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[Progressive-septic granulomatosis: improved prognosis with early diagnosis and targeted therapy. Report of 5 cases].

作者信息

Kremens B, Seger R A, von Voss H, Wahn V

出版信息

Monatsschr Kinderheilkd. 1985 May;133(5):284-90.

PMID:3925328
Abstract

The clinical and laboratory findings in chronic granulomatous disease are illustrated by five case reports. Biochemical studies of the neutrophil bactericidal defect have revealed several molecular forms of the disease. Specific therapeutic action is nowadays possible, after early diagnosis of the condition by nitroblue-tetrazolium test and chemiluminescence. Infections are treated using antibiotics and antimycotics which penetrate well into granulocytes; additional surgical intervention or granulocyte transfusion may be necessary. Prolonged infection-free periods are achieved under prophylactic sulfamethoxazole/trimethoprim therapy, promising an improved prognostic outlook for patients with chronic granulomatous disease.

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