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Kidney Week

Abstract: TH-PO853

Effect of Clinical, Radiological, and Genetic Factors on Progression to ESKD in Autosomal Dominant Polycystic Kidney Disease

Session Information

Category: Genetic Diseases of the Kidneys

  • 1001 Genetic Diseases of the Kidneys: Cystic

Authors

  • Shukoor, Shehbaz, Mayo Clinic, Rochester, Minnesota, United States
  • Edwards, Marie E., Mayo Clinic, Rochester, Minnesota, United States
  • Lavu, Sravanthi, Mayo Clinic, Rochester, Minnesota, United States
  • Kline, Timothy L., Mayo Clinic, Rochester, Minnesota, United States
  • Shrivastava, Sanskriti, Mayo Clinic, Rochester, Minnesota, United States
  • Zaatari, Ghaith, Mayo Clinic, Rochester, Minnesota, United States
  • Irazabal, Maria V., Mayo Clinic, Rochester, Minnesota, United States
  • Senum, Sarah R., Mayo Clinic, Rochester, Minnesota, United States
  • Harris, Peter C., Mayo Clinic, Rochester, Minnesota, United States
  • Hogan, Marie C., Mayo Clinic, Rochester, Minnesota, United States
  • Zoghby, Ziad, Mayo Clinic, Rochester, Minnesota, United States
  • Torres, Vicente E., Mayo Clinic, Rochester, Minnesota, United States
  • Chebib, Fouad T., Mayo Clinic, Rochester, Minnesota, United States
Background

Cystic expansion damaging the renal parenchyma is thought to lead to end-stage kidney disease (ESKD) in autosomal dominant polycystic kidney disease (ADPKD). Here we examined (1) whether factors independent of cystic growth contribute to disease progression and (2) their role in PKD1 compared to PKD2 associated disease.

Methods

We designed a cross-sectional study of ADPKD patients with ESKD seen at the Mayo Clinic between 1992 and 2018 with available abdominal imaging at or near ESKD (n=294). Clinical, laboratory, genetic, and radiological data at the time of ESKD were obtained from electronic medical records. Kidney volumes were measured and adjusted to height (HtTKV).

Results

Compared to females (n=154, 52%), males had similar mean age of ESKD (54.6 vs 54.9 years), larger HtTKV (2440 vs 1594 ml/m, p<0.01) and higher incidence of macrovascular disease (27% vs 17%, p=0.03). In the univariate analysis, age (p<0.01) and HDL cholesterol (p=0.02) were negatively associated whereas male sex (p<0.01) and ischemic heart disease (p=0.03) were positively associated with HtTKV at ESKD. In the multivariate analysis, only age, sex and ischemic heart disease were significantly associated with HtTKV at ESKD. ADPKD genotype was known in 182 patients (110 PKD1 T, 55 PKD1 NT, 17 PKD2). Age at ESKD was 49.8±9.4 years in patients with PKD1 T, 57.4±10.4 in those with PKD1 NT, and 65.2±10.3 in those with PKD2 mutations (p<0.01). HtTKV was 2129±1141, 1835±1331 and 2019±1162 ml/m in patients with PKD1T, PKD1NT and PKD2, respectively (p<0.02). A negative correlation between age and HtTKV at ESKD was observed in the PKD1 T group (r= -0.32, p<0.01) but not in the smaller and older PKD1 NT and PKD2 groups.

Conclusion

ADPKD patients who reach ESKD at an older age have smaller kidney volumes. This suggests that cyst burden is the main cause of GFR decline in patients reaching ESKD at younger age, whereas additional factors associated with aging (e.g. vascular remodeling) may contribute significantly to ESKD in older patients.