Stefan Störk

Learn More
OBJECTIVE The long-term effects of enzyme-replacement therapy (ERT) in Fabry disease are unknown. Thus, the aim of this study was to determine whether ERT in patients with advanced Fabry disease affects progression towards 'hard' clinical end-points in comparison with the natural course of the disease. METHODS A total of 40 patients with genetically(More)
BACKGROUND The Global initiative for chronic Obstructive Lung Disease (GOLD) defines COPD as a fixed post-bronchodilator ratio of forced expiratory volume in 1 second and forced vital capacity (FEV1/FVC) below 0.7. Age-dependent cut-off values below the lower fifth percentile (LLN) of this ratio derived from the general population have been proposed as an(More)
Friedreich's ataxia is a rare hereditary, predominantly neurologically defined multisystem disorder of mitochondrial function. Although the gene defect has been identified, the precise pathophysiology of the deficient mitochondrial protein, frataxin, is unknown. Besides the characteristic features of spinocerebellar ataxia the heart may also be affected,(More)
OBJECTIVE The aim of this study was to examine whether the physical and mental components of health-related quality of life (HRQoL) are independent predictors of survival in patients with chronic heart failure (CHF). METHODS A cohort of 231 outpatients with CHF was followed prospectively for 986 days (median; interquartile range=664-1120). Generic HRQoL(More)
Patients' health-related quality of life is increasingly being included as an additional endpoint when evaluating the treatment of chronic heart failure. Although generic self-report instruments measuring health-related quality of life are available, there is a lack of disease-specific instruments covering various dimensions of quality of life with high(More)
The widely shared enthusiasm about the cardioprotective potential of estrogenic compounds has come to an abrupt halt since randomized trials failed to show a cardiovascular risk reduction in postmenopausal women. This was unexpected because observational studies had strongly suggested that hormone replacement therapy would reduce the incidence of(More)
BACKGROUND In order to influence every day clinical practice professional organisations issue management guidelines. Cross-sectional surveys are used to evaluate the implementation of such guidelines. The present survey investigated screening for glucose perturbations in people with coronary artery disease and compared patients with known and newly detected(More)
OBJECTIVES The aim of this study was to explore the left ventricular (LV) deformation changes and the potential impact of deformation on outcome in patients with proven light-chain (AL) amyloidosis and LV hypertrophy. BACKGROUND Cardiac involvement in AL amyloidosis patients is associated with poor outcome. Detecting regional cardiac function by advanced(More)
For epidemiological research, the usage of standard electronic health records may be regarded a convenient way to obtain large amounts of medical data. Unfortunately, large parts of clinical reports are in written text form and cannot be used for statistical evaluations without appropriate preprocessing. This functionality is one of the main tasks in(More)
INTRODUCTION Experimental data indicates an important role of the innate immune system in cardiac remodeling and heart failure (HF). Complement is a central effector pathway of the innate immune system. Animals lacking parts of the complement system are protected from adverse remodeling. Based on these data, we hypothesized that peripheral complement levels(More)