Síndrome nefrótica primária grave em crianças: Descrição clínica e dos padrões histológicos renais de seis casos

Abstract

The authors report six children with severe primary nephrotic syndrome with unusual renal histological patterns in the daily routine of nephrologists and pathologists. The diagnosis of the disease was made at the age between 3 to 9 months (n = 4), at 2 years and 4 months (n = 1) and at 11 years (n = 1). One patient was born prematurely; two patients were sisters and their parents were first-degree cousins. All patients presented generalized edema, two patients presented malnutrition and hypothyroidism; two patients presented hypertension and renal failure. The renal histology showed diffuse mesangial sclerosis (n = 3); diffuse mesangial hypercellularity (n = 2) and nephrotic syndrome of the Finnish type (n = 1). Four patients died, causes of death were infection (n = 2), renal failure (n = 1) and metabolic acidosis (n = 1). Among the survivors, one patient was treated with vitamins, thyroxine, captopril and indomethacin and presented increase in serum albumin and of growth. The other patient, who presented end-stage renal disease, was dialyzed and transplanted.