View clinical trials related to Polycystic Kidney Diseases.
Filter by:Background : Medullary sponge kidney disease is a congenital disorder caracterised by tubular ectasia and cystic dilatation of the collecting ducts in the pericalyceal region of the renal pyramids, which can lead to nephrolithiasis and frequently associated with impaired tubular function such as distal renal tubular acidosis. The disease knowledge is limited, especially about origin, diagnosis, and physiopathology of the disease. The disease is associated with impaired tubular function such as distal renal tubular acidosis, hypocitraturia, hypercalciuria, which suggest altered kidney medulla function. Multiparametric MRI may provide further informations about the physiopathology and help in earlier diagnosis of the medullary sponge kidney. Objective : The aim of this study is to test the hypothesis that early kidney medulla function alteration in medullary sponge kidney can be detected and characterised with multiparametric MRI. We are expecting to see in medullary sponge kidney a decreased oxygenation content in BOLD MRI (Blood oxygenation level dependent magnetic resonance imaging), and decreased ADC (Apparent diffusion coefficient) value in the medulla. Design : A monocentric prospective case/control study will be conducted in adults with medullary sponge kidney. Controls are adults patients with glomerular filtration rate > 60 mL/min/1.73m2 without kidney stone attending a renal exploration. After a screening visit, patients included will be evaluated on one day with lithiasis assessment and measurement of glomerular filtration (Urinary collection of the last 24 hours, Urine sample, Blood sample) and they will perform multiparametric magnetic resonance imaging of the kidneys.
Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic, rare and life-threatening disease, characterized by the pathological formation of multiple fluid-filled cysts that arise from renal tubules and alter kidney architecture and function. In most patients, the progressive deterioration of renal function ultimately leads to end-stage kidney disease (ESKD) and the need for dialysis or kidney transplantation. Save the conventional anti-hypertensive strategies, there are currently two disease-specific treatments for ADPKD (Tolvaptan and Octreotide-LAR). However, these drugs are only available to patients at high risk of progression to ESKD, while a remarkable number of ADPKD patients progress to ESKD despite the treatments. Cyst formation in ADPKD is determined by mutations in two genes encoding two transmembrane proteins: polycystin1 and polycystin2. The pathogenesis of the disease involves a series of phenotypic alterations, including the de-differentiation of epithelial cells, uncontrolled proliferation and abnormal secretion of fluids in the cysts, metabolic remodeling, all phenomena that lead to the progressive loss of renal structure and function . Therefore, to try to investigate the mechanisms of the disease, the investigators should go in search of pleiotropic molecules capable of simultaneously modulating structure, function and metabolism. Research done so far suggests that thyroid hormones (TH) may also act as pleiotropic modulators in the patho-biology of ADPKD. TH signals play a crucial role in the regulation of cell de-differentiation and cell cycle reactivation, as well as in the metabolism and evolution of cardiac and renal diseases. Interestingly, changes in TH levels have been detected in approximately 80% of patients with chronic renal failure (CKD), whereas patients with ADPKD show a higher incidence of clinical and subclinical hypothyroidism. Despite these evidences, the ability of TH to modulate anti-cystogenic and renoprotective processes in ADPKD has not yet been studied. The objective of this study is to determine the levels of THs in the serum of ADPKD patients with normal renal function and mild, moderate or severe renal dysfunction, and to correlate them with renal functional parameters.
People with autosomal dominant polycystic kidney disease (ADPKD) develop high blood pressure and kidney disease. Previous studies have shown that a commonly occurring chemical, nitric oxide (NO), is reduced in ADPKD, and may contribute, in part, to high blood pressure in this condition. Nitrate is found in high concentrations naturally in beetroots, and increases NO. The aim of this study is to determine if beetroot juice reduces blood pressure in hypertensive people with ADPKD.
The study is designed to evaluate the safety, tolerability, pharmacokinetics and pharmacodynamics of Oral AL01211 in healthy volunteers
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.
A mild reduction in food intake significantly inhibits renal cyst growth in mouse models of ADPKD. The underlying mechanism was unknown at the time. Recently published data show that the beneficial effect is not due to caloric restriction per se but due to the induction of the state of ketosis. Dietary interventions leading to ketosis profoundly inhibited renal cyst growth in rodent models of PKD. In addition, acute fasting led to rapid regression of renal cystic burden in mouse, rat and feline models of PKD. Due to these compelling effects in a multitude of PKD animal models, and due to the fact that well-established dietary interventions have a tremendous translational potential, KETO-ADPKD will test such interventions regimens in ADPKD patients. Two well-established ketogenic dietary regimens will be tested in comparison to a control group to address the following four questions: 1. Feasibility: Are ketogenic dietary interventions acceptable to ADPKD patients in everyday life? 2. Safety: Are there adverse events of ketogenic dietary interventions in ADPKD patients? 3. Efficacy: Do the dietary interventions reach the metabolic endpoints? Do they have a short-term impact on kidney volume? 4. Which of the two diets is the optimal approach? These questions will be addressed in an exploratory, randomized, open, single center, three-arm dietary intervention study using the following interventions in 21 ADPKD patients per treatment arm: A) Ketogenic diet B) 3-day water fasting C) Control: ad libitum food intake (no diet)
Primary Objective • To assess the dose response relationship between RGLS4326 and ADPKD biomarkers Secondary Objectives - To characterize the pharmacokinetic (PK) properties of RGLS4326 in plasma and urine - To assess the safety and tolerability of RGLS4326
The proposed research will determine the feasibility of a time restricted feeding intervention,a fasting regimen that restricts eating to a feeding window (8 hrs/day) for 1 year in adults with autosomal dominant polycystic kidney disease (ADPKD) who are overweight or obese. The study will provide valuable information on the intervention in terms of safety, adherence, acceptability, and tolerability. Last, this pilot trial will provide initial insight into biological changes including abdominal adiposity, changes in kidney growth and function, and markers of biological pathways related to the intervention.
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 ability to concentrate and dilute urine is primarily regulated via vasopressin (AVP) dependent Aquaporin-2 water channels (AQP2 channels) in the kidney's collecting duct. Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder, characterized by the formation of cyst in the kidneys, causing gradual renal function-loss. Previous studies indicate that ADPKD patients have decreased urine concentration, higher plasma osmolality, and plasma AVP levels compared to healthy controls. Previous studies also indicate that ADPKD patients' dysregulated AVP is an important factor for the pathogenesis and progression of the disease. It is unclear whether ADPKD patients' ability to concentrate and dilute urine are different from those of other cause of chronic renal disease to the same degree. It is also unclear, what mechanisms cause the decreased ability to concentrate and dilute urine in chronic renal disease patients. The purpose of this trial is to investigate the difference in renal function during concentration and dilution test in a case-control, randomized, cross-examination study with ADKPD patients or other cause of chronic renal disease compared to healthy volunteers.