CHRONIC GRANULOMATOUS DISEASE TREATED WITH INTERFERON GAMMA
A. Nemeth, E. Szepesvari, J. Muller, Zs. Hajmassy,
M.Schmidt, Gy. Fekete
Semmelweis University Faculty of Medicine
II.Department of Pediatrics, Budapest, Hungary
Chronic granulomatous disease (CGD) is a rare inherited
disorder of white blood cells. Phagocytes could not generate microbicidal
reactive oxidant superoxide anion and its metabolites as a result of the
failure of cytochrome b558 nicotinamide dinucleotide phosphate
(NADPH) complex. This fact leads to a serious defect in the host defense
pathway. As a result CGD patients suffer from recurrent life-threatening
bacterial and fungal infections and granuloma formation due to the
abnormally exuberant inflammatory responses.
A follow up of a 18 years-old boy with CGD was performed
in our Department. The diagnosis was first made up according to the
abnormal phagocyte funciton tests and NBT test in 1985, when the child was
20 months old. In 1994 we confirmed the diagnosis with genetic studies (CYBB
gene, C-880 ®T).
In the course of his illness several complications were observed including
osteomyelitis, pneumonia, Salmonella sepsis, cholecystitis, cerebral
abscess, parainfectious encephalitis, pulmonary and lumbosacral granulomas.
In 1997 we introduced interferon-gamma (INF-c) in his therapeutical
regime. Since that time he had no severe infections, and the number of
inpatient days decreased. His quality of life improved significantly.
INF-c seems to be an effective
therapeutic agent in the treatmant of CGD.