
International T1 Multicentre CMR Outcome Study
CardiomyopathyHeart Failure4 moreMyocardial fibrosis is the fundamental substrate for the development of heart failure. Cardiovascular magnetic resonance (CMR) allows non-invasive assessment of myocardial fibrosis based on late gadolinium enhancement (LGE) and T1 mapping. Patients: Prospective longitudinal observational multicenter study of consecutive patients with suspected or known non-ischemic cardiomyopathy. Imaging: Non-invasive measures of myocardial fibrosis: native T1, extracellular volume fraction (ECV) and LGE. Primary endpoints: all cause and cardiovascular mortality. Secondary endpoints: arrhythmic composite and HF composite endpoints.

Uraemic Toxins in Chronic Kidney Disease Paediatric Patients: Observational Study
Chronic Kidney DiseaseChildren with chronic kidney disease (CKD) suffer from one of the most devastating diseases in childhood resulting in a lifelong need for health care, and a 3 times decreased life expectancy. In addition, they have important comorbidities that negatively impact on their quality of life and integration in society, jeopardizing their future even after a potential transplantation. Retention of uraemic toxins is accepted to play a major role in the pathogenesis of the comorbid conditions, but studies in children are lacking. Furthermore, there are currently no good tools to evaluate severity and monitor adequacy of treatment, resulting in suboptimal management. The overall scientific objective of this four years UToPaed IWT-TBM project is to provide the clinician with new diagnostic and therapeutic tools for the management of children with CKD, based on the improved understanding of uraemic toxicity. In this first part of UToPaed, the investigators will associate concentrations of a wide variety of uraemic toxins with different comorbidities in CKD children, i.e. growth, protein-energy wasting, quality of life, cardiovascular risk factors, circadian rhythm, sleep quality, and psychosocial and neurocognitive functioning (i.e. cross-sectional and longitudinal). The toxins of which concentrations are best correlated with comorbidities during the progress of CKD and eventually have representative kinetics (UToPaed - part 2: Kinetic analysis) will be selected as markers. These markers will be, together with the comorbidities, further tracked after interventions, i.e. starting on dialysis, transplantation, changes in dialysis strategy (UToPaed - part 3 - intervention study). From the validated kinetic models (UToPaed - part 2 and 3), an open access user-friendly prediction simulator (PAEDSIM) based on patient characteristics and marker concentrations will be developed to optimise and individualise the dialysis therapy. By providing clinicians with more advanced and appropriate tools to improve management of all children with CKD, i.e. better assessment of the degree of renal dysfunction, better determination of the ideal time to start renal replacement therapy, and more accurate monitoring of dialysis adequacy, the investigators aim to improve neurocognitive and psychosocial functioning (short term), growth, maturation into puberty, and social integration (median term) and survival (long term).

Sex-specific Association With Kidney Disease
Diabetic NephropathyOxidative stress is involved in the pathophysiology of diabetic nephropathy. The superoxide-generating nicotinamide adenine dinucleotide phosphate-oxidase 2 (NOX2, encoded by the CYBB gene) and the antioxidant enzyme glutathione peroxidase 4 (GPX4) play opposing roles in the balance of cellular redox status. In the present study, we investigated associations of single nucleotide polymorphisms (SNPs) in the regulatory regions of CYBB and GPX4 with kidney disease in patients with type 1 diabetes.

Methylation Biosignature in Childhood Chronic Kidney Disease
Chronic Kidney DiseaseCardiovascular DiseaseChronic kidney disease (CKD) and end-stage renal disease are highly prevalent in Taiwan. Cardiovascular disease (CVD) is the most common cause of death in children with CKD. Nitric oxide (NO) deficiency links CKD and CVD. Asymmetric dimethylarginine (ADMA), a NO synthase inhibitor, its level is increased in kidney disease and cardiovascular disease and serves as a methylation biomarker. In addition to ADMA, uremic environment, hyperhomocysteinemia (Hcy) and oxidative stress may affect DNA methylation. S-adenosylmethionine (SAM) is an important human methyl donor. S-adenosylhomocysteine (SAH) is demethylated product. Methylenetetrahydrofolate reductase (MTHFR), a folate metabolism enzyme can regulate methylation pathway. The investigators intend to examine whether ADMA, SAM/SAH ratio, Hcy, and MTHFR gene methylation can serve as biosignature to predict CVD in children with CKD children.

RenalGuard System for Prevention of Contrast Induced Nephropathy
Radiographic Contrast Agent NephropathyThe purpose of this study is to determine whether hydration with intravenous saline matched with urine output, using the device RenalGuard is superior to standard hydration with saline to prevent contrast-induced nephropathy.

Patient INformation About Options for Treatment - PINOT
Chronic Kidney DiseaseThis prospective observational study is designed to find out what treatment options new patients with chronic kidney disease learn about from their renal unit. Aims: To determine the proportion of new CKD patients who receive information about treatment options prior to commencing dialysis, pre-emptive transplantation or conservative management. To determine the timing (i.e. patient's stage of disease) when information is given. To find out whether patients have a friend or family member with them when information is given. Research Design and methods: This study is an assessment of CKD education practices. Nephrologists and pre-dialysis coordinators from each renal unit will complete questionnaires about information that was given to each new dialysis, pre-emptive transplant or conservatively managed patient that started treatment during a 3 month period. Study hypothesis: Approximately one third of CKD patients will receive information after starting treatment. There will be a positive association between in-centre haemodialysis and later referral, non-English speaking background, and advancing age. Significance: The results from this national audit will provide Australian nephrologists and renal nurses with evidence about CKD education practices and compliance with clinical practice guidelines. The results may highlight opportunities for improvement in practice.

Efficacy Study of Water Drinking on PKD Progression.
Autosomal Dominant Polycystic Kidney DiseaseDisease ProgressionThis 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.

Diurnal Variation of Uremic Solutes in Peritoneal Dialysis
Chronic Kidney DiseaseStudy on the daytime variation of uremic retention solutes and markers of bone-mineral metabolism in patients with end-stage kidney disease treated with peritoneal dialysis

Mapping Multidimensional Illness Trajectories of Patients With End-stage Kidney Disease
End Stage Kidney DiseasePatients with end-stage kidney disease (ESKD) must manage not only the demands of dialysis and comorbid conditions but also associated symptoms and acute life-threatening events that contribute to functional limitations and increased risk of mortality. Yet little is known about the trajectories of quality of life dimensions in patients with ESKD when they experience acute life-threatening episodes, to guide palliative care for this population. In this prospective, descriptive study, we will identify trajectories of symptoms, physical functioning, psychosocial needs, and emotional and spiritual well-being in 200 patients with ESKD who are at a high risk of experiencing acute life-threatening events and mortality within 12 months.

ADPKD Cohort Study
Autosomal Dominant Polycystic Kidney DiseaseThe 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.