[Nephrotic syndrome in infancy: clinical, therapeutic and follow up characteristics in 100 cases]. / Síndrome nefrótico de la infancia: características clínicas, terapéuticas y evolutivas en 100 casos.

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)

[Metatropic dysplasia: a new case]. / Displasia metatrópica: un nuevo caso.

A new case of metatropic dysplasia in a 2.5 month old child is presented. Diagnoses was made in base of special face, long thorax, shortness of extremities with restricted joint mobility, kyphoscoliosis and a caudal appendage overlying the sacrum. Radiologically, expanded and irregular metaphyses of shortened tubular bones, platyspondyly and kyphoscoliosis. With growth, patients develop changes in body proportions. This syndrome is transmitted as an autosomal recessive. Prevention by genetic counseling is the principal measure.