Chronic Granulomatous Disease in LibyaAbstract:
Chronic granulomatous disease (CGD) is a rare inherited disorder of the innate immune system, characterized by a greatly increased susceptibility to severe infections, early in childhood at different sites. We aimed to study multisystem clinical manifestations of CGD in Libyan children. Our study was retrospective, it included CGD patients seen at Pediatric hospital immunology department in Benghazi, over 7 years. Out of 35 CGD patients, 66% was males, 94% had onset of disease <1 year of life, and 60% of their parents were relatives. Reticuloendothelial system was involved in 94% followed by skin (91%), respiratory (83%), gastrointestine (69%) and bone (29%). This study is one of the largest series on CGD from north Africa and Arabic region reflecting the different modes of inheritance, as well as the wide and variable clinical manifestations of CGD.