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Cystine Lithiasis in the Netherlands;
Clinical and genetic aspects

- To evaluate the demographic, clinical and genetic characteristics of all known patients with a diagnosis of cystinuria and cystine nephrolithiasis in the Netherlands.- To evaluate the relationship between genotype and phenotype of all known…

GENETIC STUDIES OF CHRONIC KIDNEY DISEASE - GENEKID

To elucidate the molecular mechanisms underlying renal disease that lead to chronic kidney disease. To validate pathogenic variations in functional in vivo models.

Prospective comparative study between Deflux® and Vantris® for endoscopic treatment of vesico-ureteral reflux in children.

Primary Objective: The treatment result expressed as the proportion of successful treatments per treatment arm (successful = VUR <= grade 1 and/or no urinary-tract infections 6 months after the operation) does at least not show a significant…

Size and volume charts of fetal bladder during the second and third trimester of pregnancy

The main objective of the study is to construct reference curves for fetal bladder diameters and volume in the second and third trimester of pregnancy. Moreover we will investigate the variation in the dimensions of renal pelvis in relation to the…

A Phase 2a, Single-Center Study Investigating the Short-Term Renal Hemodynamic Effects, Safety and Pharmacokinetics/ Pharmacodynamics of Oral Tolvaptan in Subjects with Autosomal Dominant Polycystic Kidney Disease at Various Stages of Renal Function

The purpose of the trial is to determine the effect of multiple doses of tolvaptan on renal function in patients with autosomal dominant polycystic kidney disease (ADPKD) at various stages of renal function. Additionally, the short-term renal…

The functional consequences of the 57 kb deletion for the TRPV1-receptor in cystinosis patients

(i) To compare the DBF response to capsaicin between cystinosis patients, homozygous and heterozygous for the 57-kb deletion, and matched controls.(ii) To compare the skin sensitivity response after capsaicin application between cystinosis patients,…

A randomised, controlled clinical trial assessing the efficacy of;Lanreotide to halt disease progression in ADPKD

First, to demonstrate whether Lanreotide attenuates progression of the renal phenotype in ADPKD patients as measured by change in rate of renal function decline and change in renal volume. Second, to demonstrate whether Lanreotide modifies…

THE KIMONO-study - on the development of renal injury in children with a solitary functioning kidney.

THE KIMONO-STUDY is designed to study the consequences of having an SFK from childhood. Furthermore, a tailor made risk profil will be designed for children with an SFK, based on individual risk factor (for example co-morbidity, genetic aberrations…

A Phase 3, Multi-center, Double-blind, Placebo-controlled, Parallel-arm Trial to Determine Long-term Safety and Efficacy of Oral Tolvaptan Tablet Regimens in Adult Subjects with Autosomal Dominant Polycystic Kidney Disease

Primary objective:* Evaluate long-term effect of tolvaptan in ADPKD through rate of renal volume change(%) for tolvaptan-treated compared to placebo-treated subjects.Secondary objectives:* Evaluate long-term efficacy of tolvaptan in ADPKD through a…

Multi-center, Open-label, Extension Study to Evaluate the Long-term Efficacy and Safety of Oral Tolvaptan Tablet Regimens in Subjects with Autosomal Dominant Polycystic Kidney Disease (ADPKD)

The objective of this extension study is to find out the potential long-term benefits and safety of tolvaptan. During this study all participants will receive tolvaptan.PRIMARY OBJECTIVE is to demonstrate whether tolvaptan modifies ADPKD progression…

A Placebo-Controlled, Single-Blind, Single-Center Phase 1 Study in Normal Healthy Volunteers and Open-Label Multi Center Study in Patients with Primary Hyperoxaluria to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Single Ascending Doses of DCR PHXC Solution for Injection (subcutaneous use)

Objectives:Primary:* To evaluate the safety and tolerability of single doses of DCR-PHXC Solution for Injection (SC use) (*DCR-PHXC*) inNHV (Group A) and in patients with primary hyperoxaluria (PH) (Group B).Secondary:* To characterize the…

Hypomagnesemia as mitochondrial disease

The main objective is to investigate whether the variant indeed cause mitochondrial dysfunction leading to the observed phenotype, including characterization of the pathophysiology.

Circulation and Hemodynamics in Living Donation of Kidney Transplantation in Children

Primary objective is to design a non-invasive, bedside monitoring strategy for early detection of renal graft hypoperfusion after pediatric kidney transplantation, using transabdominal ultrasonography and biomarker surveillance in serum and urine.…

Wishing to decrease Aquaresis in ADPKD patients Treated with a V2Ra; the Effect of Regulating protein and salt

To demonstrate the effect of salt and/or protein intake on aquaresis in patients with ADPKD who are treated with a vasopressin V2 receptor antagonist as measured by 24-hour urine volume.

An exploratory, randomized, double-blind, placebo-controlled, multicenter study to evaluate the efficacy, safety, tolerability and pharmacokinetics of orally administered GLPG2737 for 52 weeks, followed by an open-label extension period of 52 weeks in subjects with autosomal dominant polycystic kidney disease (ADPKD).

Main objective- To characterize the effect of GLPG2737 on growth in total kidneyvolume (TKV) compared to placebo.- To evaluate the safety and tolerability of oral doses of GLPG2737compared to placebo.Secondary objectives: - To characterize the…