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Pulmonary manifestations of chronic granulomatous disease.

Abstract
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.
AuthorsSeyed Alireza Mahdaviani, Seyed Amir Mohajerani, Nima Rezaei, Jean-Laurent Casanova, Seyed Davood Mansouri, Ali Akbar Velayati
JournalExpert review of clinical immunology (Expert Rev Clin Immunol) Vol. 9 Issue 2 Pg. 153-60 (Feb 2013) ISSN: 1744-8409 [Electronic] England
PMID23390946 (Publication Type: Journal Article, Review)
Chemical References
  • NADPH Oxidase
Topics
  • Adult
  • Animals
  • Child
  • Diagnosis, Differential
  • Granuloma (etiology, prevention & control)
  • Granulomatous Disease, Chronic (complications, diagnosis, therapy)
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Lung (pathology)
  • Mutation (genetics)
  • NADPH Oxidase (genetics, metabolism)
  • Phagocytes (metabolism)

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