Refine
Has Fulltext
- yes (25)
Is part of the Bibliography
- yes (25)
Year of publication
- 2016 (25) (remove)
Document Type
- Journal article (25)
Language
- English (25) (remove)
Keywords
- chronic kidney disease (4)
- Fabry disease (2)
- cardiomyopathy (2)
- Addison's disease (1)
- Adrenocortial carcinomas (1)
- Alpha-Galactosidase (1)
- Anderson-Fabry Disease (1)
- Apoptosis (1)
- CKD (1)
- CML (1)
- Calcineurin-NFATsignaling (1)
- Cancer genetics (1)
- Cell lung canger (1)
- Chronic kidney disease (1)
- Cohort study (1)
- Contrast-enhanced CT (1)
- Cytoskeleton (1)
- D313Y genotype (1)
- DNA damage (1)
- DOTATOC (1)
- Diabetes mellitus (1)
- Disease prevalence (1)
- Diversity (1)
- Enzyme replacement therapy (1)
- Epidemiology (1)
- F-18-FDG PET/CT (1)
- Fabry cardiomyopathy (1)
- Fabry nephropathy (1)
- Fabry-associated pain (1)
- G protein coupled receptors (1)
- Galactosidase-A gene (1)
- Genome-wide association studies (1)
- Germany (1)
- Glycaemic control (1)
- Hemoglobin A1C (1)
- Insulin therapy (1)
- Kidney function (1)
- Lag time (1)
- MR (1)
- Methodological quality (1)
- Morbus Fabry (1)
- Myeloma (1)
- Natural-history data (1)
- Network (1)
- Oral antidiabetic drugs (1)
- Outcome survey (1)
- PET (1)
- Pain (1)
- Positron-emission-tomography (1)
- Racial differences (1)
- SCORE (1)
- SSTR (1)
- SUMO2 (1)
- Septal bulge (1)
- Spin echo (1)
- Test accuracy (1)
- activation (1)
- adenomas (1)
- adenoviruses (1)
- albumin excretion rate (1)
- allogeneic hematopoietic stem cell transplantation (1)
- antagonist (1)
- antidiabetic agents (1)
- biomarker (1)
- biomarkers (1)
- biopsy (1)
- blood pressure (1)
- blood pressure monitoring (1)
- bull’s eye plot (1)
- calcineurin signaling cascade (1)
- cancer risk (1)
- cardiac hypertrophy (1)
- cardiac pacing (1)
- cardiovascular disease (1)
- cardiovascular outcomes (1)
- chronic myeloid leukemia (1)
- clinical (1)
- converting enzyme-inhibition (1)
- cryptogenic stroke (1)
- cytoplasmic staining (1)
- diabetes (1)
- dietary sodium restriction (1)
- dogs (1)
- drug treatment (1)
- echocardiography (1)
- enzyme replacement therapy (1)
- fabry disease (1)
- fatal cardiovascular disease (1)
- glucocorticoid replacement therapy (1)
- glycemic control (1)
- health risk assessment (1)
- heart (1)
- heart disease (1)
- homeostasisIon channels (1)
- hyperexpression techniques (1)
- hypersensitivity (1)
- hypertension (1)
- hypertrophic cardiomyopathy (1)
- hyponatremia (1)
- immunofluorescence (1)
- infections (1)
- inherited metabolic disorders (1)
- insulin tolerance test (1)
- intensive glucose control (1)
- iron (1)
- late gadolinium enhancement (1)
- left ventricular hypertrophy (1)
- lysosomal storage disease (1)
- magnetic resonance spectroscopy (1)
- mutation (1)
- myocardial lipid content (1)
- myocardial fibrosis (1)
- nerve fibers (1)
- neuropathy (1)
- nuclear staining (1)
- overload (1)
- oxidative stress (1)
- pituitary adenomas (1)
- pituitary gland (1)
- protein expression (1)
- randomized trial (1)
- rare diseases (1)
- receptor (1)
- renal system (1)
- renoprotection (1)
- sarcoidosis (1)
- short Synacthen test (1)
- skin diseases (1)
- small interfering RNAs (1)
- somatostatin receptor (1)
- speckle tracking imaging (1)
- stage renal-disease (1)
- stem cell transplantation (1)
- thrombopoiesis (1)
- troponin T (1)
- tyrosine kinase inhibitors (1)
Institute
- Medizinische Klinik und Poliklinik I (25) (remove)
Estimation of absolute risk of cardiovascular disease (CVD), preferably with population-specific risk charts, has become a cornerstone of CVD primary prevention. Regular recalibration of risk charts may be necessary due to decreasing CVD rates and CVD risk factor levels. The SCORE risk charts for fatal CVD risk assessment were first calibrated for Germany with 1998 risk factor level data and 1999 mortality statistics. We present an update of these risk charts based on the SCORE methodology including estimates of relative risks from SCORE, risk factor levels from the German Health Interview and Examination Survey for Adults 2008–11 (DEGS1) and official mortality statistics from 2012. Competing risks methods were applied and estimates were independently validated. Updated risk charts were calculated based on cholesterol, smoking, systolic blood pressure risk factor levels, sex and 5-year age-groups. The absolute 10-year risk estimates of fatal CVD were lower according to the updated risk charts compared to the first calibration for Germany. In a nationwide sample of 3062 adults aged 40–65 years free of major CVD from DEGS1, the mean 10-year risk of fatal CVD estimated by the updated charts was lower by 29% and the estimated proportion of high risk people (10-year risk > = 5%) by 50% compared to the older risk charts. This recalibration shows a need for regular updates of risk charts according to changes in mortality and risk factor levels in order to sustain the identification of people with a high CVD risk.
Hyponatremia (HN) is a common condition, with a large number of etiologies and a complicated treatment. Although chronic HN has been shown to be a predictor of poor outcome, sodium-increasing treatments in chronic stable and asymptomatic HN have not proven to increase life expectancy. For symptomatic HN, in contrast, the necessity for urgent treatment has broadly been accepted to avoid the development of fatal cerebral edema. On the other hand, a too rapid increase of serum sodium in chronic HN may result in cerebral damage due to osmotic demyelinisation. Recently, administration of hypertonic saline bolus has been recommended as first-line treatment in patients with moderate-to-severe symptomatic HN. This approach is easy to memorize and holds the potential to greatly facilitate the initial treatment of symptomatic HN. First-line treatment of chronic HN is fluid restriction and if ineffective treatment with tolvaptan or in some patients other agents should be considered. A number of recommendations and guidelines have been published on HN. In the present review, the management of patients with HN in relation to everyday clinical practice is summarized with focus on the acute management.
Before the introduction of erythropoiesis-stimulating agents (ESAs) in 1989, repeated transfusions given to patients with end-stage renal disease caused iron overload, and the need for supplemental iron was rare. However, with the widespread introduction of ESAs, it was recognized that supplemental iron was necessary to optimize hemoglobin response and allow reduction of the ESA dose for economic reasons and recent concerns about ESA safety. Iron supplementation was also found to be more efficacious via intravenous compared to oral administration, and the use of intravenous iron has escalated in recent years. The safety of various iron compounds has been of theoretical concern due to their potential to induce iron overload, oxidative stress, hypersensitivity reactions, and a permissive environment for infectious processes. Therefore, an expert group was convened to assess the benefits and risks of parenteral iron, and to provide strategies for its optimal use while mitigating the risk for acute reactions and other adverse effects.
Patients with chronic kidney disease (CKD) exhibit an increased cancer risk compared to a healthy control population. To be able to estimate the cancer risk of the patients and to assess the impact of interventional therapies thereon, it is of particular interest to measure the patients’ burden of genomic damage. Chromosomal abnormalities, reduced DNA repair, and DNA lesions were found indeed in cells of patients with CKD. Biomarkers for DNA damage measurable in easily accessible cells like peripheral blood lymphocytes are chromosomal aberrations, structural DNA lesions, and oxidatively modified DNA bases. In this review the most common methods quantifying the three parameters mentioned above, the cytokinesis-block micronucleus assay, the comet assay, and the quantification of 8-oxo-7,8-dihydro-2′-deoxyguanosine, are evaluated concerning the feasibility of the analysis and regarding the marker’s potential to predict clinical outcomes.
Background
Agalsidase beta is a form of enzyme replacement therapy for Fabry disease, a genetic disorder characterised by low alpha-galactosidase A activity, accumulation of glycosphingolipids and life-threatening cardiovascular, renal and cerebrovascular events. In clinical trials, agalsidase beta cleared glycolipid deposits from endothelial cells within 6 months; clearance from other cell types required sustained treatment. We hypothesised that there might be a 'lag time' to clinical benefit after initiating agalsidase beta treatment, and analysed the incidence of severe clinical events over time in patients receiving agalsidase beta.
Methods
The incidence of severe clinical events (renal failure, cardiac events, stroke, death) was studied in 1044 adult patients (641 men, 403 women) enrolled in the Fabry Registry who received agalsidase beta (average dose 1 mg/kg every 2 weeks) for up to 5 years.
Results
The incidence of all severe clinical events was 111 per 1000 person-years (95% CI 84 to 145) during the first 6 months. After 6 months, the incidence decreased and remained stable within the range of 40-58 events per 1000 patient-years. The largest decrease in incidence rates was among male patients and those aged >= 40 years when agalsidase beta was initiated.
Conclusions
Contrary to the expected increased incidence of severe clinical events with time, adult patients with Fabry disease had decreased incidence of severe clinical events after 6 months treatment with agalsidase beta 1 mg/kg every 2 weeks.