scholarly journals Rehabilitation management of Pompe disease, from childhood trough adulthood: A systematic review of the literature

2019 ◽  
Vol 11 (2) ◽  
Author(s):  
Bruno Corrado ◽  
Gianluca Ciardi ◽  
Clemente Servodio Iammarrone

Pompe disease (PD) is a rare neuromuscular disorder caused by a deficiency of the enzyme acid alpha-glucosidase. There are three forms of PD depending on the age at onset and clinical severity. PD causes involvement of different organ systems, such as the heart, musculoskeletal system, and respiratory system. As of today, enzymere placement therapy represents the main therapeutic tool for PD. Rehabilitation is an integral part of a multidisciplinary approach to this pathology. The goal of the present review is to find scientific evidence for the rehabilitative approach to PD, with respect to both the infantile- and adult-onset forms. A systematic literature review was made using the following databases: Pubmed, Pedro, Cochrane Library, EDS Base Index, Trip, and Cinhal. Randomized controlled trials or cohort studies with a sample population of at least six subjects were retrieved. The PICO method was used to formulate the clinical query. The search resulted in 1665 articles. Of these, four cohort studies were subjected to the final phase of the review. Three studies regarded inspiratory muscle training with a threshold, while the fourth study analyzed the effectiveness of therapeutic, aerobic, and reinforcement exercises. Inspiratory muscle training with a threshold increases the pressures generated during inhalation. Aerobic exercise is capable of increasing patients’ muscular endurance and performance. To date, however, rehabilitative treatment for patients with PD has no validation in evidence-based medicine. Further studies, possibly with a larger sample size and higher quality are necessary to confirm the effectiveness of rehabilitation in patients with PD.

2020 ◽  
Author(s):  
James Manifield ◽  
Andrew Winnard ◽  
Emily Hume ◽  
Matthew Armstrong ◽  
Katherine Baker ◽  
...  

Abstract Background The ageing process can result in the decrease of respiratory muscle strength and consequently increased work of breathing and associated breathlessness during activities of daily living in older adults. Objective This systematic review and meta-analysis aims to determine the effects of inspiratory muscle training (IMT) in healthy older adults. Methods A systematic literature search was conducted across four databases (Medline/Pubmed, Web of Science, Cochrane Library CINAHL) using a search strategy consisting of both MeSH and text words including older adults, IMT and functional capacity. The eligibility criteria for selecting studies involved controlled trials investigating IMT via resistive or threshold loading in older adults (>60 years) without a long-term condition. Results Seven studies provided mean change scores for inspiratory muscle pressure and three studies for functional capacity. A significant improvement was found for maximal inspiratory pressure (PImax) following training (n = 7, 3.03 [2.44, 3.61], P = <0.00001) but not for functional capacity (n = 3, 2.42 [−1.28, 6.12], P = 0.20). There was no significant correlation between baseline PImax and post-intervention change in PImax values (n = 7, r = 0.342, P = 0.453). Conclusions IMT can be beneficial in terms of improving inspiratory muscle strength in older adults regardless of their initial degree of inspiratory muscle weakness. Further research is required to investigate the effect of IMT on functional capacity and quality of life in older adults.


2018 ◽  
Vol 266 (1) ◽  
pp. 133-147 ◽  
Author(s):  
Stephan Wenninger ◽  
Eva Greckl ◽  
Haris Babačić ◽  
Kristina Stahl ◽  
Benedikt Schoser

Lung ◽  
2016 ◽  
Vol 194 (4) ◽  
pp. 555-561 ◽  
Author(s):  
Goksen Kuran Aslan ◽  
Burcu Ersoz Huseyinsinoglu ◽  
Piraye Oflazer ◽  
Nilgun Gurses ◽  
Esen Kiyan

Author(s):  
Diego Fernández-Lázaro ◽  
David Gallego-Gallego ◽  
Luis A Corchete ◽  
Darío Fernández Zoppino ◽  
Jerónimo J González-Bernal ◽  
...  

This systematic review and meta-analysis aim to provide scientific evidence regarding the effects of training on respiratory muscle training’s impact with the PowerBreath®. A systematic analysis based on the PRISMA guides and a conducted research structured around the bases of Web of Science, Scopus, Medline/PubMed, SciELO y Cochrane Library Plus. Six articles published before January 2021 were included. The documentation and quantification of heterogeneity in every meta-analysis were directed through Cochran’s Q test and the statistic I2; additionally, a biased publication analysis was made using funnel plots, whose asymmetry was quantified Egger’s regression. The methodological quality was assessed through McMaster’s. PowerBreath® administering a ≥ 15% resistive load of the maximum inspiratory pressure (PIM) achieves significant improvements (54%) in said pressure within 4 weeks of commencing the inspiratory muscle training. The maximal volume of oxygen (VO2max) considerable enhancements was achieved from the 6 weeks associated with the maximum inspiratory pressure ≥ 21.5% post inspiratory muscle training onwards. Conversely, a significant blood lactate concentration decrement occurred from the 4th week of inspiratory muscle training, after a maximum inspiratory pressure ≥ 6.8% increment. PowerBreath® is a useful device to stimulate sport performance and increase pulmonary function.


2020 ◽  
pp. 026921552098013
Author(s):  
Jui-Fang Liu ◽  
Nai-Ying Kuo ◽  
Teng-Pei Fang ◽  
Jui-O Chen ◽  
Hung-I Lu ◽  
...  

Objective: To compare the postoperative outcomes of inspiratory muscle training and aerobic exercise, along with standard care, on lung cancer patients undergoing video-assisted thoracoscopic surgery (VATS). Design: A parallel-group, single-blind randomized clinical trial Setting: Thoracic surgery ward and outpatient clinic in a teaching hospital Subjects: Overall 63 patients underwent VATS were randomly assigned to a triaging (TG, n = 32) or control group (CG, n = 31). A total of 54 patients (TG, n = 26; CG, n = 28) completed the study. Intervention: TG: six-week threshold inspiratory muscle training and aerobic exercise. CG: standard care. Main measures: Maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) lung expansion volume, and 6-min walking test (6MWT) were performed on the day of chest tube removal (baseline), and 2, 6, and 12 weeks postoperatively. Results: The TG showed significant improvement in PImax at week 6 (71.6 ± 34.9 vs. 94.3 ± 32.8 cmH2O, P = 0.018), PEmax at week 2 (70.9 ± 24.3 vs. 90.9 ± 28.2 cmH2O, P = 0.015) and week 12 (76.1 ± 20.2 vs. 98.6 ± 35.3 cmH2O, P = 0.012), the lung expansion volume at week 2 (1080 ± 433 vs 1457 ± 624 mL, P = 0.02) and week 12 (1200 ± 387 vs 1885 ± 678 mL, P < 0.001), in addition to the 6MWT at week 2 (332 ± 78 vs 412 ± 74 m, P = 0.002), week 6 (360 ± 70 vs 419 ± 60 m, P = 0.007) and week 12 (360 ± 58 vs 402 ± 65 m, P = 0.036). Conclusion: A six weeks of inspiratory muscle training and aerobic exercise had improved respiratory muscle strength and aerobic exercise postoperatively in lung cancer patients after VATS as early as 2 weeks.


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