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Pulmonary manifestations of chronic granulomatous disease

Seyed AlirezaMahdaviani*1, Seyed Amir Mohajerani2, Nima Rezaei3, Jean-Laurent Casanova4, Seyed Davood Mansouri2 and Ali Akbar Velayati5

1Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
2Chronic Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3Research Center for Immunodeficiencies, Children’s Medical Center; and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
4St Giles Lab of Human Genetics of Infectious Diseases, The Rockefeller University, NY, USA
5Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran

 

Chronic granulomatous disease (CGD) is an inherited disorder, characterized by defects in superoxide-generating NADPH oxidase of phagocytes. The genetic defects in CGD induce failure to activate the respiratory burst in the phagocytes, leading to severe recurrent infections and unexplained prolonged inflammatory reactions that may produce granulomatous lesions. A noble advance in curative therapy for CGD is hematopoietic stem cell transplantation. Since the most common site of involvement in CGD is the lung, the pulmonologists, pediatrics or adult may be among the first to recognize the pattern of infection, inflammation and granuloma formation, leading to diagnosis of CGD. Pulmonologists need to be aware of different lung manifestations of CGD

Keywords: chronic granulomatous disease • manifestation • pulmonary • radiology • treatment

Download ZIP Download PDF                                       Expert Rev. Clin. Immunol. 9(2), 153–160; 2013

 

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