Active clinical trials for "Polycystic Kidney Diseases"

The purpose of this study is to evaluate the effectiveness and safety of Sirolimus in reducing liver volume in autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive cyst formation in both kidneys, in most patients leading to end stage renal disease. It is the most common hereditary renal disease with a prevalence of approximately 1 in 1,000 persons. The majority of patients also have progressive cyst formation in the liver, leading to pain, gastrointestinal discomfort and sometimes the need for liver transplantation. At present there is no proven therapeutic intervention to slow the rate of disease progression in human ADPKD. The development of renoprotective treatments that are well tolerated, is therefore of major importance. In this respect, somatostatin analogues are promising for especially polycystic liver disease, but also for the renal phenotype. However, the studies that have been performed thus far with these agents, were underpowered and of too short duration to reach a definitive conclusion on the potential reno- and hepatoprotective efficacy of somatostatin analogues. Therefore, the present study is designed as a randomised clinical trial with sufficient duration of follow-up to investigate whether the somatostatin analogue Lanreotide slows progression of polycystic kidney and liver disease in ADPKD-patients. To this end, 300 ADPKD patients, aged 18-60years, with an eGFR 30-60 ml/min/1.73 m2) will be randomized 1:1 to standard care or monthly subcutaneous lanreotide injections on top off standard care. These 300 subjects will go through 15 study visits in 3 years and 1 follow up visit. During these visits, questionnaires will be filled in, physical examinations will be performed, blood will be drawn and urine collected. After study completion, rate of renal function decline in lanreotide treated subjects will be compared to that of subject who received standard care.

This study evaluates the effect of regulating salt and protein intake on urinevolume in patients with ADPKD treated with a vasopressine V2 receptor antagonist (V2RA). The investigators hypothesize that changing sodium and protein intake will reduce V2RA-induced polyuria.

This study examines the safety and efficacy of calcium channel blocker (CCB) in the treatment of hypertension of Autosomal Dominant Polycystic Kidney Disease (ADPKD) patients. Angiotensin receptor blocker (ARB) was shown to have kidney protecting effects in patients with renal diseases including ADPKD, glomerulonephritis and diabetic nephropathy. In case whose blood pressure is not normalized by ARB alone, CCB is selected additionally. Recent research suggests genetic calcium channel disorder is responsible for the progression of ADPKD. It is not examined clinically if CCB treatment has any harmful effect to patients with ADPKD. This study examines the safety of Cilnidipine (CCB) in the ADPKD patients whose blood pressure is not controlled under 130/85 mmHg by Candesartan (ARB) alone.

This phase II study examines the safety and efficacy of combination therapy for hypertension in patients with autosomal dominant polycystic kidney disease (ADPKD). This study examines the safety and efficacy of combination therapy by imidapril (ACEI) or cilnidipine (CCB) in ADPKD patients whose blood pressure is not controlled under 120/80 mmHg by candesartan (ARB) alone.

Positive data originating from two polycystic liver patients treated with somatostatin analogues, showed a volume reduction of 38.3% and 14.9%. These two patients had complicated polycystic livers and no other therapeutic options were available. Patients who participated in LOCKCYST trial are able to benefit from active treatment. Participants will be actively treated for 24 weeks.

The aim of this study is to identify families with ADPKD , characterize the phenotype and screen for mutations in known genes (PKD1 and PKD2, and then HNF1b and UMOD in PKD1 PKD2 negative carriers). Genome wide analysis will be performed in families without mutations identified.

The objective of this study is to measure the influence of both short term water restriction and high water intake on total kidney volume, measured by Magnetic Resonance Imaging (MRI) scan in Autosomal Dominant Polycystic Kidney Disease (ADPKD) patients.

The purpose of this study is to determine whether patients with autosomal dominant polycystic kidney disease (ADPKD) present with abnormal endothelial function, increased levels of NOX4 activity and mitochondrial abnormalities, contributing to oxidative stress from early stages that correlate with disease severity.

ADPKD is the most common form of hereditary kidney disease and is known to occur in 1 of 400 to 1000 population in the U.S. ADPKD consists of 2.8% of patients receiving kidney transplantation in the investigator's center. It is known that ADPKD is associated with vascular anomalies, including abdominal aneurysms, valvular anomalies and especially intracranial aneurysms. Intracranial aneurysms occur in 9~12% of the ADPKD population which is higher than 2~3% in the general population and is known to be associated with PKD1 or PKD2 heritage. Until now, most of the studies regarding intracranial aneurysms in ADPKD are conducted in animal models, and there are only few cellular studies conducted from human samples. Total 154 patients received kidney transplantation for ADPKD from 1994 to December 2018 at Asan Medical Center, Seoul, Korea. While performing kidney transplantation to ESRD ADPKD patients, nephrectomy has been routinely performed for polycystic kidney and the nephrectomy specimens can be obtained. The objective of this study is to investigate the mechanism of intracranial aneurysm in ADPKD patients by analyzing gene characteristics from nephrectomy specimens.