chronic granulomatous disease represents a group of inherited disorders of phagocytic system wherein recurrent infections are seen at different sites especially in the respiratory system. to determine the clinical spectrum of respiratory manifestations in chronic granulomatous disease patients, in this retrospective study, we used data from iranian primary immunodeficiency registry. the diagnos...

chronic granulomatous disease is an inherited defect in intracellular killing of ingested microorganisms characterized by recurrent life threatening bacterial and fungal infections including invasive aspergillosis in early childhood. we report  a  disseminated aspergillosis as  the  representative  of  adult  onset  chronic granulomatous disease without previous infection, with dramatic respons...

immunodeficiency and autoimmune disease may occur concomitantly in the same individual. some of the immunodeficiency syndromes, especially humoral defects are associated with autoimmune disorders. hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. persistent antigen stimulation due to an inherent defect in the ability of the immune syst...

chronic  granulomatous  disease (cgd)  is a rare primary immunodeficiency disorder characterized by a greatly increased susceptibility to severe fungal and bacterial infections caused  by  defects  in  nadph   oxidase  of  phagocytic  cells. we  aimed  to  investigate immunophenotype alterations of naïve and memory b cells and b1a cells in peripheral whole blood from iranian patients with cgd. ...

chronic granulomatous disease (cgd) is a rare primary immunodeficiency disease. although the most affected patients are diagnosed in childhood, there are several reports of the disease presenting in adult patients. here we present a 40 years old man who was admitted in hospital due to respiratory symptoms and ground glass pattern in high resolution computed tomography of lung. open lung biopsy ...

BACKGROUND Failure to generate phagocyte-derived superoxide and related reactive oxygen intermediates (ROIs) is the major defect in chronic granulomatous disease, causing recurrent infections and granulomatous complications. Chronic granulomatous disease is caused by missense, nonsense, frameshift, splice, or deletion mutations in the genes for p22(phox), p40(phox), p47(phox), p67(phox) (autoso...