Active clinical trials for "Polycystic Kidney, Autosomal Dominant"

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of end-stage kidney disease (ESKD). The disorder is characterized by development and continued growth of multiple cysts requiring renal replacement therapy in 50% of patients by age 60 years. However, ADPKD is also a complex metabolic disorder defined by insulin resistance (IR) and mitochondrial dysfunction which may be causally related to cyst expansion, kidney function decline and contribute to reduced life expectancy. Renal hypoxia, stemming from a potential metabolic mismatch between increased renal energy expenditure and impaired substrate utilization, is proposed as a novel unifying early pathway in the development and expansion of renal cysts in ADPKD. By examining the interplay between renal O2 consumption and energy utilization in young adults with and without ADPKD, the investigators hope to identify novel therapeutic targets to impede development of cyst expansion in future trials. The investigators propose to address the specific aims in a cross-sectional study with 20 adults with ADPKD (50% female, ages 18-40 years). Comparative data will be provided from healthy adults from an ongoing study with similar study design and methods (CROCODILE Study: Control of Renal Oxygen Consumption, Mitochondrial Dysfunction, and Insulin Resistance). For this protocol, participants will complete a one day study visit at Children's Hospital Colorado. Patients will undergo a dual energy x-ray absorptiometry (DXA) to assess body composition, and a 11C-acetate positron emission tomography (PET/CT) scan to quantify renal O2 consumption. After the PET/CT, participants will undergo a hyperinsulinemic-euglycemic clamp while fasting to quantify insulin sensitivity. Glomerular Filtration Rate (GFR) and Effective Renal Plasma Flow (ERPF) will be measured by iohexol and PAH clearances during the hyperinsulinemic-euglycemic clamp.

The Division of Kidney Urology and Hematology Disease (DKUHD) of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) funded a cooperative agreement (UO1) for a consortium of participating clinical centers (PCCs) and a data coordinating and imaging analysis center (DCIAC) to develop and implement studies to test whether imaging techniques can provide accurate and reproducible markers of progression of renal disease in patients with polycystic kidney disease. The awarded participating clinical centers are Emory University, University of Kansas, and Mayo Foundation (with a subcontract to the University of Alabama). The awarded DCIAC is Washington University in St. Louis. Due to the relocation of the DCIAC P.I. from Washington University to the University of Pittsburgh, the DCIAC for CRISP II is located at the University of Pittsburgh.

There are substantial data demonstrating increased endothelial dysfunction, inflammation and oxidative stress in patients with Autosomal dominant polycystic kidney disease (ADPKD), the association between serum uric acid level and endothelial dysfunction has not been elucidated yet in these patients. Therefore, in this study, the investigators aimed to examine the relationship between the uric acid level and the ED in normotensive ADPKD patients with preserved renal function.

Pathological features of Autosomal Dominant Polycystic Kidney disease (ADPKD) include enlarged kidney volume, a higher frequency of digestive diverticulitis and abdominal wall hernias. Therefore, many nephrologists have concerns about the use of peritoneal dialysis (PD) in ADPKD patients. We aimed to analyze survival and technique failure in ADPKD patients treated with PD.

The aim of this pilot project is to assess the potential of urine micro-RNAs (miRNA) as biomarkers for characterizing patients with autosomal dominant polycystic kidney disease (ADPKD) compared with patients with other causes of chronic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is first genetic kidney disease and fourth etiology of end stage renal disease in the world. Peritoneal dialysis is underuse in this population. Indeed in this pathology, behind big kidneys and big liver, a hyper pressure is feared with technical failure. The lack of abdominal space could generate increase of peritoneal pressure. Hyper pressure is already known to be a risk factor of technical failure and over mortality in peritoneal dialysis patients (all nephropathies included). It depends on body mass index and body surface modulating injected volume for each patient. Anticipate peritoneal pressure in this population ADPKD could be an important information for distinguish those who can use peritoneal dialysis without fear and those at risk of technical failure. The primary objective is to create and validate prediction score for intra-peritoneal pressure, in peritoneal dialysis for ADPKD patients thanks to clinical and radiological values. The secondary objectives are to study the association between intra-peritoneal pressure and patient's outcome (global survival and technical survival). Retrospective, multicentric, national, cohort study will be performed. For the first step (score creation): ADPKD patients starting peritoneal dialysis for end stage renal failure between 01/01/2010 and 31/12/2015 with tomodensitometry between one year before beginning and one year after were included. For the second step (score validation): ADPKD patients starting peritoneal dialysis for end stage renal failure between 01/01/2016 and 31/12/2017 with tomodensitometry between one year before beginning and one year after were included.

This is a prospective 5-year study to compare the change in total kidney volume (TKV) before and after tolvaptan therapy, as the primary endpoint, in patients with ADPKD.

The purpose of this study is to find out if radiology tests of the kidneys as opposed to glomerular filtration (GFR) tests (GFR test - a lab test that measures kidney function) follow progression of polycystic kidney disease (PKD) the best. PKD patients at risk for progression to renal failure (dialysis or transplantation) have been identified and include those who have been diagnosed with high blood pressure early, the presence of the PKD1 gene (the inherited abnormality responsible for the majority of PKD), men as opposed to women, those with episodes of visible blood or increased protein in their urine, and women who have experience more than three pregnancies. Individuals who are diagnosed with PKD in the first year of life or in utero (before birth) are also at high risk for progression to renal failure. This study will also facilitate understanding of human diseases at the cellular and molecular level. We will be identifying genetic factors that may influence the severity of polycystic kidney disease (PKD). You are being asked to provide a sample of blood for the purpose of DNA or other biochemical analyses.

This is a prospective, clinical, multicentre study aimed to collect biological samples and study microbiota from subjects suffering from chronic kidney disease and from healthy volunteers. Microbiota is a complex consortium of microorganisms, located at the mucosal level (in particular intestinal, oral and vaginal) having a key role in human health and in the onset of several diseases. Microbiota alterations have been found in several diseases (gastrointestinal, metabolic, renal, oncological, gynaecological). The study will allow to: Provide biological samples (faeces, saliva, blood, urine) from healthy volunteers and patients suffering from chronic renal diseases to the first Italian microbiota biobank; Study microorganisms using different in vitro and in vivo techniques; Study the link between the microbiota and the disease. This study is part of the BIOMIS project (Project Code: ARS01_01220), presented as part of the "Avviso per la presentazione di progetti di ricerca industriale e sviluppo sperimentale nelle 12 aree di specializzazione individuate dal PNR 2015-2020" and admitted to funding under the National Operational Program "Ricerca e Innovazione" 2014-2020 by directorial decree of MIUR - Department for Higher Education and Research - n. 2298 of 12 September 2018. BIOMIS includes several clinical studies that enrol patients with different pathologies to collect and store biological samples and study microbiota.

EuroCYST initiative aims to build a large, well-characterized cohort of Autosomal Dominant polyCYSTic Kidney Disease (ADPKD) subjects who are followed in a longitudinal observational cohort study has the potential to identify progression factors and biomarkers, and to assess disease stage specific mortality, morbidity and health care costs.