Clinical and Radiological Aspects of Chronic Granulomatous Disease in Children: A case Series from Iran

Chronic granulomatous disease (cgd) is a rare disorder of phagocytes, predisposes patientsto bacterial and fungal infections. the main purpose of this study was to determine the clinical,radiological, pathologicial features, outcome and response to treatment of children with cgd.thirteen patients with cgd, who had been referred to national research institute oftuberculosis and lung disease (nritld), were reviewed during a 6 year period (1999-2005).there were 10 (76%) male and 3(24%) female cases. the median age of the patients was 9 years(1 month-12 years).family history of cgd was reported by 7 patients. the median diagnosticage was 8 years, with a diagnostic delay of 4.5 years. the most common manifestations of cgdwere pulmonary infections and skin involvement, followed by generalized lymphadenopathy.the most common radiological findings were multiple lymphadenopathy in mediastinal regionand fibrotic changes in lung fields. two patients died of pulmonary infections. based on theresults of this research, immunologic evaluations especially evaluation for cgd is highlyrecommended in children suffering from recurrent pulmonary infections, cutaneous or hepaticabscesses, or infections caused by uncommon pathogens. early diagnosis and prophylactictreatment both, prevent further development of the lesions, irreversible complications anddecreasing mortality and morbidity rates in children suffering from cgd.