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.\udMETHODS: 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.\udRESULTS: 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).\udCONCLUSIONS: 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.
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机译:目的:常染色体显性遗传性多囊肾病(ADPKD)是一种持续发展的慢性肾脏病,无法治愈。这项研究的目的是鉴定中国ADPKD患者的特征,并确定预测囊肿生长和肾功能恶化的因素。\ ud方法:为了分析疾病预测因素,我们对541名中国ADPKD患者进行了一项前瞻性纵向观察性研究。 eGFR≥30 ml / min / 1.73 m(2)。在临床和放射学方面对患者进行连续腹部磁共振成像(MRI)随访。临床特征和实验室数据与估计的肾小球滤过率(eGFR)和总肾脏体积(TKV)的变化有关。建立了线性回归模型以分析决定eGFR和TKV变化的因素。\结果:该未选队列的年龄范围为4至77岁。中位随访时间为14.3±10.6个月。尽管eGFR和TKV的个体差异很大,但这两个参数之间存在一致的联系。基线log10转化的TKV和尿蛋白/肌酐比值被确定为eGFR下降较快且与TKV增长率较高相关的主要预测指标。有趣的是,较低的血小板计数与较低的eGFR(r = 0.222)和较高的TKV(r = 0.134)显着相关。在中国患者中,我们发现较低的血小板计数是疾病进展的重要预测指标。这些结果对于设计未来的临床试验以延缓多囊肾疾病的进展很重要。
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