总肾脏体积在评价常染色体显性遗传多囊肾病进展中的应用

Application of total renal volume in evaluating the progress of autosomal dominant polycystic kidney disease

目的通过核磁共振测量肾脏总体积(TKV)来观察与评价常染色体显性遗传多囊肾病(ADPKD)患者的疾病进展。方法纳入166例未进入终末期肾脏病的ADPKD患者,采集病史、家族史、体格检查、血尿常规及生化检测结果,并行腹部核磁共振(MRI)检查,对MRI扫描数据进行三维重建后精确测算TKV。对TKV与各项参数进行相关性分析,并通过TKV年增长率分析肾体积快速增长的原因。结果女性患者的TKV显著低于男性患者(P<0.05),且TKV年增长率略低于男性患者(P>0.05)。ADPKD患者的TKV随年龄的增长逐渐增加,与年龄呈正相关(P<0.01),TKV与肾小球滤过率(eGFR)呈显著负相关(P<0.01)。TKV缓慢增长型(TKV年增长率≤5%)和快速增长型(TKV年增长率>5%)相比,年龄、性别、体质量指数无明显差异(P>0.05);与缓慢增长型相比,快速增长型的基线TKV明显增高(P<0.01)。结论精确测量ADPKD患者的TKV可用于评估疾病的进展以及ADPKD潜在治疗方案的疗效。

Objective To observe and evaluate the progression of autosomal dominant polycystic kidney disease(ADPKD)by measuring the total kidney volume(TKV)with magnetic resonance imaging(MRI).Methods A total of 166 ADPKD patients without end-stage renal disease were enrolled in this study.The data on history,family history,physical examination,routine blood and urine examinations,biochemical examination and abdominal MRI were obtained.The TKV was accurately measured after three-dimensional reconstruction.The correlation between TKV and various parameters was analyzed,and the reason of rapid increase of renal volume was analyzed through the annual growth rate of TKV.Results The TKV of female patients was significantly smaller than that of male patients(P<0.05),and the annual growth rate of TKV was slightly lower than that of male patients(P>0.05).TKV in ADPKD patients was increased and positively correlated with age(P<0.01),but negatively with eGFR(P<0.01).There was no significant difference in age,sex and body mass index between slow growth TKV(TKV annual growth rate<5%)and fast growth TKV(TKV annual growth rate>5%)(P>0.05);The baseline TKV of the fast growth was significantly larger than that of the slow growth(P<0.01).Conclusions Accurate TKV measurements provide a measure of ADPKD progression and can be used to assess disease progression and the efficacy of potential treatments for ADPKD.