Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients.

INTRODUCTION: Early detection of left ventricular systolic dysfunction (LVSD) is important for therapeutic strategies for Duchenne muscular dystrophy (DMD) patients. We analyzed myocardial strain using echocardiography for early detection of LVSD and determined the predictors of early LVSD. METHODS: This investigation was a cross-sectional study of 40 DMD patients with normal left ventricular ejection fraction. Global longitudinal strain (GLS) was used to analyze subtle disturbances in longitudinal contraction of the myocardium. Patients were determined to have early LVSD (GLS > -18) or normal left ventricular systolic function (GLS ≤ -18). RESULTS: Patients who had early LVSD were older and had a higher frequency of corticosteroid therapy and of mutations in exons 45, 46, 47, 48, 49, 50, and 52. DISCUSSION: Myocardial strain measurements are useful for the early diagnosis of LVSD in DMD patients. Older age, use of corticosteroids, and mutations within the "hot-spot" region of the DMD gene are associated with early LVSD. Muscle Nerve, 2018.

Evaluation of Left-Sided Heart Chambers With Novel Echocardiographic Techniques in Men With Duchenne or Becker Muscular Dystrophy.

Left ventricular systolic dysfunction (LVSD) is a common finding in patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies. Novel echocardiographic techniques have been used for the detection of LVSD in several heart diseases. We aim to compare cardiac anatomic and functional data studied by three-dimensional (3DE) and two-dimensional (2DE) echocardiography and to analyze the myocardial strain for the detection of early LVSD in DMD and BMD patients. We performed a cross-sectional study of 46 DMD and 14 BMD patients. We measured left atrium volume and left ventricle volumes and ejection fraction using 3DE and 2DE techniques. Myocardial strain analysis was derived from global longitudinal strain (GLS) measurements. GLS was measured by 2DE with the speckle tracking technique. The correlation between 3DE and 2DE for the measurement of left atrium volume as well as left ventricle diastolic and systolic volumes was strong. 2DE presented larger left atrium and left ventricle volumes. Left ventricle ejection fraction was similar between the two techniques. Myocardial strain analysis was able to detect early LVSD in 50.0% of DMD patients and in 9.1% of BMD patients. In conclusion, two-dimensional echocardiography appears to be a good alternative for the anatomical and functional evaluation of the left heart chambers in DMD and BMD patients. Myocardial strain analysis detects early LVSD in a sizable portion of patients with dystrophinopathies.

The importance of lactic acid in migraines and fibromyalgia.

BACKGROUND: Lactic acid is a byproduct of both muscle metabolism and the central nervous system. Changes in metabolism are related to various physiological and pathological conditions. The aim of this study was to determine the relationship between migraine and fibromyalgia with the levels of lactic acid in the blood. METHODS: We study of 93 patients was divided into five groups: 1) patients with fibromyalgia (n=20); 2) episodic migraine (n=20); 3) chronic migraine (n=20); 4) fibromyalgia and episodic migraine (n= 13); and 5) fibromyalgia and chronic migraine (n=20), and 20 healthy subjects (control group). Blood levels of lactic acid were measured at four different time points: at rest, during aerobic exercise, during anaerobic physical activity and while resting after anaerobic exercise. RESULTS: Lactic acid increased in all groups during anaerobic physical activity without predominance for either group. During aerobic physical activity, all groups increased lactic acid levels, but the increase was more expressive in the chronic migraine group and the chronic migraine with fibromyalgia group without statistical significance. CONCLUSIONS: We did not found abnormalities involving the metabolism of lactic acid in episodic and chronic migraine with or without fibromyalgia.

Validation of the friedewald formula in patients with metabolic syndrome.

Currently, the Friedewald formula (FF) is the main method for evaluating low-density lipoprotein cholesterol (LDL-c). Recently, many limitations have emerged regarding its use, including patients with triglyceride levels ≥400 mg/dL, diabetes mellitus, and kidney or hepatic chronic diseases. We analyzed the use of the FF in patients with metabolic syndrome. We selected patients with known metabolic syndrome that fulfilled the National Cholesterol Education Program Expert Panel on Detection, Evaluation and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III) Final Report and excluded patients with triglyceride levels ≥400 mg/dL and chronic liver and/or kidney disease. Using direct assays, we measured total cholesterol, high-density lipoprotein cholesterol, triglycerides, and LDL-c. Then, LDL-c was estimated using the FF and compared with the LDL-c by direct assay. The sample size was 135 patients. Using the FF, the mean LDL-c value was 124.4 ± 42.1 mg/dL; it was 125.1 ± 38.5 mg/dL by direct assay. The correlation coefficient between these two methods was 0.89, with statistical significance (P value < 0.001). There were no significant differences between the patients with triglyceride levels >150 mg/dL (P = 0.618). In conclusion, FF is a good method for estimating LDL-c in patients with metabolic syndrome.