Corticoresistant nephrotic syndrome associated with T-cell deficiency in two sisters.

Tabin, R; Guignard, J P; Gautier, E; Dubrit, M; Jeannet, M; Golaz, J; Moretta, L

Tabin, R; Guignard, J P; Gautier, E; Dubrit, M; Jeannet, M; Golaz, J; Moretta, L.

Pediatrics ; 71(1): 93-6, 1983 Jan.

Article en En | MEDLINE | ID: mdl-6600290

RESUMEN

The coexistence in two sisters, born to related parents, of a corticoresistant nephrotic syndrome, lymphopenia, an immune deficit, short stature, and photophobia is described. The immune deficit is mainly cellular; studies of lymphocyte markers demonstrate a pronounced deficiency of T lymphocytes and Fc-mu receptor-bearing cells. It is suggested that a thorough examination of number and function of T cells should be performed in patients with a familial corticoresistant nephrotic syndrome and recurrent infectious episodes before considering immunosuppressive treatment.

Asunto(s)

Síndrome Nefrótico/genética; Linfocitos T/inmunología; Niño; Preescolar; Consanguinidad; Femenino; Glomeruloesclerosis Focal y Segmentaria/genética; Glomeruloesclerosis Focal y Segmentaria/inmunología; Humanos; Síndromes de Inmunodeficiencia/genética; Recuento de Leucocitos; Linfopenia/genética; Síndrome Nefrótico/tratamiento farmacológico; Síndrome Nefrótico/inmunología; Prednisona/uso terapéutico; Formación de Roseta

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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Linfocitos T / Síndrome Nefrótico Tipo de estudio: Risk_factors_studies Límite: Child / Child, preschool / Female / Humans Idioma: En Revista: Pediatrics Año: 1983 Tipo del documento: Article Pais de publicación: Estados Unidos

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