Refine
Has Fulltext
- yes (54)
Is part of the Bibliography
- yes (54)
Year of publication
Document Type
- Journal article (54)
Language
- English (54)
Keywords
- chronic kidney disease (12)
- Fabry disease (11)
- enzyme replacement therapy (5)
- hemodialysis (5)
- mortality (4)
- type 2 diabetes (4)
- cardiovascular events (3)
- end-stage renal disease (3)
- epidemiology (3)
- Chronic kidney disease (2)
- Diabetes mellitus (2)
- Enzyme replacement therapy (2)
- Fabry nephropathy (2)
- Kidney function (2)
- United States (2)
- blood pressure (2)
- cardiomyopathy (2)
- cardiovascular disease (2)
- cardiovascular morbidity (2)
- convection volume (2)
- diabetes (2)
- diabetes mellitus (2)
- dialysis (2)
- dialysis adequacy (2)
- fibrosis (2)
- genome-wide association (2)
- glomerular filtration rate (2)
- glycemic control (2)
- heart failure (2)
- hemodiafiltration (2)
- hypertrophic cardiomyopathy (2)
- kidney (2)
- left ventricular hypertrophy (2)
- proteinuria (2)
- safety (2)
- stage renal-disease (2)
- 2-dimensional speckle tracking (1)
- A-delta fibers (1)
- ACC/AHA classification (1)
- ASE formula (1)
- African-americans (1)
- Agalsidase beta (1)
- Alpha-Galactosidase (1)
- Alpha-galactosidase (1)
- Amino acids (1)
- Anderson-Fabry Disease (1)
- CKD (1)
- CMR (1)
- Calcium Citrate (1)
- Cardiomyopathy (1)
- Cardiovascular diseases (1)
- Catheter Lock Solution (1)
- Catheter-related Bloodstream Infections (CRBSI) (1)
- Chronic Kidney-disease (1)
- Chronic kidney-disease (1)
- Clinical proteomics (1)
- Clinical trial (1)
- Clinical-trials (1)
- Cohort study (1)
- Cumulative incidence function (1)
- D313Y genotype (1)
- Diabetic nephropathies (1)
- Diabetic-nephropathy (1)
- Dipeptidyl-peptidase IV inhibitors (1)
- Discovery (1)
- Diversity (1)
- EUROASPIRE survey (1)
- Epidemiology (1)
- European Society (1)
- Fabry (1)
- Fabry cardiomyopathy (1)
- Fabry genotype (1)
- Fabry patient (1)
- Fabry phenotype (1)
- Fabry-associated pain (1)
- Family Investigation of Nephropathy and Diabetes (1)
- Female patients (1)
- Galactosidase-A gene (1)
- General-population (1)
- Globotriaosylceramide (1)
- Glomerular-filtration-rate (1)
- Glycaemic control (1)
- HLA (1)
- Haemodialysis (1)
- Hazards (1)
- Heart failure (1)
- Hemodialysis-patients (1)
- Hemoglobin A1C (1)
- Homoarginine (1)
- ICD-coding of CKD (1)
- Insulin therapy (1)
- KDIGO (1)
- L-arginine (1)
- LDL cholesterol (1)
- LV mass (1)
- Lag time (1)
- Linagliptin (1)
- Lyso-Gb3 (1)
- Mass-spectrometry (1)
- Model (1)
- Mortality (1)
- Natural-history data (1)
- Network (1)
- New mexico (1)
- Nrf2 (1)
- Onset hypertrophic cardiomyopathy (1)
- Oral antidiabetic drugs (1)
- Outcome survey (1)
- Pain (1)
- Pain-related evoked potentials (1)
- Postmarketing Experience (1)
- Prevalence (1)
- Probabilities (1)
- Progression (1)
- Quality of life (1)
- Racial differences (1)
- Regression (1)
- Research design (1)
- Risk (1)
- SF-36 (1)
- SGLT2 inhibitor (1)
- Sample-sizes (1)
- Small fiber neuropathy (1)
- Stage renal-disease (1)
- Subdistribution (1)
- Sudden cardiac death (1)
- Taurolidine (1)
- Teichholz formula (1)
- Tests (1)
- Treatment outcome (1)
- VEMP (1)
- Variants (1)
- X-chromosomal inactivation (1)
- Young-patients (1)
- activated-receptor gamma (1)
- add-on (1)
- agalsidase-beta (1)
- agnoists (1)
- albumin excretion rate (1)
- albuminuria (1)
- aldosterone (1)
- alkaline phosphatase (1)
- alpha galactosidase (1)
- alpha-galactosidase-A (1)
- alpha/delta agonist GFT505 (1)
- anaemia (1)
- anemia (1)
- antagonist (1)
- anti-drug antibodies (1)
- anticoagulation (1)
- antidiabetic agents (1)
- arial fibrillation (1)
- atherogenic dyslipidaemia (1)
- atherosclerosis risk (1)
- bardoxolone methyl (1)
- biocompatibility (1)
- biomarker (1)
- biopsy (1)
- biopsy findings (1)
- blood-glucose control (1)
- body weight (1)
- calcification (1)
- canagliflozin (1)
- cancer risk factors (1)
- cardiac dysfunction (1)
- cardiac energy metabolism (1)
- cardiac hypertrophy (1)
- cardiovascular death (1)
- cardiovascular magnetic resonance (1)
- cardiovascular magnetic-resonance (1)
- cardiovascular mortality (1)
- cardiovascular munster procam (1)
- cardiovascular outcomes (1)
- cardiovascularm disease (1)
- chronic kidney-disease (1)
- clinical manifestations (1)
- clinical trial (1)
- colestilan (1)
- comorbidity (1)
- consensus conference (1)
- converting enzyme-inhibition (1)
- cornea verticillata (1)
- coronary artery disease (1)
- coronary heart disease (1)
- cortisol (1)
- costs (1)
- creatine synthesis (1)
- cryptogenic stroke (1)
- diabetes complications (1)
- diabetes mellitus type 2 (1)
- diabetic kidney disease (1)
- diabetic mouse (1)
- diabetic nephropathy (1)
- diagnosis (1)
- diagnosis in Fabry disease (1)
- dietary sodium restriction (1)
- disease (1)
- doule blind (1)
- dysfunction (1)
- echocardiography (1)
- efficacy (1)
- empagliflozin (1)
- end stage renal disease (1)
- end-stage kidney disease (1)
- erythropoietin (1)
- fabry disease (1)
- failure (1)
- female Fabry patients (1)
- ferritin (1)
- fibroblast growth factor-23 (1)
- focal semental glomerulosclerosis (1)
- fractionation membranev (1)
- free light chains (1)
- gene variant (1)
- genetic renal disease (1)
- genetics (1)
- genome-wide association study (1)
- genotype/phenotype correlation (1)
- genotyping (1)
- global outcomes (1)
- glomerular-filtration-rate (1)
- glomerulonephritis (1)
- haemodiafiltration (1)
- hearing loss (1)
- hemoglobin (1)
- heodialysis patients (1)
- high denisty lipoprotein (1)
- hypercholesterolemia (1)
- hyperphosphataemia (1)
- hypersensitivity (1)
- identification (1)
- immunofluorescence (1)
- impact (1)
- incidence (1)
- induced insulin-release (1)
- infections (1)
- inflammation (1)
- inherited metabolic disorders (1)
- inos (1)
- insufficiency (1)
- intensive glucose control (1)
- iron (1)
- ischemia-reperfusion injury (1)
- italian population (1)
- kidney disease (1)
- left ventricular mass (1)
- left ventricular mass index (1)
- left-ventricular hypertrophy (1)
- linkage (1)
- lipoprotein apheresis (1)
- lipoprotein(a) (1)
- lysosomal storage disease (1)
- lysosomal storage disorder (1)
- macrovascular (1)
- medical dialysis (1)
- medium cut-off dialyzer (1)
- melanoma (1)
- men born (1)
- metformin (1)
- mices (1)
- microvascular (1)
- mineral metabolism (1)
- multiple sclerosis (1)
- myocardial infarction (1)
- myocardial fibrosis (1)
- natural history (1)
- natural-history data (1)
- nerve fibers (1)
- neuropathy (1)
- nictric-oxide (1)
- overload (1)
- oxidative stress (1)
- parathyroid hormone (1)
- pathophysiology (1)
- patients’ awareness (1)
- phase IV (1)
- phosphate homeostasis (1)
- phospholipid fatty acids (1)
- physicians’ awareness (1)
- pioglitazone (1)
- placebo (1)
- placebo-controlled trial (1)
- podocyte (1)
- prediction model (1)
- prevalence (1)
- protein-bound uremic toxins (1)
- randomized controlled trial (1)
- randomized controlled-trial (1)
- randomized trial (1)
- rat kidney (1)
- receptor (1)
- recombination hotspot (1)
- renal disease (1)
- renal dysfunction (1)
- renal fibrosis (1)
- renal function (1)
- renal replacement therapy (1)
- renal system (1)
- renoprotection (1)
- replacement therapy (1)
- residual cardiovascular risk (1)
- risk (1)
- risk factors (1)
- septal hypertrophy (1)
- serum creatinine (1)
- sevelamer (1)
- single nucleotide polymorphisms (1)
- skin diseases (1)
- sudden cardiac death (1)
- sulfonylurea (1)
- survival (1)
- term fenofibrate therapy (1)
- therapeutic options (1)
- therapy (1)
- trial design (1)
- triglyceride-rich lipoproteins (1)
- troponin T (1)
- type 2 (1)
- type-1 diabetes mellitus (1)
- type-2 diabetes mellitus (1)
- urinary protein excretion (1)
- vertigo (1)
- vitamin D (1)
Institute
- Medizinische Klinik und Poliklinik I (54) (remove)
Sonstige beteiligte Institutionen
EU-Project number / Contract (GA) number
- 602133 (1)
Background: Evidence concerning the importance of glucose lowering in the prevention of cardiovascular (CV) outcomes remains controversial. Given the multi-faceted pathogenesis of atherosclerosis in diabetes, it is likely that any intervention to mitigate this risk must address CV risk factors beyond glycemia alone. The SGLT-2 inhibitor empagliflozin improves glucose control, body weight and blood pressure when used as monotherapy or add-on to other antihyperglycemic agents in patients with type 2 diabetes. The aim of the ongoing EMPA-REG OUTCOME (TM) trial is to determine the long-term CV safety of empagliflozin, as well as investigating potential benefits on macro-/microvascular outcomes.
Methods: Patients who were drug naive (HbA(1c) >= 7.0% and <= 9.0%), or on background glucose-lowering therapy (HbA(1c) >= 7.0% and <= 10.0%), and were at high risk of CV events, were randomized (1:1:1) and treated with empagliflozin 10 mg, empagliflozin 25 mg, or placebo (double blind, double dummy) superimposed upon the standard of care. The primary outcome is time to first occurrence of CV death, non-fatal myocardial infarction, or non-fatal stroke. CV events will be prospectively adjudicated by an independent Clinical Events Committee. The trial will continue until >= 691 confirmed primary outcome events have occurred, providing a power of 90% to yield an upper limit of the adjusted 95% CI for a hazard ratio of <1.3 with a one-sided a of 0.025, assuming equal risks between placebo and empagliflozin (both doses pooled). Hierarchical testing for superiority will follow for the primary outcome and key secondary outcomes (time to first occurrence of CV death, non-fatal myocardial infarction, non-fatal stroke or hospitalization for unstable angina pectoris) where non-inferiority is achieved.
Results: Between Sept 2010 and April 2013, 592 clinical sites randomized and treated 7034 patients (41% from Europe, 20% from North America, and 19% from Asia). At baseline, the mean age was 63 +/- 9 years, BMI 30.6 +/- 5.3 kg/m(2), HbA1c 8.1 +/- 0.8%, and eGFR 74 +/- 21 ml/min/1.73 m(2). The study is expected to report in 2015.
Discussion: EMPA REG OUTCOME (TM) will determine the CV safety of empagliflozin in a cohort of patients with type 2 diabetes and high CV risk, with the potential to show cardioprotection.
Background: Impairments of health related quality of life (HRQoL) are frequently observed in Fabry disease (FD) and are known to be related to neuropathic pain and cardiovascular events. This study aimed to explore the role of chronic kidney disease (CKD) in a large cohort of patients with FD.
Methods: In 96 patients (53% female; age 40 ± 12 yrs) with genetically proven FD, HRQoL was assessed by the Medical Outcomes Study (SF-36) questionnaire. All patients were naïve to enzyme replacement therapy. Three categories for kidney dysfunction were chosen, eGFR ≥/<60 ml/min/1.73 m2 or need of renal replacement therapy (RRT). Minor (e.g. arrhythmia, angina pectoris, etc.) and major (e.g. myocardial infarction, coronary artery bypass, stroke or implantable cardioverter-defibrillator) vascular events as well as pain and pain therapy were considered in linear regression analyses with the dimensions of HRQoL.
Results: Ten patients (10%) had impaired kidney function and a further nine were on RRT (9.4%). Kidney function and pain emerged as the main factors associated with lower scores on the SF 36, in particular on physical components (PCS beta-coefficients for CKD −6.2, for RRT −11.8, for pain −9.1, p < 0.05, respectively), while controlling for gender, vascular event and pain-therapy. Relationships were found for mental aspects of HRQoL. Age and history of vascular events were not related to HRQoL.
Conclusion: Cardiovascular events and pain are important factors related to HRQoL, social functioning and depression. Our study highlights impaired chronic kidney disease, in particular after initiation of RRT, as a strong determinant of reduced HRQoL in FD.
Background
Fabry disease is an inborn lysosomal storage disorder which is associated with small fiber neuropathy. We set out to investigate small fiber conduction in Fabry patients using pain-related evoked potentials (PREP).
Methods
In this case–control study we prospectively studied 76 consecutive Fabry patients for electrical small fiber conduction in correlation with small fiber function and morphology. Data were compared with healthy controls using non-parametric statistical tests. All patients underwent neurological examination and were investigated with pain and depression questionnaires. Small fiber function (quantitative sensory testing, QST), morphology (skin punch biopsy), and electrical conduction (PREP) were assessed and correlated. Patients were stratified for gender and disease severity as reflected by renal function.
Results
All Fabry patients (31 men, 45 women) had small fiber neuropathy. Men with Fabry disease showed impaired cold (p < 0.01) and warm perception (p < 0.05), while women did not differ from controls. Intraepidermal nerve fiber density (IENFD) was reduced at the lower leg (p < 0.001) and the back (p < 0.05) mainly of men with impaired renal function. When investigating A-delta fiber conduction with PREP, men but not women with Fabry disease had lower amplitudes upon stimulation at face (p < 0.01), hands (p < 0.05), and feet (p < 0.01) compared to controls. PREP amplitudes further decreased with advance in disease severity. PREP amplitudes and warm (p < 0.05) and cold detection thresholds (p < 0.01) at the feet correlated positively in male patients.
Conclusion
Small fiber conduction is impaired in men with Fabry disease and worsens with advanced disease severity. PREP are well-suited to measure A-delta fiber conduction.
Nephrogenic Systemic Fibrosis is a rare condition appearing only in patients with severe renal impairment or failure and presents with dermal lesions and involvement of internal organs. Although many cases are mild, an estimated 5 % have a progressive debilitating course. To date, there is no known effective treatment thus stressing the necessity of ample prevention measures. An association with the use of Gadolinium based contrast agents (GBCA) makes Nephrogenic Systemic Fibrosis a potential side effect of contrast enhanced magnetic resonance imaging and offers the opportunity for prevention by limiting use of gadolinium based contrast agents in renal failure patients. In itself toxic, Gadolinium is embedded into chelates that allow its safe use as a contrast agent. One NSF theory is that Gadolinium chelates distribute into the extracellular fluid compartment and set Gadolinium ions free, depending on multiple factors among which the duration of chelates exposure is directly related to the renal function. Major medical societies both in Europe and in North America have developed guidelines for the usage of GBCA. Since the establishment of these guidelines and the increased general awareness of this condition, the occurrence of NSF has been nearly eliminated. Giving an overview over the current knowledge of NSF pathobiochemistry, pathogenesis and treatment options this review focuses on the guidelines of the European Medicines Agency, the European Society of Urogenital Radiology, the FDA and the American College of Radiology from 2008 up to 2011 and the transfer of this knowledge into every day practice.