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Published in: BMC Nephrology 1/2024

Open Access 01-12-2024 | Chronic Kidney Disease | Research

Mortality risk in patients with autosomal dominant polycystic kidney disease

Authors: Deirdre Mladsi, Xiaolei Zhou, Gregory Mader, Myrlene Sanon, Jinyi Wang, Christine Barnett, Cynthia Willey, Stephen Seliger

Published in: BMC Nephrology | Issue 1/2024

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Abstract

Background

Autosomal dominant polycystic kidney disease (ADPKD) is the leading inheritable cause of end-stage renal disease (ESRD). Mortality data specific to patients with ADPKD is currently lacking; thus, the aim of this study was to estimate mortality in patients with ADPKD.

Methods

We analyzed data from the United States Renal Data System (USRDS) for patients with ADPKD available during the study period of 01/01/2014–12/31/2016, which included a cohort of patients with non-ESRD chronic kidney disease (CKD) and a cohort of patients with ESRD. Mortality rates with 95% confidence intervals (CIs) were calculated overall and by age group, sex, and race for the full dataset and for a subset of patients aged ≥ 65 years. Adjusted mortality hazard ratios (HRs) were calculated using Cox regression modeling by age group, sex, race, and CKD stage (i.e., non-ESRD CKD stages 1–5) or ESRD treatment (i.e., dialysis and transplant).

Results

A total of 1,936 patients with ADPKD and non-ESRD CKD and 37,461 patients with ADPKD and ESRD were included in the analysis. Age-adjusted mortality was 18.4 deaths per 1,000 patient-years in the non-ESRD CKD cohort and 37.4 deaths per 1,000 patient-years in the ESRD cohort. As expected, among the non-ESRD CKD cohort, patients in CKD stages 4 and 5 had a higher risk of death than patients in stage 3 (HR = 1.59 for stage 4 and HR = 2.71 for stage 5). Among the ESRD cohort, patients receiving dialysis were more likely to experience death than patients who received transplant (HR = 2.36). Age-adjusted mortality among patients aged ≥ 65 years in the non-ESRD CKD cohort was highest for Black patients (82.7 deaths per 1,000 patient-years), whereas age-adjusted mortality among patients aged ≥ 65 years in the ESRD cohort was highest for White patients (136.1 deaths per 1,000 patient-years).

Conclusions

Mortality rates specific to patients aged ≥ 65 years suggest racial differences in mortality among these patients in both non-ESRD CKD and ESRD cohorts. These data fill an important knowledge gap in mortality estimates for patients with ADPKD in the United States.
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Metadata
Title
Mortality risk in patients with autosomal dominant polycystic kidney disease
Authors
Deirdre Mladsi
Xiaolei Zhou
Gregory Mader
Myrlene Sanon
Jinyi Wang
Christine Barnett
Cynthia Willey
Stephen Seliger
Publication date
01-12-2024
Publisher
BioMed Central
Published in
BMC Nephrology / Issue 1/2024
Electronic ISSN: 1471-2369
DOI
https://doi.org/10.1186/s12882-024-03484-3

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