[Congenital nephrotic syndrome of Finnish type (report of a case)].
The case reported was that of a 3-month-old infant without important history. The onset of the disease took place one week before admission with edema, abdominal distention, diarrhea and vomiting. At the physical examination, he showed anasarca with important ascites and a right pneumonic process. The laboratory tests reported anemia, massive proteinuria, hypocalcemia, hypocomplementemia and drop of IgG. A renal biopsy showed cystic dilatation of proximal and distal tubuli. The electronic microscopy, fusion of podocytes and the immunoflorescence, focal deposits of IgG and fibrin. The management was difficult and the course insidious. Death followed septicemia 38 days after admission. The hereditary, etiopathogenetic and clinical characteristics are discussed and compared with other national and foreign reports.
D005260:Female; D005387:Finland; D006801:Humans; D007223:Infant; D007668:Kidney / Q000473:pathology; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000473:pathology
Síndrome nefrótico congénito de tipo finlandés (presentación de un caso).