The idiopathic nephrotic syndrome.

Abstract

The nephrotic syndrome is defined by edema, hypoalbuminemia, proteinuria, and hyperlipemia with elevated cholesterol and triglyceride concentrations in serum or plasma. It may be primary or secondary to systemic disorders. Because of its incidence--between 2.0 and 7.0 cases per 100,000 children per year--this disorder is not uncommon in a busy pediatric practice, and it forms the basis of the referrals evaluated by a pediatric nephrology service. Over the 2 years since this subject was reviewed, several articles have appeared that have provided insights into the clinical features, etiology, pathology, pathogenesis, complications, and therapy of the idiopathic primary nephrotic syndrome. Nevertheless, a remarkable feature of this condition remains that the fundamental cause and pathogenesis of the most common form of primary nephrotic syndrome, minimal lesion nephrotic syndrome of childhood, is largely unknown. Hence, a huge challenge remains for researchers in the area of pediatric nephrology.