RESUMEN

Four patients, followed at the Buenos Aires Cildren's Hospital are reported: one with a nephrotic syndrome and male pseudohermaphroditism, the second with nephrotic syndrome, male pseudohermaphroditism and Wilms' tumor, the third with pseudohermaphroditism and Wilms' tumor and the fourth with a nephrotic syndrome and Wilms' tumor. The cases presenting nephrotic syndrome, all had an early onset and a rapid and fatal course leading to death in renal failure or secondary to a related bacterial infection. The renal histology in all of the cases with nephrotic syndrome, was very similar: diffuse involvement of all glomeruli consisting in a severe increase of the mesangial matrix, with scarce mesengial proliferation.

Asunto(s)

Trastornos del Desarrollo Sexual/patología , Síndrome Nefrótico/patología , Tumor de Wilms/patología , Adulto , Preescolar , Femenino , Humanos , Lactante , Glomérulos Renales/patología , Masculino

Asunto(s)

Equilibrio Ácido-Base , Enfermedades Renales/fisiopatología , Adolescente , Cloruro de Amonio/orina , Bicarbonatos/orina , Niño , Preescolar , Femenino , Tasa de Filtración Glomerular , Humanos , Concentración de Iones de Hidrógeno , Lactante , Masculino , Tasa de Depuración Metabólica , Orina