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Kidney Week

Abstract: SA-PO491

PROPKD Score Predicts Kidney Decline in Australian Patients with Autosomal Dominant Polycystic Kidney Disease in Clinical Practice

Session Information

  • ADPKD: Clinical Studies
    October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidney

  • 1001 Genetic Diseases of the Kidney: Cystic

Authors

  • Mallett, Andrew John, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
  • Chan, Samuel Shun-Kwan, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
  • Patel, Chirag, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
Background

The PROPKD score was recently developed from a single-country cohort study to enable risk stratification of progression to end-stage kidney disease in patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). The aim of this study was to validate whether the PROPKD score predicts kidney function decline in clinical practice amongst Australian ADPKD patients.

Methods

A cross-sectional study was conducted of 39 unrelated individuals with genetically confirmed ADPKD encountered by the Queensland Conjoint Renal Genetics Clinic Service between 1stJanuary 1994 to 31stMay, 2018. Clinical and genetic factors were examined using the PROPKD score, to assess its relationship with kidney function decline prediction over a three year period.

Results

Of the 39 individuals in this study, 15 patients were low risk, 17 were intermediate risk and 7 were high risk. The median age for the low risk group commencing renal replacement therapy (RRT) was 60 years, 54.5 years for the intermediate risk group, and 55.3 years for the high risk group. Of those who commenced RRT, 1 was high risk, 6 were intermediate risk and 1 was low risk. One high risk patient underwent pre-empt live transplant at 48 years. The median change in estimated glomerular filtration rate (CKD-EPI) in the low risk group was 1.5ml/min/1.73m2/yr, 12.5 ml/min/1.73m2/yr in the intermediate risk group and 15 ml/min/1.73m2/yr in the high risk group (p<0.001). There were no deaths from this cohort.

Conclusion

The PROPKD score accurately predicts kidney function decline in patients with ADPKD in this Australian cohort in clinical practice. This prediction tool may enable future personalised clinical prognostication and therapeutic management of ADPKD, as well as for potential participants to be identified for clinical trials.