leptin and Muscular-Dystrophy--Duchenne

To evaluate the potential bone defect in neuromuscular diseases, we conducted a longitudinal study including three groups of patients: 14 Duchenne muscular dystrophies (DMD) and 2 limb-girdle muscular dystrophies (LGMD); 3 Becker muscular dystrophies (BeMD) and 7 spinal muscular atrophies (SMA). Yearly osteodensitometries assessed body composition and bone mineral density (BMD) associated with bone markers and leptin. Along the 7-year study, 107 osteodensitometries showed that bone status evolved to osteopenia in most patients except BeMD. When analyzing the crude values, BMD improved with age in BeMD and SMA but not in DMD/LGMD. The correlation using the Z-scores displayed a decrease in BMD with age in DMD/LGMD for all regions, in SMA at total body less head, whereas BMD increased in BeMD at lumbar spine. As observed in healthy persons, muscular mass and bone tissue were significantly correlated. Glucocorticoids were deleterious on trabecular and cortical bone. Leptin was high in most patients and correlated to fat mass and bone parameters. This study confirms a secondary bone defect in neuromuscular diseases, further confirming the functional relationship between bone and muscle and arguing for regular bone follow-up in patients to prevent fracture risk. Adipose tissue seems to interfere with bone remodeling in neuromuscular diseases.

Topics: Absorptiometry, Photon; Adolescent; Adult; Bone Density; Bone Diseases, Metabolic; Child; Child, Preschool; Female; Humans; Leptin; Longitudinal Studies; Male; Muscular Atrophy, Spinal; Muscular Dystrophies, Limb-Girdle; Muscular Dystrophy, Duchenne; Young Adult

To determine whether patients with Duchenne/Becker muscular dystrophy (DMD/BMD) have components of metabolic syndrome (MetSy) and to evaluate whether leptin is associated with components of MetSy.. This study included 78 patients (nine, <6 years of age; 54, 6 to <16 years of age; and 15 patients, ≥16 years of age). Obesity and body fat mass were determined by waist circumference and dual-energy X-ray absorptiometry, respectively. A 12-h fasting blood sample was collected in the morning. Patients were categorized into four groups according to the number of criteria for MetSy: group 0: none; group 1: one; group 2: two and group 3: three or more criteria.. All age groups showed components of MetSy. The concentration of these components was significantly higher in patients ≥16 years old. The prevalence of hypertriglyceridemia was from ~37% to 46% in all age groups. The prevalence of MetSy was 7.1% for patients from 6 to <16 years of age and 24% for patients ≥16 years of age. Serum leptin levels increased significantly (P < 0.05) with age; the highest (13.43 ± 9.4 ng/ml) value was observed in patients >16 years of age. Total leptin was correlated with the number of patients with MetSy (r = 0.383; P = 0.001).. Components of MetSy are significant in patients with DMD/BMD. A high prevalence of hypertriglyceridemia was observed. Younger patients with DMD/BMD have risk factors for MetSy. Although leptin increased according to different degrees of MetSy, this relation disappeared when the body fat was corrected by leptin; therefore, the association could be caused by a common risk factor-fat.

Topics: Adolescent; Adult; Child; Female; Humans; Leptin; Male; Metabolic Syndrome; Muscular Dystrophy, Duchenne; Prevalence

This cross-sectional study examined bone mineral density, bone turnover, body composition and calciotropic hormones in 24 boys with Duchenne muscular dystrophy (DMD) (2.3-19.7 years), most of whom were being treated with prednisolone, and 24 age-matched healthy boys. Our study demonstrated lower bone mineral density in the DMD group for total body, spine, hip, heel and forearm measurements. These differences between DMD patients and controls increased with increasing age. Biochemical markers of both bone formation and resorption revealed reduced bone turnover in DMD patients. The fracture rate was not higher in DMD patients. The DMD group had low vitamin D levels but high leptin levels in comparison with the control group. Muscle strength correlated with bone mineral density assessed at the hip and heel in the DMD group. Interventions that increase bone formation should be considered, as DMD patients have reduced bone turnover in addition to their low bone mineral density.

Topics: Adolescent; Adult; Biomarkers; Bone and Bones; Bone Density; Bone Resorption; Calcium; Child; Child, Preschool; Cross-Sectional Studies; Diet; Glucocorticoids; Humans; Leptin; Male; Muscle Strength; Muscular Dystrophy, Duchenne; Nutrition Assessment; Nutritional Physiological Phenomena; Osteogenesis; Osteoporosis; Prednisolone; Vitamin D Deficiency