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Revista Cubana de Hematología, Inmunología y Hemoterapia

versión impresa ISSN 0864-0289versión On-line ISSN 1561-2996

Resumen

COS PADRON, Yanelkys; MARSAN SUAREZ, Vianed; SANCHEZ SEGURA, Miriam  y  MACIAS ABRAHAM, Consuelo. Chronic granulomatous disease: Current aspects. Rev Cubana Hematol Inmunol Hemoter [online]. 2004, vol.20, n.3. ISSN 0864-0289.

Chronic granulomatous disease is a primary immunodeficiency caused by mutations in genes encoding any of the 4 subunits that make up adenine dinucleotide phosphate oxidase (NADPH oxidase), the enzyme that regulates the production of microbial oxidizing species that are the first defensive pathway of the body against infectious microorganisms. This is a very heterogeneous deficiency classified as X chromosome-linked CGD (gp91-phox subunit) and as autosomal recessive chronic granulomatous disease, where any of the subunits of the enzyme, that is, p22-phox, p47-phox and p67-phox, may be affected. The most frequent clinical findings include, among others, lymphadenopathy, splenohepatomegaly and pneumonia. Broad-spectrum antibiotics for the treatment of the disease and gamma interferon for the treatment of severe infections are used in X chromosome-linked chronic granulomatous disease. At present, studies are being carried out with a view to using somatic gene therapy as a likely disease cure

Palabras clave : chronic granulomatous disease; NADPH oxidase; phagocytes; immunodeficiency.

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