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Abstract: TH-PO826

Expanded Imaging Classification of Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Session Information

Category: Genetic Diseases of the Kidneys

  • 1001 Genetic Diseases of the Kidneys: Cystic

Authors

  • Bae, Kyongtae Ty, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
  • Shi, Tiange, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
  • Tao, Cheng, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
  • Yu, Alan S.L., University of Kansas Medical Center, Kansas City, Kansas, United States
  • Torres, Vicente E., Mayo Clinic, Rochester, Minnesota, United States
  • Chapman, Arlene B., University of Chicago, Chicago, Illinois, United States
  • Perrone, Ronald D., Tufts Medical Center, Boston, Massachusetts, United States
  • Brosnahan, Godela M., University of Colorado Denver, Aurora, Colorado, United States
  • Steinman, Theodore I., Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Braun, William E., Cleveland Clinic, Cleveland, Ohio, United States
  • Abebe, Kaleab, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
  • Harris, Peter C., Mayo Clinic, Rochester, Minnesota, United States
  • Landsittel, Doug, University of Pittsburgh, Pittsburgh, Pennsylvania, United States

Group or Team Name

  • HALT PKD Consortium
Background

Mayo imaging classification of ADPKD is a widely accepted tool that uses height-adjusted total kidney volume (htTKV) and age to identify patients at highest risk for disease progression. The current Mayo classification, however, is applicable only to patients who have typical disease with diffuse cystic involvement (Class 1) by excluding 5-10% of patients (Class 2) with atypical kidney morphology whose htTKV do not appear to predict eGFR decline. Thus, for Class 2 patients, predicting the risk for progression remains uncertain.

Methods

Twenty-one patients of Class 2 with predominant exophytic cyst distribution were identified from the HALT-A study (558 ADPKD adults), and their htTKV were remeasured by excluding exophytic cysts to estimate revised htTKV (rev-htTKV). For the analysis, the odds ratio of reaching CKD stage 3 (CKD3) per 100 ml/m increment in htTKV were compared in both unadjusted and adjusted (covariates: baseline age, eGFR, BMI, sex and race) logistic models for (1) only Class 1 participants, (2) all participants with original htTKV and (3) all participants with rev-htTKV.

Results

All 6 logistic models showed significant association (p<0.001) between baseline htTKV and reaching CKD3. Estimated odds ratios of reaching CKD3 for all participants increased from use of htTKV to rev-htTKV for both unadjusted (from 1.26 to 1.31) and adjusted (from 1.18 to 1.26) models. Because rev-htTKV was always less than htTKV, the probability of reaching CKD3 decreased for all Class 2 participants. Furthermore, with rev-htTKV, the probability of outcome for Class 2 participants who did not reach CKD3 decreased more than those who reached CKD3.

Conclusion

For Class 2 with predominant exophytic cyst distribution, the association between baseline htTKV and CKD3 outcome became stronger with the use of htTKV remeasured after excluding exophytic cysts, compared to the use of original htTKV.

Comparison of odd ratios of reaching CKD3 with and without including class 2 in two different models
Outcome: CKD3, eGFR<60 Unadjusted modelAdjusted model
ModelsNOR95% CIPOR95% CIP
Excluding Class 24331.291.21-1.38<0.0011.231.14-1.33<0.001
Including Class 2, original htTKV4511.261.18-1.34<0.0011.181.10-1.27<0.001
Including Class 2, rev-htTKV4511.311.22-1.40<0.0011.261.16-1.36<0.001

Funding

  • NIDDK Support