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Background Myotonic dystrophy type 2 (DM2) is a neuromuscular disorder characterized by myotonia and muscle weakness, with no medical treatment to prevent a decline in decline. It is unknown whether exercise training is effective in DM2. The aim of this study was to investigate the effect of exercise training on functional capacity and body composition in these patients. Methods Body composition and functional capacity were evaluated at the beginning (T1) and end (T2) of a 12 wk control period, and again after 16 wk of exercise training (T3) in 10 patients. Results No changes were recorded after the control period. Handgrip strength, 5× sit to stand, timed up and go, 6 min walk distance, lean body mass (LBM), and bone mineral density (BMD) increased while arterial pressure decreased after training. Conclusions These results suggest that supervised exercise training improves functional capacity, LBM, and BMD in ambulatory DM2 patients.

Pompe disease is a rare autosomal recessive disorder caused by the deficiency of acid α-glycosidase resulting in accumulation of glycogen in the lysosomes. The late-onset form of the disease (LOPD) causes primarily progressive muscle weakness and respiratory insufficiency. Enzyme replacement therapy (ERT) introduced in 2006, showed mild improvement or stabilization of the symptoms although long-term data are limited. Aim of the study was to describe the progression of body composition and walking ability in LOPD patients receiving ERT consistently for 9 years.Lean body mass, bone mineral density, body fat and 6 min walking distance were assessed in three male and three female LOPD patients (height 165.8 ± 11.2 cm, age 42.3 ± 11.8yrs, body mass 71.1 ± 20.8 kg, at study entry), every three years, for 9 years since ERT initiation (T0, T3, T6, T9).Total body and upper extremities' lean mass remained unchanged (The current data show that enzyme replacement therapy may preserve lean body mass, bone mineral density and walking capacity in LOPD patients.

The present study aimed to investigate the effect of different volumes of fast eccentric-based training on body composition and lipidemic-glycemic profiles in females, as well as to explore the relationship between the change in glycemic-lipidemic profiles and the change in muscle fibre composition. Twenty-nine young females were assigned into three groups and performed 10 weeks (2 training sessions per week) of either 3 (LV), 6 (MV) or 9 (HV) sets/session of four fast velocity eccentric-only half-squats against 70% of concentric 1RM, followed by 3 maximum countermovement jumps (CMJ) after each set. Body composition, vastus lateralis fibre-type composition, and resting blood lipidemic and glycemic indices were evaluated 1 week before and after the training intervention. Significant changes in body composition, fasting glucose, HOMA-IR and blood lipids were found after training with MV and HV (

Kontou, EI, Berberidou, FT, Pilianidis, TC, Mantzouranis, NI, and Methenitis, SK. Acute effect of upper and lower body postactivation exercises on shot put performance. J Strength Cond Res 32(4): 970-982, 2018-The purpose of this study was to investigate the effect of different types of upper and lower' extremities exercises on acute increase of shot put performance, in moderate experienced throwers. Eight (n = 8) males and 9 (n = 9) female throwers participated in this study. Their bench press and squat maximum strength were measured while their shot put performance from power position was evaluated before and after 4 interventions: (a) plyometric push-ups (Plyo), (b) 6 s isometric push-ups (Iso), (c) 3 countermovement jumps (CMJs) and (d) 10 reps. of skipping (Skip). Interventions were performed in counterbalanced order with a 48-hour interval. Significant increase (p < 0.05) of shot put performances was observed after Plyo, Iso, and CMJ (range: 2.30 ± 1.82%-5.72 ± 4.32%). In addition, Iso induced the highest increase while Skip did not induce any improvement of throwing performance. The highest increases were recorded in men's performance after CMJ (5.72 ± 4.32%) while in women's performance after Iso (3.59 ± 2.7%). Javelin and discus throwers increase higher their performance after CMJs while shot putters after Iso. Significant correlations were found between training experience, maximum/relative strength, shot put performance and increase of throwing performance (%) after the interventions (r: 0.519-0.991, p < 0.05). Percentage increase of performance between Iso and Plyo have negative correlations (r: -0.569, p < 0.05) in contrast of those between Skip and CMJ (r: 0.710, p < 0.05). These results suggest that upper or lower body postactivation interventions may acutely increase the throwing performance. However, experience and strength are significant determinant of this increase.