[Association of Wilms' tumor, pseudohermaphroditism and glomerular nephropathy. Partial and complete forms of the syndrome].
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.
D000328:Adult; D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000473:pathology*; D009396:Wilms Tumor / Q000473:pathology*
Asociación de tumor de Wilms, pseudohermafroditismo y nefropatía glomerular. Formas incompletas y completa del síndrome.