title: Clinical characteristics and disease predictors of a large Chinese cohort of patients with autosomal dominant polycystic kidney disease.
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.
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Dongping
authors.initials: D
authors.lastname: Chen
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Yiyi
authors.initials: Y
authors.lastname: Ma
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Xueqi
authors.initials: X
authors.lastname: Wang
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Shengqiang
authors.initials: S
authors.lastname: Yu
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Lin
authors.initials: L
authors.lastname: Li
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Bing
authors.initials: B
authors.lastname: Dai
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Zhiguo
authors.initials: Z
authors.lastname: Mao
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Lijun
authors.initials: L
authors.lastname: Sun
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Chenggang
authors.initials: C
authors.lastname: Xu
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Shu
authors.initials: S
authors.lastname: Rong
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Mengjun
authors.initials: M
authors.lastname: Tang
authors.affiliation: Department of Radiology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Hongbo
authors.initials: H
authors.lastname: Zhao
authors.affiliation: Department of Radiology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Hongchao
authors.initials: H
authors.lastname: Liu
authors.affiliation: Division of Nephrology, University Hospital, Zürich, Switzerland.
authors.forename: Andreas L
authors.initials: AL
authors.lastname: Serra
authors.affiliation: Graf biostatistics, Winterthur, Switzerland.
authors.forename: Nicole
authors.initials: N
authors.lastname: Graf
authors.affiliation: Department of Radiology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Shiyuan
authors.initials: S
authors.lastname: Liu
authors.affiliation: Division of Nephrology, University Hospital, Zürich, Switzerland.
authors.forename: Rudolf P
authors.initials: RP
authors.lastname: Wüthrich
authors.affiliation: Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, P. R. China.
authors.forename: Changlin
authors.initials: C
authors.lastname: Mei
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D044466:Asian People; D001792:Blood Platelets / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002681:China; D015331:Cohort Studies; D003710:Demography; D018450:Disease Progression; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000503:physiopathology; D007677:Kidney Function Tests; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D009929:Organ Size; D016891:Polycystic Kidney, Autosomal Dominant / Q000175:diagnosis* / Q000473:pathology* / Q000503:physiopathology; D055815:Young Adult