Cardiovascular disease, closely related to an early appearance of hypertension, is the most common mortality cause among autosomal dominant polycystic kidney disease patients (ADPKD). The development of hypertension is related to an increase in renal volume. Whether the increasing in the renal volume before the onset of hypertension leads to a major cardiovascular risk in ADPKD patients remains...

Background: Autosomal dominant polycystic kidney disease (ADPKD) affects 12.5 million worldwide. Vasopressin drives cysts growth and in animal models can be suppressed through high water intake. A randomized controlled trial of 'high' versus 'standard' water intake in ADPKD is essential to determine if this intervention is beneficial. We conducted an ADPKD patient survey to gain an understandin...

A 78-year-old man with end-stage kidney disease secondary to autosomal dominant polycystic disease (ADPKD) on haemodialysis, presented to our institution following a myocardial infarction. He was known to have cystic involvement of his liver and spleen, significant mitral regurgitation secondary to mitral valve prolapse, and a distant history of abdominal aortic aneurysm repair. There was no fa...

This is the first report of a case of sacral radicular cysts in a patient with autosomal dominant polycystic kidney disease (ADPKD). A 46-year-old woman with ADPKD was found to have bilateral sacral radicular cysts discovered incidentally by magnetic resonance imaging (MRI). Cysts arising from arachnoid or spinal meningeal sac should be considered one of the manifestations of a more widespread ...

A frequent question asked by parents of children at risk of inheriting autosomal dominant polycystic kidney disease (ADPKD) is: “should my child be tested?” Until recently, most doctors answered “no.” They regarded ADPKD as an “adult” condition and advised families to wait until their children were grown up and not to worry them during childhood. But is this an ethical response today given grow...

BACKGROUND Patients who harbor intracranial aneurysms (IAs) run a risk for aneurysm rupture and subsequent subarachnoid hemorrhage which frequently results in permanent deficits or death. Prophylactic treatment of unruptured aneurysms is possible and recommended depending on the size and location of the aneurysm as well as patient age and condition. IAs are major manifestations of autosomal dom...

CONTEXT Autosomal dominant polycystic kidney disease (ADPKD) is a common disorder that can cause hypertension during childhood, but the true prevalence of hypertension during childhood is not known. OBJECTIVE We undertook a systematic review and meta-analysis to determine the prevalence of hypertension in children with ADPKD. DATA SOURCES Systematic review of articles published between 1980...

Introduction Mutations in PKD1 and PKD2 cause autosomal dominant polycystic kidney disease (ADPKD). Experimental evidence suggests an important role of the polycystins in cardiac development and myocardial function. To determine whether ADPKD may predispose to the development of cardiomyopathy, we have evaluated the coexistence of diagnoses of ADPKD and primary cardiomyopathy in our patients. ...

BACKGROUND The pathogenesis of progressive renal insufficiency in autosomal dominant polycystic kidney disease (ADPKD) is unclear. Evidence from experimental models of ADPKD suggests that elevated endothelin-1 (ET-1) drives cyst growth, renal fibrosis and loss of renal function, but whether ET-1 is elevated in humans with ADPKD is uncertain. METHODS In a cross-sectional study of ADPKD we meas...

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is characterized by renal tubular cell proliferation and dedifferentiation, which may influence tubular secretion of creatinine (CCr[TS]). STUDY DESIGN Diagnostic test study. SETTING & PARTICIPANTS We therefore investigated CCr(TS) in patients with ADPKD and controls and studied consequences for the performance of glomerular fi...