Initial Steroid Sensitivity in Children with Steroid-Resistant Nephrotic Syndrome Predicts Post-Transplant Recurrence
- 1 June 2014
- journal article
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of the American Society of Nephrology
- Vol. 25 (6), 1342-1348
- https://doi.org/10.1681/asn.2013080852
Abstract
Of children with idiopathic nephrotic syndrome, 10%-20% fail to respond to steroids or develop secondary steroid resistance (termed initial steroid sensitivity) and the majority progress to transplantation. Although 30%-50% of these patients suffer disease recurrence after transplantation, with poor long-term outcome, no reliable indicator of recurrence has yet been identified. Notably, the incidence of recurrence after transplantation appears reduced in patients with steroid-resistant nephrotic syndrome (SRNS) due to monogenic disorders. We reviewed 150 transplanted patients with SRNS to identify biomarkers that consistently predict outcome of SRNS after transplantation. In all, 25 children had genetic or familial SRNS and did not experience post-transplant recurrence. We reviewed phenotypic factors, including initial steroid sensitivity, donor type, age, ethnicity, time to ESRD, and time on dialysis, in the remaining 125 children. Of these patients, 57 (45.6%) developed post-transplant recurrence; 26 of 28 (92.9%) patients with initial steroid sensitivity recurred after transplantation, whereas only 26 of 86 (30.2%) patients resistant from the outset recurred (odds ratio, 30; 95% confidence interval, 6.62 to 135.86; P<0.001). We were unable to determine recurrence in two patients (one with initial steroid sensitivity), and nine patients did not receive initial steroids. Our data show that initial steroid sensitivity is highly predictive of post-transplant disease recurrence in this pediatric patient population. Because a pathogenic circulating permeability factor in nephrotic syndrome remains to be confirmed, we propose initial steroid sensitivity as a surrogate marker for post-transplant recurrence.Keywords
This publication has 32 references indexed in Scilit:
- Active proteases in nephrotic plasma lead to a podocin‐dependent phosphorylation of VASP in podocytes via protease activated receptor‐1The Journal of Pathology, 2012
- Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical EntityInternational Journal of Nephrology, 2012
- Worldwide Distribution of the MYH9 Kidney Disease Susceptibility Alleles and Haplotypes: Evidence of Historical Selection in AfricaPLOS ONE, 2010
- Potential Donor–Recipient MYH9 Genotype Interactions in Posttransplant Nephrotic Syndrome After Pediatric Kidney TransplantationAmerican Journal of Transplantation, 2009
- Successful Anti-TNFα Treatment in a Child with Posttransplant Recurrent Focal Segmental GlomerulosclerosisAmerican Journal of Transplantation, 2009
- Demographics and response to therapeutic plasma exchange in pediatric renal transplantation for focal glomerulosclerosis: A single center experiencePediatric Transplantation, 2008
- Plasma Exchange and Retransplantation in Recurrent Nephrosis of Patients With Congenital Nephrotic Syndrome of the Finnish Type (NPHS1)Transplantation, 2007
- Nephrotic Plasma Alters Slit Diaphragm–Dependent Signaling and Translocates Nephrin, Podocin, and CD2 Associated Protein in Cultured Human PodocytesJournal of the American Society of Nephrology, 2005
- Early recurrent nephrotic syndrome after renal transplantation in children with focal segmental glomerulosclerosisNephrology Dialysis Transplantation, 2000
- PATHOGENESIS OF LIPOID NEPHROSIS: A DISORDER OF T-CELL FUNCTIONThe Lancet, 1974