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Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.

Publication ,  Journal Article
Crisp, KD; Case, LE; Kravitz, RM; Kishnani, PS; Jones, HN
Published in: J Pediatr Rehabil Med
2020

BACKGROUND: Respiratory muscle weakness is a primary cause of morbidity and mortality in patients with Pompe disease. We previously described the effects of our 12-week respiratory muscle training (RMT) regimen in 8 adults with late-onset Pompe disease [1] and 2 children with infantile-onset Pompe disease [2]. CASE REPORT: Here we describe repeat enrollment by one of the pediatric participants who completed a second 12-week RMT regimen after 7 months of detraining. We investigated the effects of two 12-week RMT regimens (RMT #1, RMT #2) using a single-participant A-B-A experimental design. Primary outcome measures 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. RELEVANCE: From pretest to posttest, RMT #2 was associated with a 25% increase in MIP and a 22% increase in MEP, corresponding with very large effect sizes (d= 2.92 and d= 2.65, respectively). Following two 12-week RMT regimens over 16 months, MIP increased by 69% and MEP increased by 97%, corresponding with very large effect sizes (d= 3.57 and d= 5.10, respectively). MIP and MEP were largely stable over 7 months of detraining between regimens. Magnitude of change was greater for RMT #1 relative to RMT #2.

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Published In

J Pediatr Rehabil Med

DOI

EISSN

1875-8894

Publication Date

2020

Volume

13

Issue

1

Start / End Page

71 / 80

Location

Netherlands

Related Subject Headings

  • Treatment Outcome
  • Retreatment
  • Respiratory Muscles
  • Respiratory Insufficiency
  • Muscle Strength
  • Infant
  • Humans
  • Glycogen Storage Disease Type II
  • Female
  • Breathing Exercises
 

Citation

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Crisp, K. D., Case, L. E., Kravitz, R. M., Kishnani, P. S., & Jones, H. N. (2020). Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease. J Pediatr Rehabil Med, 13(1), 71–80. https://doi.org/10.3233/PRM-190601
Crisp, Kelly D., Laura E. Case, Richard M. Kravitz, Priya S. Kishnani, and Harrison N. Jones. “Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.J Pediatr Rehabil Med 13, no. 1 (2020): 71–80. https://doi.org/10.3233/PRM-190601.
Crisp KD, Case LE, Kravitz RM, Kishnani PS, Jones HN. Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease. J Pediatr Rehabil Med. 2020;13(1):71–80.
Crisp, Kelly D., et al. “Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.J Pediatr Rehabil Med, vol. 13, no. 1, 2020, pp. 71–80. Pubmed, doi:10.3233/PRM-190601.
Crisp KD, Case LE, Kravitz RM, Kishnani PS, Jones HN. Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease. J Pediatr Rehabil Med. 2020;13(1):71–80.

Published In

J Pediatr Rehabil Med

DOI

EISSN

1875-8894

Publication Date

2020

Volume

13

Issue

1

Start / End Page

71 / 80

Location

Netherlands

Related Subject Headings

  • Treatment Outcome
  • Retreatment
  • Respiratory Muscles
  • Respiratory Insufficiency
  • Muscle Strength
  • Infant
  • Humans
  • Glycogen Storage Disease Type II
  • Female
  • Breathing Exercises