Blood pressure and renal function in autosomal dominant polycystic kidney disease (original) (raw)
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The Journal of Pediatric Academy
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary renal cystic diseases. Althoughits clinical manifestations usually occur in adulthood, hypertension (HT) is known to develop in most patients before the decline inrenal function and it is associated with faster progression to end stage kidney disease (ESKD). We investigated ambulatory bloodpressure monitoring (ABPM) results of 23 patients with ADPKD, followed up in the Pediatric Nephrology Clinic of Ondokuz MayısUniversity Medical Faculty Hospital. Patients’ demographic characteristics, laboratory and ultrasonography (US) results, officeblood pressure, and ABPM measurements were evaluated. The parameters of gender, age, increased kidney size, proteinuria,glomerular filtration rate (GFR) was compared in hypertensive and non-hypertensive group. Twenty three patients (13 girls, tenboys) with a mean age of 11.94±4.01 (min-max: 4.6-18) years and a female/male ratio of 1.3/1 were examined. Ultrasound...
Frontiers in Molecular Biosciences
Autosomal dominant (ADPKD) and autosomal recessive (ARPKD) polycystic kidney disease are the most widely known cystic kidney diseases. They are significantly different from each other in terms of genetics and clinical manifestations. Hypertension is one of the main symptoms in both diseases, but the age of onset and secondary cardiovascular complications are significantly different. Most ARPKD children are hypertensive in the first year of life and need high doses of hypertensive drugs. ADPKD patients with a very early onset of the disease (VEOADPKD) develop hypertension similarly to patients with ARPKD. Conversely, a significantly lower percentage of patients with classic forms of ADPKD develops hypertension during childhood, although probably more than originally thought. Data published in the past decades show that about 20%–30% of ADPKD children are hypertensive. Development of hypertension before 35 years of age is a known risk factor for more severe disease in adulthood. The c...
Ambulatory blood pressure in hypertensive patients with autosomal dominant polycystic kidney disease
Nephrology Dialysis Transplantation, 1997
It accelerates the rate of progression of renal failure [1,2], and may play a role in the pathogenesis of Background. Ambulatory blood pressure is more closely correlated with various indices of hypertensive cerebral haemorrhage and aneurysmal subarachnoid haemorrhage in ADPKD [3]. It may also be a contrib-target-organ damage, and is a better prognostic predictor of cardiovascular morbidity and mortality than utory factor to the increased incidence of cardiovascular deaths reported in affected patients [2]. conventional methods of blood pressure measurement. Autosomal dominant polycystic kidney disease There is little information on ambulatory blood pressure in these patients. The mean blood pressure (ADPKD) is complicated by hypertension, progressive renal failure, and an increased risk of cardiovascular values derived from ambulatory blood pressure measurements made over 24-h, or during some specific time mortality. This study investigated the 24-h ambulatory blood pressure profile in patients with ADPKD in periods thereof, are more reproducible than clinic blood pressures [4 ]. They are better at predicting view of the sparsity of such data in these patients and the possibility that abnormal diurnal blood pressure cardiovascular morbidity and mortality [5,6 ], and are more closely correlated with various indices of hyper-variations may have prognostic consequences. Methods. Ambulatory blood pressure was measured tensive target-organ damage, including left ventricular hypertrophy [7-9], vascular abnormalities in the retina over a 24-h period by the oscillometric method with an automatic non-invasive recorder (SpaceLabs 90207 [10,11] and urinary markers of renal damage [12 ], than conventional methods of blood pressure measure-system) in matched groups of 25 hypertensive patients with ADPKD and 25 patients with essential hyper-ment. An inadequate lowering of blood pressure during sleeping hours may be an important determinant of tension. Results. Both groups showed a nocturnal decrease in left ventricular hypertrophy [8,9], and there is evidence that the absence of, or a reduction in, the normal blood pressure, but this was significantly smaller in patients with ADPKD. There was no evidence of decline in nocturnal blood pressure (so-called nondippers) in a small minority of patients with essential enhanced lability of blood pressure in ADPKD. Conclusions. The nocturnal fall in blood pressure was hypertension is associated with a higher frequency of stroke [13 ]. attenuated in patients with ADPKD. Further studies are required to assess the importance of this finding This study was designed to investigate the 24-h ambulatory blood pressure profiles of hypertensive and its possible contribution to the progression of renal failure or increased cardiovascular mortality in patients with ADPKD in view of the lack of such data in these patients and the possibility that abnormal these patients. diurnal blood pressure variations may have prognostic consequences. Comparisons were made with patients Key words: ambulatory blood pressure; autosomal dominant polycystic kidney disease; hypertension with essential hypertension. Medical Renal Unit, Royal Infirmary of Edinburgh, gave their informed consent to take part in this study which was
Progress in blood pressure control in autosomal dominant polycystic kidney disease
American Journal of Kidney Diseases, 2000
Hypertension occurs commonly in autosomal dominant polycystic kidney disease (ADPKD) and is an important factor in the progression of the disease and cardiovascular mortality. The aim of this prospective 15-year study is to report the rate of blood pressure control and the potential effect of a 10-point education program developed by our center for ADPKD patients and their physicians. The patients' blood pressure treatment was managed by their primary care physicians. Three 5-year periods were analyzed in which similar rates of hypertension in patients with ADPKD were present (63% to 68%). In the first period (1985 to 1989), the rate of blood pressure control (<140/90 mm Hg) was 38% for 216 hypertensive patients with ADPKD. From 1990 to 1994, the percentage of blood pressure control increased to 55% in 194 hypertensive patients with ADPKD (P < 0.001 versus 1985 to 1989); and the level of blood pressure control increased to 64% in 181 hypertensive patients with ADPKD during 1995 to 1999 (P < 0.001 versus 1985 to 1989). Although this percentage of blood pressure control in patients with ADPKD remains suboptimal, it compares very favorably with the 27% estimated blood pressure control in patients with essential hypertension from 1991 to 1994 in the United States.
Hypertension in Autosomal Dominant Polycystic Kidney Disease
2010
Hypertension in autosomal dominant polycystic kidney disease. Autosomal dominant polycystic kidney disease (ADPKD) has been shown to be associated with a greater than 50 percent incidence of hypertension prior to deterioration in renal function as assessed by glomerular filtration rate. The present study provides evidence for increased cardiac pre-load, as assessed by plasma atrial natriuretic factor (ANF) and cardiac index, in hypertensive as compared to normotensive ADPKD. The hypertensive ADPKD patients exhibited an increased renal vascular resistance as compared to the normotensive patients in spite of comparable glomerular filtration rates. It is hypothesized that the renal involvement of hypertensive ADPKD patients causes an impaired renal response to the observed increase in cardiac index, and also may release a venoconstrictor (such as angiotensin) which contributes to the enhanced cardiac pre-load and thus the hypertension.
Physiological research / Academia Scientiarum Bohemoslovaca, 2004
Impaired glomerular filtration rate (GFR) is a risk factor for the development of hypertension in patients with autosomal dominant polycystic kidney disease (ADPKD). However, markers of tubular function were not tested whether they are linked to hypertension or blood pressure (BP) level. The aim of our study was to investigate the relationship between renal concentrating capacity and BP in children with ADPKD. Fifty-three children (mean age 11.8+/-4.4 years) were investigated. Standardized renal concentrating capacity test was performed after nasal drop application of desmopressin, BP was measured by ambulatory BP monitoring (ABPM). Renal concentrating capacity was decreased in 58 % of children. The prevalence of hypertension was significantly higher in children with decreased renal concentrating capacity (35 %) than in children with normal renal concentrating capacity (5 %) (p<0.05). Significant negative correlations were found between renal concentrating capacity, ambulatory BP...
Blood Pressure Monitoring, 2003
Objective In adult patients with autosomal dominant polycystic kidney disease (ADPKD) renal volume was found to be significantly greater in hypertensive compared to normotensive patients. The purpose of this study was to find out if blood pressure (BP) is related to renal size also in children with ADPKD, for example, in an early stage of the disease. Method and results Sixty-two children with ADPKD and normal renal function (mean age 12.3 ± 4.3 years) were examined by renal ultrasound and ambulatory BP monitoring (ABPM). Twenty-two children were hypertensive and 40 normotensive. Mean renal volume was significantly greater in hypertensive than in normotensive children (2.7 ± 2.3 SDS versus 1.2 ± 2.5 SDS, P < 0.01) despite similar anthropometric data and renal function. Similarly the mean number of cysts was significantly higher in hypertensive patients than in normotensive (35 ± 15 cysts versus 23 ± 14 cysts, P < 0.01). Renal volume correlated with daytime as well as with night-time systolic and diastolic BP (r = 0.41-0.47, P < 0.01). Correlations with renal length and the number of renal cysts were somewhat less (r = 0.29-0.43, P < 0.05 and 0.01, respectively). Conclusions This study revealed a significant relationship between renal volume, renal length and number of renal cysts and BP. It is suggested that children with ADPKD should regularly be checked for BP changes by ABPM, especially those who show increased renal size or a high number of renal cysts on ultrasound. All these children are at high risk for development of hypertension. Blood Press Monit 8:107-110 c 2003 Lippincott Williams & Wilkins.
Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
The presence of hypertension in autosomal dominant polycystic kidney disease (ADPKD) appear to be related with progressive renal failure and end-organ damage, including left ventricular hypertrophy (LVH) in these patients. The aim of this study was to evaluate the frequency of hypertension in ADPKD patients and its correlation with renal function, renal structure and its influence in left ventricular wall. Two hundred patients were included in the study. The patients were divided in two groups: first group of 92 patients with normal renal function, and second group of 108 patients with chronic renal failure. All patients performed an abdominal ultrasound and a M-mode echocardiography. Hypertension was observed in 140 ADPKD patients (70%). Subjects who developed hypertension before age 35 had worse renal survival than those who remained normotensive after age 35 (50 years vs. 62 years; p<0.0001; risk ratio = 4.3). Hypertensive patients had significantly higher serum creatinine con...
2010
BACKGROUND AND AIMS Cardiovascular complications are major causes of morbidity and mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). In particular, hypertension is insidious and remains a continuous problem that evolves during the course of the disease. Hypertension in ADPKD has been associated with abnormality in the renin-angiotensin-aldosterone system (RAAS). Early vascular changes have also been reported in young ADPKD patients. In addition, the cellular functions of mechanosensory cilia within vascular system have emerged recently. The basic and clinical perspectives of RAAS, vascular remodeling and sensory cilia are reviewed with regard to hypertension in ADPKD.
Autosomal dominant polycystic kidney disease in young adults
Clinical Kidney Journal
Background The clinical manifestations of autosomal dominant polycystic kidney disease (ADPKD) usually appear in adulthood, however pediatric series report a high morbidity. The objective of the study was to analyze the clinical characteristics of ADPKD in young adults. Methods Family history, hypertension, albuminuria, estimated glomerular filtration rate (eGFR) and imaging tests were examined in 346 young adults (18–30 years old) out of 2521 patients in the Spanish ADPKD registry (REPQRAD). A literature review searched for reports on hypertension in series with more than 50 young (age <30 years) ADPKD patients. Results The mean age of this young adult cohort was 25.24 (SD 3.72) years. The mean age at diagnosis of hypertension was 21.15 (SD 4.62) years, while in the overall REPQRAD population was aged 37.6 years. The prevalence of hypertension was 28.03% and increased with age (18–24 years, 16.8%; 25–30 years, 36.8%). Although prevalence was lower in women than in men, the age a...