Chronic granulomatous disease of the lung case
report
C. Mihail Strantzia, A. Garoufi, S. Drakonaki
“Ñ.Á. Kyriakou”, Childrens Hospital. Pathology Department, and
B´ Paediatric Clinic, Áthens
Á case of Chronic Granulomatous disease with lung involvement
in a 11 years old boy is described. The disease was presented
with persistent fever despite antibiotic treatment, and a circumscribed
lesion of the upper lobe of the right lung. Histologically there
was the picture of a necrotizing granulomatous bronchopneumonia.
The diagnosis of Chronic Granulomatous disease was confirmed by
a completely negative reduction of NBT dye in patients neutrophils.
Western blot analysis showed a clear deficiency of the p47-phox
component of the NADPH oxidase enzyme of his neutrophils but a
normal expression in the cells from his parents. These results
indicate the autosomal recessive form of genetic transmission
of the disease.
Key words: Chronic Granulomatous disease, dysfunction,
neutrophils, lung.
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