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Feasibility of resistance training in adult McArdle patients: clinical outcomes and muscle strength and mass benefits.

Santalla A, Munguía-Izquierdo D, Brea-Alejo L, Pagola-Aldazábal I, Díez-Bermejo J, Fleck SJ, Ara I, Lucia A - Front Aging Neurosci (2014)

Bottom Line: The training program had a significant impact on total and lower extremities' lean mass (P < 0.05 for the time effect), with mean values increasing with training by +855 g (95% confidence interval (CI): 30, 1679) and +547 g (95%CI: 116, 978), respectively, and significantly decreasing with detraining.Yet muscle strength and power detraining values were significantly higher than pre-training values, indicating that a training effect was still present after detraining.No significant time effect (P > 0.05) was noted for baseline or post strength assessment values of serum CK activity, which remained essentially within the range reported in our laboratory for McArdle patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Sports Sciences, Universidad Pablo de Olavide Seville, Spain ; Research Institute "i+12", Hospital 12 de Octubre Madrid, Spain.

ABSTRACT
We analyzed the effects of a 4-month resistance (weight lifting) training program followed by a 2-month detraining period in 7 adult McArdle patients (5 female) on: muscle mass (assessed by DXA), strength, serum creatine kinase (CK) activity and clinical severity. Adherence to training was ≥84% in all patients and no major contraindication or side effect was noted during the training or strength assessment sessions. The training program had a significant impact on total and lower extremities' lean mass (P < 0.05 for the time effect), with mean values increasing with training by +855 g (95% confidence interval (CI): 30, 1679) and +547 g (95%CI: 116, 978), respectively, and significantly decreasing with detraining. Body fat showed no significant changes over the study period. Bench press and half-squat performance, expressed as the highest value of average muscle power (W) or force (N) in the concentric-repetition phase of both tests showed a consistent increase over the 4-month training period, and decreased with detraining. Yet muscle strength and power detraining values were significantly higher than pre-training values, indicating that a training effect was still present after detraining. Importantly, all the participants, with no exception, showed a clear gain in muscle strength after the 4-month training period, e.g., bench press: +52 W (95% CI: 13, 91); half-squat: +173 W (95% CI: 96, 251). No significant time effect (P > 0.05) was noted for baseline or post strength assessment values of serum CK activity, which remained essentially within the range reported in our laboratory for McArdle patients. All the patients changed to a lower severity class with training, such that none of them were in the highest disease severity class (3) after the intervention and, as such, they did not have fixed muscle weakness after training. Clinical improvements were retained, in all but one patient, after detraining, such that after detraining all patients were classed as class 1 for disease severity.

No MeSH data available.


Related in: MedlinePlus

Patients’ individual scores of disease severity using the scale by Martinuzzi et al (Martinuzzi et al., 2003): “class 0 = asymptomatic or virtually asymptomatic (mild exercise intolerance, but no functional limitation in any daily life activity); 1 = exercise intolerance, contractures, myalgia, and limitation of acute strenuous exercise, and occasionally in daily life activities; no record of myoglobinuria, no muscle wasting or weakness; 2 = same as 1, plus recurrent exertional myoglobinuria, moderate restriction in exercise, and limitation in daily life activities; 3 = same as 2, plus fixed muscle weakness, with or without wasting and severe limitations on exercise and most daily life activities”.
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Figure 6: Patients’ individual scores of disease severity using the scale by Martinuzzi et al (Martinuzzi et al., 2003): “class 0 = asymptomatic or virtually asymptomatic (mild exercise intolerance, but no functional limitation in any daily life activity); 1 = exercise intolerance, contractures, myalgia, and limitation of acute strenuous exercise, and occasionally in daily life activities; no record of myoglobinuria, no muscle wasting or weakness; 2 = same as 1, plus recurrent exertional myoglobinuria, moderate restriction in exercise, and limitation in daily life activities; 3 = same as 2, plus fixed muscle weakness, with or without wasting and severe limitations on exercise and most daily life activities”.

Mentions: The clinical course of the disease is shown in Figure 6. All patients changed to a lower severity class with training, such that none of them belonged to the highest disease severity category (class 3) after training and, as such, did not have fixed muscle weakness anymore. Further, 2 patients moved to class 0, which is essentially symptom-free. Most of the clinical improvements (except for one patient) were retained at detraining, that is, all patients were classed as class 1 for disease severity.


Feasibility of resistance training in adult McArdle patients: clinical outcomes and muscle strength and mass benefits.

Santalla A, Munguía-Izquierdo D, Brea-Alejo L, Pagola-Aldazábal I, Díez-Bermejo J, Fleck SJ, Ara I, Lucia A - Front Aging Neurosci (2014)

Patients’ individual scores of disease severity using the scale by Martinuzzi et al (Martinuzzi et al., 2003): “class 0 = asymptomatic or virtually asymptomatic (mild exercise intolerance, but no functional limitation in any daily life activity); 1 = exercise intolerance, contractures, myalgia, and limitation of acute strenuous exercise, and occasionally in daily life activities; no record of myoglobinuria, no muscle wasting or weakness; 2 = same as 1, plus recurrent exertional myoglobinuria, moderate restriction in exercise, and limitation in daily life activities; 3 = same as 2, plus fixed muscle weakness, with or without wasting and severe limitations on exercise and most daily life activities”.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4263173&req=5

Figure 6: Patients’ individual scores of disease severity using the scale by Martinuzzi et al (Martinuzzi et al., 2003): “class 0 = asymptomatic or virtually asymptomatic (mild exercise intolerance, but no functional limitation in any daily life activity); 1 = exercise intolerance, contractures, myalgia, and limitation of acute strenuous exercise, and occasionally in daily life activities; no record of myoglobinuria, no muscle wasting or weakness; 2 = same as 1, plus recurrent exertional myoglobinuria, moderate restriction in exercise, and limitation in daily life activities; 3 = same as 2, plus fixed muscle weakness, with or without wasting and severe limitations on exercise and most daily life activities”.
Mentions: The clinical course of the disease is shown in Figure 6. All patients changed to a lower severity class with training, such that none of them belonged to the highest disease severity category (class 3) after training and, as such, did not have fixed muscle weakness anymore. Further, 2 patients moved to class 0, which is essentially symptom-free. Most of the clinical improvements (except for one patient) were retained at detraining, that is, all patients were classed as class 1 for disease severity.

Bottom Line: The training program had a significant impact on total and lower extremities' lean mass (P < 0.05 for the time effect), with mean values increasing with training by +855 g (95% confidence interval (CI): 30, 1679) and +547 g (95%CI: 116, 978), respectively, and significantly decreasing with detraining.Yet muscle strength and power detraining values were significantly higher than pre-training values, indicating that a training effect was still present after detraining.No significant time effect (P > 0.05) was noted for baseline or post strength assessment values of serum CK activity, which remained essentially within the range reported in our laboratory for McArdle patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Sports Sciences, Universidad Pablo de Olavide Seville, Spain ; Research Institute "i+12", Hospital 12 de Octubre Madrid, Spain.

ABSTRACT
We analyzed the effects of a 4-month resistance (weight lifting) training program followed by a 2-month detraining period in 7 adult McArdle patients (5 female) on: muscle mass (assessed by DXA), strength, serum creatine kinase (CK) activity and clinical severity. Adherence to training was ≥84% in all patients and no major contraindication or side effect was noted during the training or strength assessment sessions. The training program had a significant impact on total and lower extremities' lean mass (P < 0.05 for the time effect), with mean values increasing with training by +855 g (95% confidence interval (CI): 30, 1679) and +547 g (95%CI: 116, 978), respectively, and significantly decreasing with detraining. Body fat showed no significant changes over the study period. Bench press and half-squat performance, expressed as the highest value of average muscle power (W) or force (N) in the concentric-repetition phase of both tests showed a consistent increase over the 4-month training period, and decreased with detraining. Yet muscle strength and power detraining values were significantly higher than pre-training values, indicating that a training effect was still present after detraining. Importantly, all the participants, with no exception, showed a clear gain in muscle strength after the 4-month training period, e.g., bench press: +52 W (95% CI: 13, 91); half-squat: +173 W (95% CI: 96, 251). No significant time effect (P > 0.05) was noted for baseline or post strength assessment values of serum CK activity, which remained essentially within the range reported in our laboratory for McArdle patients. All the patients changed to a lower severity class with training, such that none of them were in the highest disease severity class (3) after the intervention and, as such, they did not have fixed muscle weakness after training. Clinical improvements were retained, in all but one patient, after detraining, such that after detraining all patients were classed as class 1 for disease severity.

No MeSH data available.


Related in: MedlinePlus