Síndrome nefrótico en el primer año de vida

Abstract

Among 99 children enrolled to a chilean multicentric protocol on nephrotic syndrome along a 30 months period, eight patients (five girls) are reported which were younger than 1 year of age (median 7.5 months). Clinical symptoms begun before age 10 days in three of them, microscopic hematuria was observed in saveral arterial hypertension and acute renal failure were respectively found in four cases each laboratory findings in nephrotic infants were not different from those in older children with the syndrome. Known causes of secondary nephrotic syndrome were discarded in these patients. Histopathological studies were done in six cases and showed diffuse mesangial sclerosis (n = 2) (in one infant it was associated to Drash's syndrome); finnish type (n = 2); foca segmental gomerulosclerosis (n = 1) and minimal change nephrotic syndrome (n = 1) Six patients were given prednisone treatment: three were considered to be steroid resistant (one patient each of the finnish type, diffuse mesangial sclerosis and focal segmental nephrosclerosis): two were steroid dependent [one without biopsy and another one with minimal change disease], and one was steroid responsive. Cyclophosphamide was used in two patients (minimal change and focal segmental nephrosclerosis) with a favourable outcome. Severe bacterial infections were observed in seven and thrombosis in two affected infants. Four patients died, two had diffuse mesangia sclerosis (both of septic shock) and the other two the finnish type (one of pulmonary artery thrombosis), in all of them clinical disease begun before age four months. Currently four patients remain alive, three are under remission (after 36, 37 and 39 month follow-up time) and one has persistent protenuria (after 17 months follow-up). Prognosis is poor in patients with nephrotic syndrome associated to diffuse mesangial sclerosis or finnish type beginning before age four months. Nephrotic infants with focal segmental nephrosclerosis and minimal change disease behave like older pediatric patients with this syndrome.