[Nephrotic syndrome in infancy: clinical, therapeutic and follow up characteristics in 100 cases]

In order to understand the natural history, clinical features, anatomopathological findings and response to the initial treatment with steroids of the nephrotic syndrome (NS) during childhood, a group of 100 children under 14 years (46 with renal biopsy) observed for an average period of 4 years and 8 months, was reviewed retrospectively. The patients were divided into 3 groups: idopathic NC (89), secondary NS (9) and NS in the first six months of life (2). In the group of idiopathic NS, in which the significant predominance of males (2.8/1) was confirmed, in 69% of the patients the diagnosis was made between the ages of 2 and 5 years. Edemas were found, sometimes accompanied by oliguria and weigh gain, in 77% of the cases. The periods of hypertension and hematuria were infrequent and brief. Thirty eight kidney biopsies were carried out and showed: 25 minimal changes NS, 7 diffuse mesangial proliferative glomerulonephritis (DMPGN), 3 membranoproliferative glomerulonephritis (MPGN), 2 membranous glomerulonephritis (MGN) and 1 segmental and focal glomerulosclerosis. Of the 83 patients treated in first instance with corticosteroids, a positive response was obtained in 72 (87%), although 44% of them have shown to be cortico-dependent. After an average follow up period of 4 years and 8 months, only 2 children, one with MPGN and the other with MGN, are now in chronic renal failure (CRF). In 5 ot the 9 patients included in the second group, the NS was secondary to a Henoch Schönlein purpura. The kidney biopsies showed DMPGN in three of them.(ABSTRACT TRUNCATED AT 250 WORDS)