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Abstract: PO2357

Clinical and Biopsy Characteristics in a Pediatric Cohort of C3 Glomerulopathy (C3G) and Immune Complex Membranoproliferative Glomerulonephritis (IC-MPGN)

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Dixon, Bradley P., Renal Section, Department of Pediatrics, University of Colorado School of Medicine, Aurora, Colorado, United States
  • Goodwin Davies, Amy, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Razzaghi, Hanieh, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Meloni, Sherin, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Thomas, Melissa E., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Flynn, Joseph T., Seattle Children's Hospital, Seattle, Washington, United States
  • Claes, Donna J., Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Mitsnefes, Mark, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
  • Stotter, Brian Ross, Washington University in Saint Louis School of Medicine, Saint Louis, Missouri, United States
  • Dharnidharka, Vikas R., Washington University in Saint Louis School of Medicine, Saint Louis, Missouri, United States
  • Gluck, Caroline A., Nemours/AI Dupont Hospital for Children, Wilmington, Delaware, United States
  • Zaritsky, Joshua, Nemours/AI Dupont Hospital for Children, Wilmington, Delaware, United States
  • Somers, Michael J., Boston Children's Hospital, Boston, Massachusetts, United States
  • Kallash, Mahmoud, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Furth, Susan L., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Forrest, Christopher B., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Laskin, Benjamin L., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Denburg, Michelle, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
Background

C3G and IC-MPGN are rare diseases. The ability to identify and phenotype children with C3G and IC-MPGN using electronic health records (EHR) would aid description of natural history and prognosticate therapeutic response.

Methods

Using a computable phenotype algorithm, a pediatric cohort of children with glomerular disorders was identified in PEDSnet, a national network of pediatric health systems with aggregated EHR data, and refined using MPGN-specific SNOMED-CT codes to identify C3G and IC-MPGN patients at 6 centers. Discrete data elements were captured from electronic health records, and additional clinical data were extracted by standardized chart review. Biopsy diagnosis was classified as C3G or IC-MPGN by applying an automated algorithm to immunofluorescence data.

Results

Of 285 identified patients, 173 were true cases of C3G or IC-MPGN (Tables 1 and 2). Median C3 level at diagnosis was lower in C3G compared to IC-MPGN (p=0.005). There were no significant differences in light microscopic injury pattern or ultrastructure between C3G and IC-MPGN biopsies, but C3 intensity was higher in C3G compared to IC-MPGN (p = 0.006) (Table 3).

Conclusion

Patients with C3G and IC-MPGN can be identified and characterized by the use of a computable phenotype, allowing the creation of robust databases to define clinical predictors of treatment response. This may prove to be a vital asset for recruitment into clinical trials of complement-targeted agents likely beneficial to this patient population.

Funding

  • NIDDK Support