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Kidney Week

Abstract: PO1971

Clinical Characteristics of Recurrent Focal Segmental Glomerulosclerosis (rFSGS) After Kidney Transplant (KTx) Through Computable Phenotypic Algorithm Analyses of Multicenter Data

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Dharnidharka, Vikas R., Washington University in St Louis, St Louis, Missouri, United States
  • Scobell, Rebecca R., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Kallash, Mahmoud, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Goodwin Davies, Amy, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Marchesani, Nicole, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Maltenfort, Mitchell, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Walther, Leslie, Washington University in St Louis, St Louis, Missouri, United States
  • Kelton, Megan, Seattle Children's Hospital, Seattle, Washington, United States
  • Bock, Margret, Children's Hospital Colorado, Aurora, Colorado, United States
  • Blanchette, Eliza, University of Colorado, Denver, Colorado, United States
  • Stone, Hillarey, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Gluck, Caroline A., Nemours Biomedical Research Center for Pharmacogenomics and Translational Research, Wilmington, Delaware, United States
  • Hullekes, Frank E., Massachusetts General Hospital, Boston, Massachusetts, United States
  • Riella, Leonardo V., Massachusetts General Hospital, Boston, Massachusetts, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Mitsnefes, Mark, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Dixon, Bradley P., Children's Hospital Colorado, Aurora, Colorado, United States
  • Flynn, Joseph T., Seattle Children's Hospital, Seattle, Washington, United States
  • Somers, Michael J., Boston Children's Hospital, Boston, Massachusetts, United States
  • Forrest, Christopher B., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Furth, Susan L., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Denburg, Michelle, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
Background

Primary FSGS, a glomerular disease, has a high rate of progression to end stage kidney failure and varying rates of recurrence after KTx. Treatment effects are hard to determine, requiring both large multicenter populations and granular site level data.

Methods

Using the PEDSnet research network of >11 million records, we refined a published computational phenotype (Denburg et al 2019) for a pediatric nephrotic glomerular disorders cohort to identify patients with evidence of KTx. Standardized chart review was used to identify patients with FSGS and rFSGS (urine protein/creatinine ratio > 2.0 mg/mg post-KTx).

Results

In PEDSnet v4.0 data from 1/2009-11/2020 across 6 centers, 4380 patients met criteria for glomerular disorders and 1994 among those were identified as nephrotic. 220 had evidence for KTx. In charts reviewed to-date for these 220 patients, 89/133 had non-genetic FSGS, and 71 received a KTx after 2009. rFSGS was identified in 29/71 patients, mostly early after KTx (Fig. Panel A). Demographic characteristics of those with FSGS (n=89) and rFSGS (n=29) are shown in the Table. After rFSGS, plasmapheresis (n=26) or rituximab (n=24) were the most common treatments used, remission was complete in 14/29 (48%), and partial in another 6. Allograft loss occurred in 7 patients, not significantly worse than in those without recurrence (Fig. Panel B).

Conclusion

PEDSnet can identify and characterize patients with rare diseases such as rFSGS to create robust databases to compare clinical efficacy of treatments, and for recruitment into clinical trials.