[Chronic granulomatous disease: pathogenesis and therapy of associated fungal infections].

Abstract

Chronic granulomatous disease is a genetic disorder responsible for a defect in the NADPH oxidase of phagocytic cells. It impairs the oxidative burst necessary to the intracellular inactivation of microorganisms and predisposes to an increased risk of infections by various microorganisms, including fungi like Aspergillus spp. and other less frequently encountered or emerging fungal species. Here we review the genetic basis, pathogenesis and clinical presentation associated with fungal infections in chronic granulomatous disease as well as the current prophylaxis and newly available therapies.

• Authors: Anne Desjardins, Hélène Coignard-Biehler, Nizar Mahlaoui, Pierre Frange, Marie-Elisabeth Bougnoux, Stéphane Blanche, Alain Fischer, Sophie Blumental, Olivier Lortholary
• Journal: Medecine sciences : M/S (Med Sci (Paris)) Vol. 28 Issue 11 Pg. 963-9 (Nov 2012) ISSN: 0767-0974 [Print] France
• Vernacular Title: Infections fongiques et granulomatose septique chronique - De la physiopathologie aux nouvelles perspectives thérapeutiques.
• PMID: 23171900 (Publication Type: Journal Article, Review)
• Copyright: © 2012 médecine/sciences – Inserm / SRMS.

Chemical References

• Anti-Inflammatory Agents
• Antifungal Agents
• Indoleamine-Pyrrole 2,3,-Dioxygenase
• Protein Subunits
• Tryptophan
• NADPH Oxidases

Topics

• Animals
• Anti-Inflammatory Agents (therapeutic use)
• Antifungal Agents (therapeutic use)
• Aspergillosis (etiology, immunology, therapy)
• Disease Susceptibility
• Genetic Therapy
• Granulomatous Disease, Chronic (complications, enzymology, epidemiology, immunology, surgery)
• Hematopoietic Stem Cell Transplantation
• Humans
• Immunocompromised Host
• Incidence
• Indoleamine-Pyrrole 2,3,-Dioxygenase (metabolism)
• Lymphocyte Subsets (immunology)
• Mice
• Mycoses (epidemiology, etiology, immunology, prevention & control, therapy)
• NADPH Oxidases (deficiency, genetics, physiology)
• Neutrophils (enzymology, immunology)
• Protein Subunits
• Respiratory Burst
• Tryptophan (metabolism)