ABSTRACT
Exercise intolerance due to impaired oxidative metabolism is a prominent symptom in patients with mitochondrial myopathy (MM), but it is still uncertain whether L-carnitine supplementation is beneficial for patients with MM. The aim of our study was to investigate the effects of L-carnitine on exercise performance in MM. Twelve MM subjects (mean age±SD=35.4±10.8 years) with chronic progressive external ophthalmoplegia (CPEO) were first compared to 10 healthy controls (mean age±SD=29±7.8 years) before they were randomly assigned to receive L-carnitine supplementation (3 g/daily) or placebo in a double-blind crossover design. Clinical status, body composition, respiratory function tests, peripheral muscle strength (isokinetic and isometric torque) and cardiopulmonary exercise tests (incremental to peak exercise and at 70% of maximal), constant work rate (CWR) exercise test, to the limit of tolerance [Tlim]) were assessed after 2 months of L-carnitine/placebo administration. Patients with MM presented with lower mean height, total body weight, fat-free mass, and peripheral muscle strength compared to controls in the pre-test evaluation. After L-carnitine supplementation, the patients with MM significantly improved their Tlim (14±1.9 vs 11±1.4 min) and oxygen consumption ( V ˙ O 2 ) at CWR exercise, both at isotime (1151±115 vs 1049±104 mL/min) and at Tlim (1223±114 vs 1060±108 mL/min). These results indicate that L-carnitine supplementation may improve aerobic capacity and exercise tolerance during high-intensity CWRs in MM patients with CPEO.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , Carnitine/therapeutic use , Exercise Tolerance/drug effects , Ophthalmoplegia, Chronic Progressive External/drug therapy , Vitamin B Complex/therapeutic use , Cross-Over Studies , Double-Blind Method , Exercise Test/drug effects , Lactic Acid/blood , Mitochondrial Myopathies/drug therapy , Muscle Strength/drug effects , Oxidative Phosphorylation/drug effects , Oxygen Consumption/drug effects , Oxygen Consumption/physiology , SpirometryABSTRACT
We determined the response characteristics and functional correlates of the dynamic relationship between the rate (Δ) of oxygen consumption ( VO2) and the applied power output (work rate = WR) during ramp-incremental exercise in patients with mitochondrial myopathy (MM). Fourteen patients (7 males, age 35.4 ± 10.8 years) with biopsy-proven MM and 10 sedentary controls (6 males, age 29.0 ± 7.8 years) took a ramp-incremental cycle ergometer test for the determination of the VO2 on-exercise mean response time (MRT) and the gas exchange threshold (GET). The ΔVO2/ΔWR slope was calculated up to GET (S1), above GET (S2) and over the entire linear portion of the response (S T). Knee muscle endurance was measured by isokinetic dynamometry. As expected, peak VO2 and muscle performance were lower in patients than controls (P < 0.05). Patients had significantly lower ΔVO2/ΔWR than controls, especially the S2 component (6.8 ± 1.5 vs 10.3 ± 0.6 mL·min-1·W-1, respectively; P < 0.001). There were significant relationships between ΔVO2/ΔWR (S T) and muscle endurance, MRT-VO2, GET and peak VO2 in MM patients (P < 0.05). In fact, all patients with ΔVO2/ΔWR below 8 mL·min-1·W-1 had severely reduced peak VO2 values (<60 percent predicted). Moreover, patients with higher cardiopulmonary stresses during exercise (e.g., higher Δ ventilation/carbon dioxide output and Δ heart rate/ΔVO2) had lower ΔVO2/ΔWR (P < 0.05). In conclusion, a readily available, effort-independent index of aerobic dysfunction during dynamic exercise (ΔVO2/ΔWR) is typically reduced in patients with MM, being related to increased functional impairment and higher cardiopulmonary stress.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Exercise Test/methods , Mitochondrial Myopathies/physiopathology , Oxygen Consumption/physiology , Architectural Accessibility , Case-Control Studies , Exercise Tolerance/physiology , Heart Rate/physiology , Mitochondrial Myopathies/metabolism , Pulmonary Gas Exchange/physiology , Respiratory Function TestsABSTRACT
Foi realizada cineradioscopia com intensificador de imagem em 19 pacientes portadores de protese de Omni Science, valvula dotada de disco radiopaco que permite facil visibilizacao na radioscopia. Para determinar a sensibilidade e a especificidade do metodo foram estudados 16 pacientes assintomaticos e 3 com suspeita clinica de trombose de protese. Nos assintomaticos, visibilizou-se o disco em todos.Nos outros nao foi visto o disco em 2 casos e, no terceiro, a excursao estava bastante prejudicada. Conclui-se que a radioscopia e um importante auxiliar nao invasivo na avaliacao deste tipo de protese metalica