Exercise training alone or in combination with high-protein diet in patients with late onset Pompe disease: results of a cross over study.
Journalorphanet journal of rare diseases3.523Date
2020 Jun 06
5 months ago
Research Support, Non-U.S. Gov't
Journal Article
2020-06-06 / 15 : 143
Sechi A 1, Zuccarelli L 2, Grassi B 2, Frangiamore R 3, De Amicis R 4, Marzorati M 5, Porcelli S 5, Tullio A 6, Bacco A 7, Bertoli S 4, Dardis A 8, Biasutti L 2, Pasanisi MB 3, Devigili G 9, Bembi B 8
  • 2. Department of Medicine, University of Udine, Udine, Italy.
  • 3. Neuroimmunology and Muscle Pathology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
  • 4. International Center for the Assessment of Nutritional Status (ICANS), Department of Food Environmental and Nutritional Sciences (DeFENS), University of Milan, Milan, Italy.
  • 5. Institute of Biomedical Technologies, National Research Council, Segrate, Italy.
  • 6. Institute of Hygiene and Clinical Epidemiology, Academic hospital of Udine, Udine, Italy.
  • 7. Division of Endocrinology, Metabolic Diseases and Nutrition, Academic Hospital of Udine, Udine, Italy.
  • 8. Regional Coordinator Center for Rare Diseases, Academic hospital of Udine, p.zzale SM della Misericordia 15, 33100, Udine, Italy.
  • 9. Neurological Unit 1, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
BACKGROUND: Late onset Pompe disease (LOPD) is a lysosomal neuromuscular disorder which can progressively impair the patients' exercise tolerance, motor and respiratory functions, and quality of life. The available enzyme replacement therapy (ERT) does not completely counteract disease progression. We investigated the effect of exercise training alone, or associated with a high-protein diet, on the exercise tolerance, muscle and pulmonary functions, and quality of life of LOPD patients on long term ERT.
METHODS: The patients were asked to participate to a crossover randomized study comprehending a control period (free diet, no exercise) followed by 2 intervention periods: exercise or exercise + diet, each lasting 26 weeks and separated by 13 weeks washout periods. Exercise training included moderate-intensity aerobic exercise on a cycle ergometer, stretching and balance exercises, strength training. The diet was composed by 25-30% protein, 30-35% carbohydrate and 35-40% fat. Before and after each period patients were assessed for: exercise tolerance test on a cycle-ergometer, serum muscle enzymes, pulmonary function tests and SF36 questionnaire for quality of life. Compliance was evaluated by training and dietary diaries. Patients were contacted weekly by researchers to optimize adherence to treatments.
RESULTS: Thirteen LOPD patients, median age 49 ± 11 years, under chronic ERT (median 6.0 ± 4.0 years) were recruited. Peak aerobic power (peak pulmonary O2 uptake) decreased after control, whereas it increased after exercise, and more markedlyafter exercise + diet. Serum levels of lactate dehydrogenase (LDH) significantly decreased after exercise + diet; both creatine kinase (CK) and LDH levels were significantly reduced after exercise + diet compared to exercise. Pulmonary function showed no changes after control and exercise, whereas a significant improvement of forced expiratory volume in 1 sec (FEV1) was observed after exercise + diet. SF36 showed a slight improvement in the "mental component" scale after exercise, and a significant improvement in "general health" and "vitality" after exercise + diet. The compliance to prescriptions was higher than 70% for both diet and exercise.
CONCLUSIONS: Exercise tolerance (as evaluated by peak aerobic power) showed a tendency to decrease in LOPD patients on long term ERT. Exercise training, particularly if combined with high-protein diet, could reverse this decrease and result in an improvement, which was accompanied by improved quality of life. The association of the two lifestyle interventions resulted also in a reduction of muscle enzyme levels and improved pulmonary function.
Keywords: Enzyme replacement therapy Exercise tolerance Exercise training High-protein diet Pompe disease
Orphanet J Rare Disorphanet journal of rare diseases

No Data

© 2017 - 2020 Medicgo
Powered by some medical students