CHALLENGING INFANTILE NEPHROTIC SYNDROME – MANAGEMENT AND DIAGNOSTIC ISSUES

Abstract

Infantile nephrotic syndrome (INS) is a kidney disorder characterized by nephrotic syndrome presenting between 4 and 12 months of age with hypoalbuminemia, ( 40 mg/m2 ) and edema. Children with infantile nephrotic syndrome appear normal at birth, proteinuria with a bland urine sediment develops postnatally, increasing progressively during the first or the second year of life. From the point of view of etiology, nephrotic syndrome may be idiopathic, genetic or secondary. Renal histopathology is not anymore a key criterion for diagnostic and prognostic in children with SRNS (steroid resistant nephrotic syndrome), having limited value in distinguishing genetic from nongenetic etiologies. Genetic podocytopathies changed diagnostic, prognostic judgment and therapeutic approaches in early onset SRNS. Therapeutic decisions are based on the underlying etiology.

Keywords

• infantile nephrotic syndrome
• genetic basis
• genetic podocytopathies