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Abstract: TH-PO427

Prognostic Model for Progression of Autosomal Dominant Polycystic Kidney Disease to Kidney Failure

Session Information

Category: Genetic Diseases of the Kidneys

  • 1201 Genetic Diseases of the Kidneys: Cystic

Authors

  • Yu, Alan S.L., University of Kansas Medical Center Department of Internal Medicine, Kansas City, Kansas, United States
  • Cohen, Aaron, Indiana University, Bloomington, Indiana, United States
  • Ables, Erin, Indiana University, Bloomington, Indiana, United States
  • Landsittel, Doug, Indiana University, Bloomington, Indiana, United States
  • Torres, Vicente E., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Chebib, Fouad T., Mayo Clinic in Florida, Jacksonville, Florida, United States
  • Chapman, Arlene B., University of Chicago Pritzker School of Medicine, Chicago, Illinois, United States
  • Harris, Peter C., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Mrug, Michal, The University of Alabama at Birmingham Department of Medicine, Birmingham, Alabama, United States
  • Rahbari-Oskoui, Frederic F., Emory University School of Medicine, Atlanta, Georgia, United States
  • Bae, Kyongtae Ty, The University of Hong Kong Li Ka Shing Faculty of Medicine, Hong Kong, Hong Kong
  • Dickinson, Stephanie, Indiana University, Bloomington, Indiana, United States
  • Bennett, William M., Legacy Health System, Portland, Oregon, United States
Background

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disease that leads to kidney failure (ESKD), typically in the fifth decade of life. Given the variability between individuals in the rate of progression to ESKD there is significant unmet need for an accurate prognostic model that can predict the course of disease early in individual patients. CRISP IV is the longest extant ADPKD cohort (21 years), providing a unique opportunity to determine the association between clinical observations early in the disease and hard clinical outcomes.

Methods

Data collected from participants in the CRISP and HALT-A studies between 2001 and 2022 were modeled using multivariable Cox proportional hazards. The outcome was a composite of kidney failure (dialysis or kidney transplant) and eGFR <15 mL/min/1.73 m2. Candidate dependent variables were picked on the basis of face validity, and selected for inclusion in the model by iterative forward selection.

Results

Of 759 included participants with a median follow-up of 8.2 years, 123 (16.2%) reached the composite endpoint. A prognostic model was built using 5 essential variables. Four clinical variables (serum CO2, hemoglobin, BMI, diastolic BP) were found to add independently to this model.

Conclusion

We have developed a prognostic model that can predict kidney failure in an ADPKD patient over a time horizon of 15 years. The next step is to validate this in an external ADPKD registry cohort and create a risk calculator.

Table. Multivariable model showing hazard ratios for the outcome of kidney failure or eGFR <15 mL/min/1.73 m2

Funding

  • NIDDK Support