Chronic Granulomatous Disease
Chronic granulomatous disease is a hereditary immunodeficiency disorder in which phagocytes (neutrophils, eosinophils, monocytes, and macrophages) malfunction.
In this disorder, neutrophils, eosinophils, monocytes, and macrophages do not produce hydrogen peroxide, superoxide, and other substances that kill certain bacteria and fungi. Chronic granulomatous disease usually affects boys.
Symptoms usually first appear during early childhood but sometimes not until adolescence. Chronic infections occur in the skin, lungs, lymph nodes, mouth, nose, and intestines. Pockets of pus (abscesses) can develop around the anus and in the lungs, bones, and liver. The lymph nodes tend to fill with bacteria and enlarge. The skin over the lymph nodes may break down. As a result, the abscess drains. The liver and spleen enlarge. Children may grow slowly.
Antibiotics are given continuously or intermittently. Interferon-gamma, injected 3 times a week, can reduce the number and severity of infections. Stem cell transplantation has been successful in some people but because of the risks is not usually recommended.
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