Nephrotic syndrome in children

Nephrotic syndrome is the commonest glomerular disease affecting children and is frequently encountered in general paediatrics. The most common subtype is minimal change nephrotic syndrome which typically occurs in preschool children and responds well to corticosteroids. Another subtype, congenital nephrotic syndrome, also presents in the neonatal period or in early infancy when it may be associated with genetic mutations. Such patients do not respond to immunosuppressant therapy. The study of congenital nephrotic syndromes has identified several causative genetic mutations and there have been significant recent advances in the understanding of disease mechanisms in nephrotic syndrome. Complications of prolonged nephrosis, seen in congenital, frequently relapsing, steroid dependent and particularly steroid resistant nephrotic syndromes can lead to significant morbidity. These include sepsis, thrombosis, hypothyroidism and hyperlipidaemia. It is important to identify and manage these complications. Children with nephrotic syndrome should complete the standard vaccination schedule and in addition should be vaccinated against varicella zoster. Live vaccines should be administered when children are in disease remission and on minimal immunosuppression. The management options for children with frequently relapsing or steroid resistant nephrotic syndrome include additional immunosuppression and several agents have been used in this context. These agents will often require additional therapeutic monitoring. © 2009.