Por favor, use este identificador para citar o enlazar este ítem:
http://repositorio.pediatria.gob.mx:8180/handle/20.500.12103/2265| Título : | Reporte de una familia con enfermedad granulomatosa crónica con afección en gp91 phox A report of a family with chronic granulomatous disease with gp91 phox disorder |
| Creador: | García Hernández A. |
| Nivel de acceso: | Open access |
| Palabras clave : | Enfermedad Granulomatosa Crónica - genética Humanos Lactante Masculino Glicoproteínas de Membrana - genética NADPH Oxidasa - genética Linaje Granulomatous Disease, Chronic - genetics Humans Infant Male Membrane Glycoproteins - genetics NADPH Oxidase - genetics Pedigree Chronic granulomatous disease gp91phox Mycobacterium infections |
| Descripción : | The chronic granulomatous disease is a primary immunodeficiency with a defect of the phagocytosis process; its main alteration resides in the incapacity of the NADPH oxidase system to produce reactive oxygen species capable of destruct pathogenic organisms such as bacteria, fungus and mycobacteria. Patients are susceptible to severe and mild infections, mainly pneumonias, linfadenitis and gastroenteritis that tend to be repetitive; in addition, they presented granulomatous inflammation and autoimmunity. We presented the case of two brothers with X-linked chronic granulomatous disease with alteration in the sub-unit gp91phox; heredofamilial background was endogamy and consanguinity. Both patients suffered severe infections, frequent abscesses and a poor growth. Diagnosis was confirmed with nitroblue tetrazolium test. During their evolution, the patients presented also BCGitis, BCGosis and septic shock. They began prophylactic treatment with trimetoprim sulfametoxazole and itraconazole, as well as gamma interferon, with favorable response, presenting a lower amount of infectious episodes, as well as a recovery of their weight and height. The early diagnosis of the patients has improved their prognosis. |
| Colaborador(es) u otros Autores: | Lugo Reyes S. Yamazaki Nakashimada M.A. Gonzáles Serrano M.E. Espinosa Rosales F. Blancas Galicia L. |
| Fecha de publicación : | 2010 |
| Tipo de publicación: | Artículo |
| Formato: | |
| Fuente: | Revista Alergia México 57(2):60 - 65 |
| URI : | http://repositorio.pediatria.gob.mx:8180/handle/20.500.12103/2265 |
| Idioma: | spa |
| Aparece en las colecciones: | Artículos |
Ficheros en este ítem:
No hay ficheros asociados a este ítem.
Los ítems de DSpace están protegidos por copyright, con todos los derechos reservados, a menos que se indique lo contrario.