Clinical characteristics and disease predictors of a large Chinese cohort of patients with autosomal dominant polycystic kidney disease

PLoS One. 2014 Mar 20;9(3):e92232. doi: 10.1371/journal.pone.0092232. eCollection 2014.

Abstract

Objective: Autosomal dominant polycystic kidney disease (ADPKD) is a relentlessly progressing form of chronic kidney disease for which there is no cure. The aim of this study was to characterize Chinese patients with ADPKD and to identify the factors which predict cyst growth and renal functional deterioration.

Methods: To analyze disease predicting factors we performed a prospective longitudinal observational study in a cohort of 541 Chinese patients with ADPKD and an eGFR ≥ 30 ml/min/1.73 m(2). Patients were followed clinically and radiologically with sequential abdominal magnetic resonance imaging (MRI). Clinical characteristics and laboratory data were related to changes in estimated glomerular filtration rate (eGFR) and total kidney volume (TKV). A linear regression model was developed to analyze the factors which determine eGFR and TKV changes.

Results: The age range of this unselected cohort ranged from 4 to 77 years. Median follow-up time was 14.3 ± 10.6 months. Although inter-individual differences in eGFR and TKV were large, there was a consistent link between these two parameters. Baseline log10-transformed TKV and urinary protein/creatinine ratio were identified as the major predictors for a faster eGFR decline and were associated with a higher TKV growth rate. Interestingly, a lower thrombocyte count correlated significantly with lower eGFR (r = 0.222) and higher TKV (r = 0.134).

Conclusions: This large cohort of Chinese patients with ADPKD provides unique epidemiological data for comparison with other cohorts of different ethnicity. In Chinese patients we identified a lower thrombocyte count as a significant predictor of disease progression. These results are important for the design of future clinical trials to retard polycystic kidney disease progression.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Asian People
  • Blood Platelets / pathology
  • Child
  • Child, Preschool
  • China
  • Cohort Studies
  • Demography
  • Disease Progression
  • Female
  • Glomerular Filtration Rate
  • Humans
  • Kidney / pathology
  • Kidney / physiopathology
  • Kidney Function Tests
  • Linear Models
  • Male
  • Middle Aged
  • Organ Size
  • Polycystic Kidney, Autosomal Dominant / diagnosis*
  • Polycystic Kidney, Autosomal Dominant / pathology*
  • Polycystic Kidney, Autosomal Dominant / physiopathology
  • Young Adult

Grants and funding

This work was supported by an Institutional Partnership Grant from the Sino Swiss Science & Technology Cooperation (SSSTC) Program, National High Technology Reseach Development (863) Project of China (2007AA02Z3Z1), National Natural Science Foundation of China (30570867, 30771016, 30971368), National Natural Science Foundation of China for Young Scientists (81000281, 81100482), Shanghai Key Clinical Disciplines Construction Project, Key Scientific and Technological Project of Shanghai Committee of Science and Technology (11431920800). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.