Familial early-onset nephrotic syndrome: diffuse mesangial sclerosis. Clinico-pathological study of a kindred

Four children from two related families are described with a clinical picture characterized by the onset of asymptomatic proteinuria with subsequent nephrotic syndrome in infancy, and progression to renal failure and death before the age of three years. The clinical picture and the renal pathological studies were consistent with the entity described by Habib as infantile mesangial sclerosis. We propose that this entity possibly represents a genetic disorder which is transmitted as an autosomal recessive trait.