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Schmidt, Anja (2012): Fettstoffwechselstörungen und Statinunverträglichkeit bei Myotonen Dystrophien. Dissertation, LMU München: Medizinische Fakultät



Lipid metabolism alteration and statin intake in myotonic dystrophies A. Schmidt, MS a; L. Kirzinger, MS a, W. Kress, PhD b, and B. Schoser, MD a a Friedrich-Baur Institute, Department of Neurology, Ludwig Maximilians University Munich, Munich, Germany b Institute of Human Genetics, University of Würzburg, Würzburg, Germany. Background: There have been no systematic reports on lipid metabolism alteration in myotonic dystrophies. We assessed the frequency, type, and severity of lipid alteration in both types of DM. Furthermore, the frequency of statin intake and side effects was investigated. Methods: A retrospective multicenter study was conducted. 134 DM2 patients and 29 DM1 patients participated by completing questionnaires. Additionally, their clinical and serological records, including blood lipid parameters for dyslipoproteinemia (cholesterol, HDL, LDL and TG) were screened and reviewed. Results: 71.6% of DM2 and 55.2% of DM1 patients show evidence to suffer from dyslipoproteinemia. In DM2 and DM1 patients the age of onset of dyslipoproteinemia was 47.6y±15.1 and 46.2y±9.7, respectively. Comparing both DM groups, all measured parameters did not differ statistically significant. Nevertheless, comparing all lipid parameters of both types of DM with an average German population data set, we could find a no significant discrepancy for DM1 patients (Cholesterol: p=0.435; HDL: p=0.129), but for DM2 patients (Cholesterol p=0.021; HDL: p=0.001). 23.1% of DM2 and 11.1% of DM1 patients were treated with statins. 50% of all patients discontinued statin therapy because of side effects. Conclusions: According to Robert-Koch-Institute, Germany, one third of German adults show elevated cholesterol levels, which is significantly lower than the rate found here in DM patients. With this high rate of dyslipoproteinemia in DMs, it seems to be obvious, that DM patients show a predisposition for this metabolic disorder. Furthermore, compared with data from Robert-Koch-Institute, DM patients (DM2 23.1%, DM1 11.1%) use significantly more statins than the German average. In addition, the rate of discontinuation is high based on side effects like augmented symptoms (hyperCKemia in 27.8% DM2, muscle pain or weakness in 33.3% DM2) in relation to former studies revealing statin side effects in one of 10000 patients.