Chronic Granulomatous Disease: A Recent Approach

Abstract

Chronic granulomatous disease (CGD) is a fatal inborn error of immunity due to defective respiratory burst in phagocytes resulting in recurrent bacterial and fungal infection. It has to be suspected when in early infancy children presents with recurrent bacterial,fungal infection in the form of pneumonia,deep seated abscess.In Indian setting in tubercular infections presenting as persistent pneumonia CGD has to be considered.It is screened by Dihydro rhodamine assay and confirmed by molecular genetic testing.Prophylactic antibiotics, antifungal administration, aggressive management of infections and its complicationare key for longterm survival.Hematopoetic stem cell transplantation (HSCT) remains only curative treatment at present and future lies in gene therapy.