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Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining.
Mol Genet Metab 2016; 117(2):120-8MG

Abstract

BACKGROUND

Determine the effects of a 12-week respiratory muscle training (RMT) program in late-onset Pompe disease (LOPD).

METHODS

We investigated the effects of 12-weeks of RMT followed by 3-months detraining using a single-subject A-B-A experimental design replicated across 8 adults with LOPD. To assess maximal volitional respiratory strength, our primary outcomes were maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP). Effect sizes for changes in MIP and MEP were determined using Cohen's d statistic. Exploratory outcomes targeted motor function, and peak cough flow (PCF) was measured in the last 5 subjects.

RESULTS

From pretest to posttest, all 8 subjects exhibited increases in MIP, and 7 of 8 showed increases in MEP. Effect size data reveal the magnitude of increases in MIP to be large in 4 (d≥1.0) and very large in 4 (d≥2.0), and effect sizes for increases in MEP were large in 1 (d≥1.0) and very large in 6 (d≥2.0). Across participants, pretest to posttest MIP and MEP increased by a mean of 19.6% (sd=9.9) and 16.1% (sd=17.3), respectively. Respiratory strength increases, particularly for the inspiratory muscles, were generally durable to 3-months detraining.

CONCLUSIONS

These data suggest our 12-week RMT program results in large to very large increases in inspiratory and expiratory muscle strength in adults with LOPD. Additionally, increases in respiratory strength appeared to be relatively durable following 3-months detraining. Although additional research is needed, RMT appears to offer promise as an adjunctive treatment for respiratory weakness in LOPD.

Authors+Show Affiliations

Department of Surgery, Division of Head and Neck Surgery & Communication Sciences, Duke University, Durham, NC, USA. Electronic address: harrison.jones@duke.edu.Department of Speech Pathology & Audiology, Duke University, Durham, NC, USA. Electronic address: kelly.crisp@duke.edu.Communication Disorders, University of Virginia, Charlottesville, VA, USA. Electronic address: rrr7w@eservices.virginia.edu.Physical Therapy Division, Duke University, Durham, NC, USA. Electronic address: laura.case@duke.edu.Division of Pediatric Pulmonary and Sleep Medicine, Duke University, Durham, NC, USA. Electronic address: richard.kravitz@duke.edu.Department of Pediatrics, Division of Medical Genetics, Duke University, Durham, NC, USA. Electronic address: priya.kishnani@duke.edu.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

26381077

Citation

Jones, Harrison N., et al. "Respiratory Muscle Training (RMT) in Late-onset Pompe Disease (LOPD): Effects of Training and Detraining." Molecular Genetics and Metabolism, vol. 117, no. 2, 2016, pp. 120-8.
Jones HN, Crisp KD, Robey RR, et al. Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining. Mol Genet Metab. 2016;117(2):120-8.
Jones, H. N., Crisp, K. D., Robey, R. R., Case, L. E., Kravitz, R. M., & Kishnani, P. S. (2016). Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining. Molecular Genetics and Metabolism, 117(2), pp. 120-8. doi:10.1016/j.ymgme.2015.09.003.
Jones HN, et al. Respiratory Muscle Training (RMT) in Late-onset Pompe Disease (LOPD): Effects of Training and Detraining. Mol Genet Metab. 2016;117(2):120-8. PubMed PMID: 26381077.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining. AU - Jones,Harrison N, AU - Crisp,Kelly D, AU - Robey,Randall R, AU - Case,Laura E, AU - Kravitz,Richard M, AU - Kishnani,Priya S, Y1 - 2015/09/08/ PY - 2015/08/06/received PY - 2015/09/01/revised PY - 2015/09/01/accepted PY - 2015/9/19/entrez PY - 2015/9/19/pubmed PY - 2016/11/4/medline KW - Breathing exercises KW - Exercise KW - Glycogen storage disease type II KW - Rehabilitation KW - Skeletal muscle SP - 120 EP - 8 JF - Molecular genetics and metabolism JO - Mol. Genet. Metab. VL - 117 IS - 2 N2 - BACKGROUND: Determine the effects of a 12-week respiratory muscle training (RMT) program in late-onset Pompe disease (LOPD). METHODS: We investigated the effects of 12-weeks of RMT followed by 3-months detraining using a single-subject A-B-A experimental design replicated across 8 adults with LOPD. To assess maximal volitional respiratory strength, our primary outcomes were maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP). Effect sizes for changes in MIP and MEP were determined using Cohen's d statistic. Exploratory outcomes targeted motor function, and peak cough flow (PCF) was measured in the last 5 subjects. RESULTS: From pretest to posttest, all 8 subjects exhibited increases in MIP, and 7 of 8 showed increases in MEP. Effect size data reveal the magnitude of increases in MIP to be large in 4 (d≥1.0) and very large in 4 (d≥2.0), and effect sizes for increases in MEP were large in 1 (d≥1.0) and very large in 6 (d≥2.0). Across participants, pretest to posttest MIP and MEP increased by a mean of 19.6% (sd=9.9) and 16.1% (sd=17.3), respectively. Respiratory strength increases, particularly for the inspiratory muscles, were generally durable to 3-months detraining. CONCLUSIONS: These data suggest our 12-week RMT program results in large to very large increases in inspiratory and expiratory muscle strength in adults with LOPD. Additionally, increases in respiratory strength appeared to be relatively durable following 3-months detraining. Although additional research is needed, RMT appears to offer promise as an adjunctive treatment for respiratory weakness in LOPD. SN - 1096-7206 UR - https://www.unboundmedicine.com/medline/citation/26381077/Respiratory_muscle_training__RMT__in_late_onset_Pompe_disease__LOPD_:_Effects_of_training_and_detraining_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1096-7192(15)30049-4 DB - PRIME DP - Unbound Medicine ER -