCTION: Chronic granulomatous disease (CGD) is a primary immunodeficiency
characterized by recurrent, life-threatening bacterial and fungal infections of
the skin, the airways, the lymph nodes, the liver, the brain and the bones.
Frequently found pathogens are Staphylococcus aureus, Aspergillus species,
Klebsiella species, Burkholderia cepacia, Serratia marcescens and Salmonella
species. SOURCES OF DATA: CGD is a rare (?1:250 000 individuals) disease caused
by mutations in any one of the five components of the NADPH oxidase in phagocytic
leucocytes. This enzyme generates superoxide and is essential for intracellular
killing of pathogens by phagocytes. AREAS OF AGREEMENT: CGD patients suffer not
only from life-threatening infections, but also from excessive inflammatory
reactions. AREAS OF CONTROVERSY: Neither the cause of these inflammatory
reactions nor the way to treat them is clear. AREAS TIMELY FOR DEVELOPING
RESEARCH: Patient selection for and timing of bone marrow transplantation along
with gene therapy.
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