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<p>Metabolic myopathies impair muscle energy metabolism. Exercise is challenging, as patients may suffer from rhabdomyolysis attacks. This study investigated long-term effects of an individualized strength training program. Eighteen patients (12–45 years) with fatty acid oxidation disorders and glycogen storage diseases (types III and V) participated in a supervised progressive strength exercise program over two periods: biweekly assessments for 12 weeks and monthly in the second 12 weeks. Clinical assessments (anthropometry, food intake, laboratory tests), muscle strength/endurance (isokinetic tests), balance (postural stability tests), aerobic capacity (exercise duration, anaerobic threshold rate, maximal oxygen consumption), and quality of life (calculated in eight subscales) were assessed at baseline, 12, and 24 weeks. Biweekly nutritional and medical adjustments were made based on physiological responses, including modifications in caloric intake, macronutrient composition, pre-exercise sucrose and creatine supplementation. Significant improvements in strength (<i>p</i> &lt; 0.001), balance (<i>p</i> &lt; 0.05), and aerobic capacity (<i>p</i> &lt; 0.001) were observed after 12 weeks and persisted for 24 weeks. No patients experienced rhabdomyolysis. Quality of life improved, particularly in the subscales of emotional role limitations, pain, and general health (<i>p</i> &lt; 0.05). These findings suggest that dynamic, multidisciplinary, and individualized exercise program, combined with medical nutrition therapy and pharmacological adjustments, is effective and safe for patients with metabolic myopathies.</p>

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Impact of individualized and supervised strength training on muscle physiology, metabolic control and quality of life in metabolic myopathies

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年7月18日

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  • Tanyel ZUBARIOGLU,
  • Sertaç YAKAL,
  • Fatmanur YEGİN,
  • Esma UYGUR,
  • Türker ŞAHİNKAYA,
  • Şensu DİNÇER,
  • Mehmet Şerif CANSEVER,
  • Esra İŞAT,
  • Neslihan YILDIRIM SARAL,
  • Aykut OKUMUŞ,
  • Mehmet Güven GÜNVER,
  • Emine GENÇ,
  • Burcu ÖZTÜRK HİŞMİ,
  • Mehmet Cihan BALCI,
  • Ertuğrul KIYKIM,
  • Gökhan METİN,
  • Çiğdem AKTUĞLU ZEYBEK

摘要

Metabolic myopathies impair muscle energy metabolism. Exercise is challenging, as patients may suffer from rhabdomyolysis attacks. This study investigated long-term effects of an individualized strength training program. Eighteen patients (12–45 years) with fatty acid oxidation disorders and glycogen storage diseases (types III and V) participated in a supervised progressive strength exercise program over two periods: biweekly assessments for 12 weeks and monthly in the second 12 weeks. Clinical assessments (anthropometry, food intake, laboratory tests), muscle strength/endurance (isokinetic tests), balance (postural stability tests), aerobic capacity (exercise duration, anaerobic threshold rate, maximal oxygen consumption), and quality of life (calculated in eight subscales) were assessed at baseline, 12, and 24 weeks. Biweekly nutritional and medical adjustments were made based on physiological responses, including modifications in caloric intake, macronutrient composition, pre-exercise sucrose and creatine supplementation. Significant improvements in strength (p < 0.001), balance (p < 0.05), and aerobic capacity (p < 0.001) were observed after 12 weeks and persisted for 24 weeks. No patients experienced rhabdomyolysis. Quality of life improved, particularly in the subscales of emotional role limitations, pain, and general health (p < 0.05). These findings suggest that dynamic, multidisciplinary, and individualized exercise program, combined with medical nutrition therapy and pharmacological adjustments, is effective and safe for patients with metabolic myopathies.