{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T02:41:02Z","timestamp":1780368062847,"version":"3.54.1"},"reference-count":41,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,9,14]],"date-time":"2022-09-14T00:00:00Z","timestamp":1663113600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004243","name":"Prinses Beatrix Spierfonds","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013428","name":"Stichting Spieren voor Spieren","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100013428","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Respiratory failure is a major cause of morbidity and mortality in patients with Spinal Muscular Atrophy (SMA). Lack of endurance, or \u201cfatigability,\u201d is an important symptom of SMA. In addition to respiratory muscle weakness, respiratory function in SMA may be affected by Respiratory Muscle Fatigability (RMF).<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>The purpose of this study was to explore RMF in patients with SMA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We assessed a Respiratory Endurance Test (RET) in 19 children (median age [years]: 11) and 36 adults (median age [years]: 34) with SMA types 2 and 3. Participants were instructed to breath against an inspiratory threshold load at either 20%, 35%, 45%, 55%, or 70% of their individual maximal inspiratory mouth pressure (PImax). RMF was defined as the inability to complete 60 consecutive breaths. Respiratory fatigability response was determined by change in maximal inspiratory mouth pressure (\u0394PImax) and perceived fatigue (\u2206perceived fatigue).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The probability of RMF during the RET increased by 59%\u221269% over 60 breaths with every 10% increase in inspiratory threshold load (%PImax). Fatigability response was characterized by a large variability in \u0394PImax (\u221221% to +16%) and a small increase in perceived fatigue (<jats:italic>p<\/jats:italic>\u2009=\u20090.041, range 0 to +3).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion and Key Findings<\/jats:title><jats:p>Patients with SMA demonstrate a dose\u2010dependent increase in RMF without severe increase in exercise\u2010induced muscle weakness or perceived fatigue. Inspiratory muscle loading in patients with SMA seems feasible and its potential to stabilize or improve respiratory function in patients with SMA needs to be determined in further research.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26133","type":"journal-article","created":{"date-parts":[[2022,8,30]],"date-time":"2022-08-30T10:08:34Z","timestamp":1661854114000},"page":"3050-3059","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Respiratory muscle fatigability in patients with spinal muscular atrophy"],"prefix":"10.1002","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6187-0849","authenticated-orcid":false,"given":"Kim","family":"Kant\u2010Smits","sequence":"first","affiliation":[{"name":"Child Development and Exercise Center, Wilhelmina Children's Hospital University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erik H. J.","family":"Hulzebos","sequence":"additional","affiliation":[{"name":"Child Development and Exercise Center, Wilhelmina Children's Hospital University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura E.","family":"Habets","sequence":"additional","affiliation":[{"name":"Child Development and Exercise Center, Wilhelmina Children's Hospital University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fay\u2010Lynn","family":"Asselman","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Center University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2918-9989","authenticated-orcid":false,"given":"Esther S.","family":"Veldhoen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Intensive Care, University Medical Centre Utrecht Utrecht University  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruben P. A.","family":"van Eijk","sequence":"additional","affiliation":[{"name":"University Medical Center Utrecht,\u00a0Biostatistics &amp; Research Support, Julius Center for Health Sciences and Primary Care, Utrecht, The Netherlands and University Medical Center Utrecht UMC Utrecht Brain Center  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janke F.","family":"de Groot","sequence":"additional","affiliation":[{"name":"Research Group Lifestyle and Health HU University of Applied Health Sciences Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. 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