Kidney Week

Abstract: FR-PO1171

Risk Stratification for Disease Recurrence in Children with Steroid Resistant Nephrotic Syndrome (SRNS) Following Kidney Transplantation (Midwest Pediatric Nephrology Consortium [MWPNC] Study)

Session Information

• Pediatric Nephrology - I
  October 26, 2018 | Location: Exhibit Hall, San Diego Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

• 1600 Pediatric Nephrology

Authors

• Gbadegesin, Rasheed A., Duke University Medical Center, Durham, North Carolina, United States

Rasheed A. Gbadegesin,

• Pelletier, Jonathan H., Duke University Hospital, Durham, North Carolina, United States

Jonathan H. Pelletier,

• Kumar, Karan, Duke University, Durham, North Carolina, United States

Karan Kumar,

• Engen, Rachel M., Seattle Children's Hospital, Seattle, Washington, United States

Rachel M. Engen,

• Bensimhon, Adam R., Duke, Chapel Hill, North Carolina, United States

Adam R. Bensimhon,

• Varner, Jennifer D., Duke University School of Medicine, Durham, North Carolina, United States

Jennifer D. Varner,

• Rheault, Michelle N., University of Minnesota, Minneapolis, Minnesota, United States

Michelle N. Rheault,

• Srivastava, Tarak, Childrens's Mercy Hospital, Kansas City, Missouri, United States

Tarak Srivastava,

• D'Alessandri-Silva, Cynthia J., Connecticut Children's Medical Center, Hartford, Connecticut, United States

Cynthia J. D'Alessandri-Silva,

• Davis, T. Keefe, Duke University Medical Center, Durham, North Carolina, United States

T. Keefe Davis,

• Wenderfer, Scott E., Baylor College of Medicine, Houston, Texas, United States

Scott E. Wenderfer,

• Gibson, Keisha L., University of North Carolina Kidney Center, Chapel Hill, North Carolina, United States

Keisha L. Gibson,

• Selewski, David T., Duke University Medical Center, Durham, North Carolina, United States

David T. Selewski,

• Barcia, John P., UVA Pediatric Nephrology, Charlottesville, Virginia, United States

John P. Barcia,

• Weng, Patricia L., UCLA, Los Angeles, California, United States

Patricia L. Weng,

• Licht, Christoph, The Hospital for Sick Children, Toronto, Ontario, Canada

Christoph Licht,

• Foreman, John W., Duke University Health System, Durham, North Carolina, United States

John W. Foreman,

• Wigfall, Delbert R., Duke University Medical Center, Durham, North Carolina, United States

Delbert R. Wigfall,

• Chua, Annabelle N., Duke University, Durham, North Carolina, United States

Annabelle N. Chua,

• Tsai, Eileen W., UCLA, Los Angeles, California, United States

Eileen W. Tsai,

• Hornik, Christoph, Duke University, Durham, North Carolina, United States

Christoph Hornik,

• Nagaraj, Shashi K., Duke University Medical Center, Durham, North Carolina, United States

Shashi K. Nagaraj,

• Greenbaum, Larry A., Emory University, Atlanta, Georgia, United States

Larry A. Greenbaum,

Background

Steroid resistant nephrotic syndrome (SRNS) due to FSGS and MCD is a leading cause of end stage kidney disease (ESKD) in children. Recurrence of primary disease following transplantation is a major cause of allograft loss. The clinical determinants of disease recurrence are not completely known. Our objective was to determine risk factors for recurrence of FSGS/MCD following kidney transplantation.

Methods

Multicenter study of pediatric patients with kidney transplants performed for ESKD due to SRNS between 1/2006-12/2015. Patients with primary-SRNS (PSRNS) were defined as those initially resistant to corticosteroid therapy at diagnosis, and patients with late-SRNS (LSRNS) as those initially responsive to steroids who subsequently developed steroid resistance. We performed logistic regression to determine risk factors associated with nephrotic syndrome (NS) recurrence.

Results

We included 158 eligible participants in our analysis; 64 (41%) had recurrence of NS in their renal allograft. Disease recurrence occurred in 78% of patients with LSRNS compared to 39% of those with PSRNS. Patients with MCD on initial native kidney biopsy had a 76% recurrence rate compared with a 40% recurrence rate in those with FSGS. The recurrence rate in patients with monogenic SRNS was 0%. Multivariable analysis showed that MCD histology (OR; 95% CI: 5.6; 1.3-23.7) compared to FSGS predicted disease recurrence.

Conclusion

Pediatric patients with MCD and LSRNS are at higher risk of disease recurrence following kidney transplantation. Based on our findings, it is possible to use genetic findings, native kidney biopsy findings and pattern of therapy response prior to kidney transplant to stratify patients with SRNS undergoing renal transplant into a low-risk group (monogenic NS), a medium-risk group (FSGS on native kidney biopsy and PSRNS), and a high-risk group (MCD on native kidney biopsy and/or LSRNS).

Funding

• NIDDK Support