title: [Progressive-septic granulomatosis: improved prognosis with early diagnosis and targeted therapy. Report of 5 cases].
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.
mesh_terms: D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006098:Granulocytes / Q000637:transplantation; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007231:Infant, Newborn; D017053:Infection Control; D008163:Luminescent Measurements; D008297:Male; D009580:Nitroblue Tetrazolium; D010587:Phagocytosis; D011379:Prognosis; D013420:Sulfamethoxazole / Q000627:therapeutic use; D014295:Trimethoprim / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D000935:Antifungal Agents; D009580:Nitroblue Tetrazolium; D014295:Trimethoprim; D013420:Sulfamethoxazole
vernacular_title: Progressiv-septische Granulomatose: Verbesserte Prognose durch frühzeitige Diagnostik und gezielte Therapie. 5 Kasuistiken.