Handa S Paul
Department of Nephrology, Atlantic Health Sciences Corporation, Saint John, New Brunswick, Canada.
Clin Invest Med. 2006 Dec;29(6):339-46.
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common hereditary renal disorder. In addition to renal structure and function abnormalities, the cardiovascular changes (extra renal manifestations) are the frequent findings observed in these subjects. This pilot study describes the viscoelastic properties of the aorta, a predisposing factor for the genesis of hypertension and left ventricular hypertrophy in young adults diagnosed with ADPKD.
Twenty new patients with ADPKD, all the off springs of previously diagnosed patients with ADPKD were recruited to participate in the study. Each patient underwent the measurement of 24-hour creatinine clearance (Ccr), 24-hour Ambulatory Blood Pressure Monitoring (ABPM), and ultrasonographic determination of the aortic dimensions and left ventricular measurements by M-mode echocardiogram and 12-lead electrocardiogram (ECG) in the echo laboratory.
The mean age of our ADPKD subjects was 29.9 +/- 6.5 yr. Five patients had impaired renal functions (Ccr < 1.48 ml/sec). Fourteen patients had hypertension and nine were considered Non-dippers on ABPM. The median value of LVMI and AOD was 84.9 g/m2 and 28.5 x 10(-3)/kPa respectively. Forty five percent of these subjects had non-dipping circadian pattern with smaller nocturnal BP reduction. No relationship between AOD, LVMI and 24-hr ABPM was observed; however, a positive trend towards Ccr and AOD was evident.
The majority of our ADPKD subjects have an unfavourable cardiovascular risk profile: hypertension and a non-dipping circadian BP rhythm. Subjects with evidence of renal function impairment had reduced aortic distensibility, placing them at an increased risk for cardiovascular morbidity and mortality.
常染色体显性多囊肾病(ADPKD)是最常见的遗传性肾脏疾病。除了肾脏结构和功能异常外,心血管变化(肾外表现)是这些患者常见的发现。这项初步研究描述了主动脉的粘弹性特性,这是诊断为ADPKD的年轻成年人发生高血压和左心室肥厚的一个易感因素。
招募了20名新诊断的ADPKD患者,他们均为先前诊断为ADPKD患者的后代,参与本研究。每位患者在超声心动图实验室进行了24小时肌酐清除率(Ccr)测量、24小时动态血压监测(ABPM),并通过M型超声心动图和12导联心电图(ECG)进行主动脉尺寸和左心室测量的超声检查。
我们的ADPKD患者平均年龄为29.9±6.5岁。5名患者肾功能受损(Ccr<1.48 ml/秒)。14名患者患有高血压,9名在ABPM中被认为是非勺型血压者。左心室质量指数(LVMI)和主动脉外径(AOD)的中位数分别为84.9 g/m²和28.5×10⁻³/kPa。这些患者中有45%的昼夜节律模式为非勺型,夜间血压下降较小。未观察到AOD、LVMI与24小时ABPM之间的关系;然而,Ccr与AOD之间存在明显的正相关趋势。
我们的大多数ADPKD患者具有不良的心血管风险特征:高血压和非勺型昼夜血压节律。有肾功能损害证据的患者主动脉扩张性降低,使他们面临心血管疾病发病率和死亡率增加的风险。