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1.
Invest. clín ; Invest. clín;53(2): 157-167, jun. 2012. ilus
Article in English | LILACS | ID: lil-664574

ABSTRACT

Chronic Granulomatous Disease (CGD) is a primary immunodeficiency characterized by defects in superoxide (O2-) production, which result from mutations in one of the four NADPH oxidase components, predisposing to bacterial and fungal infections. Besides the O2-defect, it has been described that neutrophils from CGD patients are resistant to cell death, a phenomenon that has been connected to chronic inflammation and predisposition to autoimmune diseases. A diminished expression of Fas and its counterpart FasL, molecules known to play a major role in cell death, has been described in lymphocytes depleted of O2-reactive oxygen species (ROS), suggesting an involvement of ROS in Fas/FasL expression. In this work, Fas and FasL expressions were analyzed in T cells and neutrophils from two CGD families, previously known to harbor two different molecular defects: absence of either p47-phox or p67-phox. We found that T lymphocytes from CGD patients express low levels of Fas and FasL, while a diminished FasL expression was observed on neutrophils from a CGD A470 patient. These defects may contribute to understand altered cell death in CGD patients.


La Enfermedad Granulomatosa Crónica (EGC) es una inmunodeficiencia primaria caracterizada por un defecto en la producción de superóxido (O2-), que se genera como consecuencia de mutaciones en uno de los cuatro componentes del complejo NADPH oxidasa y predispone a infecciones por bacterias y hongos. Además de los defectos en la producción de O2-, se ha descrito que los neutrófilos de los pacientes con EGC exhiben una resistencia a la muerte celular, evento que se asocia con la inflamación crónica y predisposición a enfermedades autoinmunes. Se ha descrito que linfocitos en medios desprovistos de O2-especies reactivas del oxigeno (ROS), muestran reducida expresión de Fas y FasL, moléculas que juegan un papel relevante en el control de la muerte celular, sugiriendo la participación de los ROS su regulación. En este trabajo analizamos la expresión de Fas y FasL en linfocitos T y neutrófilos en dos familias portadores de dos defectos genéticos diferentes asociados con EGC: ausencia de p47-phox o de p67-phox. Evidenciamos una baja expresión de Fas y FasL en los linfocitos T de los pacientes con EGC, pero solo los neutrófilos de los pacientes con defecto de p47-phox, fueron incapaces de expresar FasL. Estos defectos pudieran contribuir a entender la alteración de la muerte celular observada en los pacientes con EGC.


Subject(s)
Adolescent , Adult , Female , Humans , Young Adult , /biosynthesis , Fas Ligand Protein/biosynthesis , Granulomatous Disease, Chronic/metabolism , Leukocytes/metabolism
2.
Article in Spanish | LILACS | ID: lil-652122

ABSTRACT

La enfermedad granulomatosa crónica es una enfermedad hereditaria rara producida por un defecto en el metabolismo de oxidación de las células fagocíticas, que afecta la capacidad microbicida y da como resultado una tendencia a presentar infecciones recurrentes por hongos y bacterias en la piel y en las superficies epiteliales. Se presenta un caso clínico.


Subject(s)
Granulomatous Disease, Chronic/metabolism , Infections , Skin Diseases, Infectious
3.
Rev. paul. med ; 111(6): 472-6, nov.-dez. 1993. tab, graf
Article in English | LILACS | ID: lil-130200

ABSTRACT

Of a total of 111 children with primary immunodeficiency, 20 had phagocytic disorders (18 per cent) and 10 of them (8 boys and 2 girls) were diagnosed as chronic granulomatous disease (CGD). The children presented with repeated infections already during the first months of life. The main clinical findings were: abscess (n=8), otitis (n=8), pneumonia (n=8), lymphadenitis and pyodermits (n=6) and septicemia (4), NBT reduction was almost absent in all the children, except one of them. Bactericidal activity against S. aureus and phagocytosis were impaired in CGD patients. Different patterns of laboratory tests and prognosis were observed and girls had a better evolution


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Adult , Granulomatous Disease, Chronic/diagnosis , Phagocytes/physiology , NADP/metabolism , Diagnosis, Differential , Granulomatous Disease, Chronic/classification , Granulomatous Disease, Chronic/metabolism , Prognosis
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