Respiratory muscle training in late-onset Pompe disease: Results of a sham-controlled clinical trial.

ABSTRACT: To address progressive respiratory muscle weakness in late-onset Pompe disease (LOPD), we developed a 12-week respiratory muscle training (RMT) program. In this exploratory, double-blind, randomized control trial, 22 adults with LOPD were randomized to RMT or sham-RMT. The primary outcome was maximum inspiratory pressure (MIP). Secondary and exploratory outcomes included maximum expiratory pressure (MEP), peak cough flow, diaphragm ultrasound, polysomnography, patient-reported outcomes, and measures of gross motor function. MIP increased 7.6 cmH₂O (15.9) in the treatment group and 2.7 cmH₂O (7.6) in the control group (P = 0.4670). MEP increased 14.0 cmH₂O (25.9) in the treatment group and 0.0 cmH₂O (12.0) in the control group (P = 0.1854). The on

SUBMITTER: Jones HN 

PROVIDER: S-EPMC7680435 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature