Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium.

Creator(s)

Caroline Straatmann
Rose Ayoob
Rasheed Gbadegesin
Keisha Gibson
Michelle N. Rheault
Tarak Srivastava, Children's Mercy HospitalFollow
Cheryl L. Tran
Debbie S. Gipson
Larry A. Greenbaum
William E. Smoyer
V Matti Vehaskari

Document Type

Article

Publication Date

8-1-2013

Identifier

DOI: 10.1007/s00467-013-2483-y

Abstract

Background: Idiopathic nephrotic syndrome (NS) in children is classified as steroid sensitive or steroid resistant. Steroid sensitivity typically portends a low risk of permanent renal failure. However, some initially steroid-sensitive patients later develop steroid resistance. These patients with late steroid resistance (LSR) are often treated with immunosuppressant medications, but the effect of these additional drugs on the long-term prognosis of LSR is still unknown.

Methods: A retrospective chart review was performed on patients diagnosed with idiopathic NS and subsequent LSR during the 8-year study period from 2002 up to and including 2009, with a minimum of 2 years of follow-up. Primary outcome measures were proteinuria and renal function.

Results: A total of 29 patients were classified as having LSRNS. The majority of patients received treatment with calcineurin inhibitors and/or mycophenolate mofetil. Seven patients received three or more non-steroid immunosuppressant medications. Sustained complete or partial remission was achieved in 69 % of patients. Three developed end-stage renal disease, and all others maintained normal renal function. There were 13 episodes of serious adverse events, none of which were fatal or irreversible.

Conclusion: Most patients with LSRNS responded to immunosuppressive therapy by reduction or resolution of proteinuria and preservation of renal function. The results suggest that immunosuppressive treatment is a viable option in NS patients who develop LSR.

Journal Title

Pediatric nephrology (Berlin, Germany)

Volume

28

Issue

8

First Page

1235

Last Page

1241

MeSH Keywords

Analysis of Variance; Child; Child, Preschool; Disease Progression; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Infant; Kidney; Kidney Failure, Chronic; Male; Midwestern United States; Nephrotic Syndrome; Proteinuria; Remission Induction; Retrospective Studies; Time Factors; Treatment Outcome

Comments

Comment in

• Late steroid resistance in childhood nephrotic syndrome: do we now know more than 40 years ago? [Pediatr Nephrol. 2013]

Recommended Citation

Straatmann, C., Ayoob, R., Gbadegesin, R., Gibson, K., Rheault, M. N., Srivastava, T., Tran, C. L., Gipson, D. S., Greenbaum, L. A., Smoyer, W. E., Vehaskari, V. M. Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium. Pediatric nephrology (Berlin, Germany) 28, 1235-1241 (2013).