SUBCLINICAL TARGET ORGAN DAMAGE IN A SAMPLE OF CHILDREN WITH AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

Abstract

Objective: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common monogenic hereditary kidney disease, characterized by the development of cysts along the renal tubule. Its prevalence is estimated at 1 per 1000 population. Previous studies suggest that hypertension and vascular damage can start during childhood. The aim of this study is the evaluation of markers of vascular damage and left ventricular geometry in a sample of children with ADPKD. Design and method: Several vascular measurements were obtained: Ambulatory Blood Pressure Monitoring (ABPM), carotid Intima Media Thickness (cIMT), Carotid Distensibility (CD), Pulse Wave Velocity (PWV) and Echocardiographic measurements: Relative Wall Thickness (RWT) and Left Ventricular Mass Index (LVMI). Results: 11 children with ADPKD were recruited (4 females and 7 males, mean age 9.5 ± 3.2 years): 3 children were overweight, 8 were normal weight. Four children resulted hypertensive at the ABPM, 5 were normotensive, 2 ABPM were not available. One child was already on therapy with an ACE-inhibitor. RWT was tendentially high (mean 0.39 ± 0.47) while cIMT was above the 95°percentile for sex and height in 80% of children (0.5 ± 0.05 mm). Average PWV and CD were between the normal range (4.6 ± 0.64 m/sec and 57.1 ± 16.1 x10–3/KPa respectively). We observed a positive correlation between the PWV and RWT (r = 0.616; p = 0.044) and a negative correlation between CD and RWT (r = −0.770; p = 0.015). None of the subjects have altered Glomerular Filtration Rate (GFR). Conclusions: Increased RWT and high cIMT, indicating subclinical organ damage, are present in ADPKD children. The level of RWT was significantly correlated to that of CD and PWV, implying that vascular stiffening is associated with cardiac remodelling. None of the children has an alteration of renal function, suggesting that subclinincal cardiovascular alterations may precede the overt decline in Glomerular Filtration Rate. These data underline the importance of a comprehensive cardiovascular screening in all the children with ADPKD.