Chronic granulomatous disease

Abstract

Chronic Granulomatous Disease (CGD) is an uncommon inherited primary immunodeficiency, that can lead to life-threatening infections due to defect in phagocytes to kill some microbes. The incidence is variable among different countries, and the clinical course range from mild to severe disease. This study was carried out to increase the awareness of the disease among medical media which is very deficit especially in the absence of diagnostic investigations in most centers in Libya.To the best of our knowledge, this survey can be considered as the largest Libyan study which demonstrated the clinical characteristics of 54 CGD patients. Male cases were 34 out of the surveyed patients. The parents were consanguineous in 30 CGD patients (55.6%).About 16 patients were from Benghazi city and 38 were from outside Benghazi.The most common manifestation in CGD patients was infection in the lymph nodes (87%), followed by skin infection (85%), infection in gastrointestinal tract (77%), lung infection (72%), BCG disease (44%), urinary tract infection and septicemia (33%), bone and joint infections (19%), inflammatory complications and brain infection (11%) and infection in the liver (9%). 33.3% of patients received antibiotic prophylaxis, 40.7% received combined antibiotic plus antifungal prophylaxis, while 26% underwent hematopoietic stem cell transplantation (HSCT).