Treatment of steroid-resistant pediatric nephrotic syndrome |
Hee Gyung Kang (Kang HG) |
Division of Pediatric Nephrology, Department of Pediatrics, Research Center for Rare Diseases, Seoul National University Children`s Hospital, Seoul National University College of Medicine, Seoul, Korea |
Corresponding Author:
Hee Gyung Kang ,Tel: +82-2-2072-0658, Fax: +82-2-2072-0658, Email: kanghg@snu.ac.kr |
Copyright © 2011 by The Korean Pediatric Society |
ABSTRACT |
Children who suffer from steroid-resistant nephrotic syndrome (SRNS)
require aggressive treatment to achieve remission. When intravenous
high-dose methylprednisolone fails, calcineurin inhibitors, such as
cyclosporine and tacrolimus, are used as the first line of treatment.
A significant number of patients with SRNS progress to end-stage
renal disease if remission is not achieved. For these children, renal
replacement therapy can also be problematic; peritoneal dialysis may
be accompanied by significant protein loss through the peritoneal
membrane, and kidney allograft transplantation may be complicated by
recurrence of SRNS. Plasmapheresis and rituximab were initially used
for treatment of recurrent SRNS after transplantation; these are now
under consideration as rescue therapies for refractory SRNS. Although
the prognosis of SRNS is complicated and unfavorable, intensive
treatment in the early stages of the disease may achieve remission in
more than half of the patients. Therefore, timely referral of pediatric
SRNS patients to pediatric nephrology specialists for histological and
genetic diagnosis and treatment is highly recommended. |
Keywords:
Nephrotic syndrome | Steroid-resistant | Treatment | Calcineurin inhibitor | Transplantation | Child
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