{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T17:16:31Z","timestamp":1776878191575,"version":"3.51.2"},"reference-count":43,"publisher":"Public Library of Science (PLoS)","issue":"5","license":[{"start":{"date-parts":[[2024,5,15]],"date-time":"2024-05-15T00:00:00Z","timestamp":1715731200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:sec id=\"sec001\">\n<jats:title>Introduction<\/jats:title>\n<jats:p>This cohort study aimed to investigate the factors associated with noninvasive positive pressure ventilation adherence and assess the long-term effects of noninvasive positive pressure ventilation adherence in patients with amyotrophic lateral sclerosis (ALS).<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec002\">\n<jats:title>Methods<\/jats:title>\n<jats:p>The medical records of patients with ALS admitted to a tertiary hospital for noninvasive positive pressure ventilation initiation were retrospectively reviewed. Pulmonary function parameters, variables of blood gas analysis, the site of symptom onset, the time from onset and diagnosis to noninvasive positive pressure ventilation application, ALS Functional Rating Scale-Revised, neurophysiological index, and the length of hospital stay were evaluated. The adherence to noninvasive positive pressure ventilation was defined as the use of noninvasive positive pressure ventilation for \u2265 2 h\/day or \u2265 4 h\/day. The correlations between noninvasive positive pressure ventilation adherence or length of hospital stay and other clinical parameters were analyzed.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec003\">\n<jats:title>Results<\/jats:title>\n<jats:p>Fifty-one patients with ALS were included in the study. The time from onset and diagnosis to NIPPV application was reduced by 16 months in the adherent group than that in the non-adherent group; however, the parameters of blood gas analysis and pulmonary function tests did not differ significantly between the groups. Furthermore, the neurophysiological index of the abductor digiti minimi muscle was higher by 4.05 in the adherent group than that in the non-adherent group. The adherence to noninvasive positive pressure ventilation prolonged tracheostomy-free survival compared to that of non-adherence. Desaturation events, lower forced vital capacity, last pCO2, bicarbonate, and base excess, and higher differences in pCO2, were associated with an increase in the length of hospital stay.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec004\">\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Noninvasive positive pressure ventilation application shortly after symptom onset and ALS diagnosis in patients with CO<jats:sub>2<\/jats:sub> retention and reduced forced vital capacity can be considered for successful adherence. Adherence to noninvasive positive pressure ventilation may result in reduced tracheostomy conversion rates and prolonged tracheostomy-free survival.<\/jats:p>\n<\/jats:sec>","DOI":"10.1371\/journal.pone.0302515","type":"journal-article","created":{"date-parts":[[2024,5,15]],"date-time":"2024-05-15T17:38:34Z","timestamp":1715794714000},"page":"e0302515","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":1,"title":["Factors associated with adherence to noninvasive positive pressure ventilation in amyotrophic lateral sclerosis"],"prefix":"10.1371","volume":"19","author":[{"given":"Hee Soo","family":"Kim","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyeonseong","family":"Woo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seok-Jin","family":"Choi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jong-Gyu","family":"Baek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3299-3038","authenticated-orcid":true,"given":"Ju Seok","family":"Ryu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyung-Ik","family":"Shin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1553-5932","authenticated-orcid":true,"given":"Kyung Seok","family":"Park","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7984-9661","authenticated-orcid":true,"given":"Jaewon","family":"Beom","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2024,5,15]]},"reference":[{"key":"pone.0302515.ref001","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1111\/j.1468-1331.2008.02307.x","article-title":"Causes of death amongst French patients with amyotrophic lateral sclerosis: a prospective study","volume":"15","author":"J Gil","year":"2008","journal-title":"Eur J Neurol"},{"key":"pone.0302515.ref002","doi-asserted-by":"crossref","first-page":"2000","DOI":"10.1093\/brain\/124.10.2000","article-title":"Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis","volume":"124","author":"RA Lyall","year":"2001","journal-title":"Brain"},{"key":"pone.0302515.ref003","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1080\/21678421.2018.1452945","article-title":"Respiratory measures in amyotrophic lateral sclerosis.","volume":"19","author":"N Lechtzin","year":"2018","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"pone.0302515.ref004","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1212\/WNL.57.1.153","article-title":"A prospective study of quality of life in ALS patients treated with noninvasive ventilation","volume":"57","author":"RA Lyall","year":"2001","journal-title":"Neurology"},{"key":"pone.0302515.ref005","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/S1474-4422(05)70326-4","article-title":"Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial","volume":"5","author":"SC Bourke","year":"2006","journal-title":"Lancet Neurol"},{"key":"pone.0302515.ref006","doi-asserted-by":"crossref","first-page":"556","DOI":"10.1111\/ene.13547","article-title":"Impact of an early respiratory care programme with non\u2010invasive ventilation adaptation in patients with amyotrophic lateral sclerosis","volume":"25","author":"M Vitacca","year":"2018","journal-title":"Eur J Neurol"},{"key":"pone.0302515.ref007","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1080\/14660820500514974","article-title":"Effect of non-invasive ventilation on survival, quality of life, respiratory function and cognition: a review of the literature.","volume":"7","author":"S Piepers","year":"2006","journal-title":"Amyotroph Lateral Scler."},{"key":"pone.0302515.ref008","doi-asserted-by":"crossref","first-page":"e0177555","DOI":"10.1371\/journal.pone.0177555","article-title":"Is survival improved by the use of NIV and PEG in amyotrophic lateral sclerosis (ALS)? A post-mortem study of 80 ALS patients","volume":"12","author":"C Burkhardt","year":"2017","journal-title":"PLOS ONE."},{"key":"pone.0302515.ref009","doi-asserted-by":"crossref","first-page":"1218","DOI":"10.1212\/WNL.0b013e3181bc0141","article-title":"Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology.","volume":"73","author":"RG Miller","year":"2009","journal-title":"Neurology"},{"key":"pone.0302515.ref010","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1111\/j.1468-1331.2011.03501.x","article-title":"EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)-revised report of an EFNS task force.","volume":"19","author":"EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis","year":"2012","journal-title":"Eur J Neurol"},{"key":"pone.0302515.ref011","doi-asserted-by":"crossref","first-page":"1878","DOI":"10.1212\/WNL.0000000000003158","article-title":"Trial of early noninvasive ventilation for ALS: A pilot placebo-controlled study","volume":"87","author":"TL Jacobs","year":"2016","journal-title":"Neurology"},{"key":"pone.0302515.ref012","first-page":"53","article-title":"The ALS Patient Care Database: goals, design, and early results","volume":"54","author":"RG Miller","year":"2000","journal-title":"ALS C.A.R.E. 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Sci"},{"key":"pone.0302515.ref022","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.pulmoe.2019.05.007","article-title":"Does timing of initiation influence acceptance and adherence to NIV in patients with ALS?","volume":"26","author":"M Vitacca","year":"2020","journal-title":"Pulmonology"},{"key":"pone.0302515.ref023","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1513\/AnnalsATS.202002-169OC","article-title":"Noninvasive ventilation use is associated with better survival in amyotrophic lateral sclerosis.","volume":"18","author":"J Ackrivo","year":"2021","journal-title":"Ann Am Thorac Soc"},{"key":"pone.0302515.ref024","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1002\/mus.20450","article-title":"Pulmonary predictors of survival in amyotrophic lateral sclerosis: use in clinical trial design","volume":"33","author":"EP Schmidt","year":"2006","journal-title":"Muscle Nerve"},{"key":"pone.0302515.ref025","first-page":"CD001447","article-title":"Riluzole for amyotrophic lateral sclerosis (ALS)\/motor neuron disease (MND).","volume":"3","author":"RG Miller","year":"2012","journal-title":"Cochrane Database Syst Rev."},{"issue":"3","key":"pone.0302515.ref026","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1080\/17482960701262392","article-title":"Early use of non\u2010invasive ventilation prolongs survival in subjects with ALS.","volume":"8","author":"N Lechtzin","year":"2007","journal-title":"Amyotrophic Lateral Sclerosis."},{"key":"pone.0302515.ref027","doi-asserted-by":"crossref","first-page":"373","DOI":"10.3109\/21678421.2013.776086","article-title":"Prognostic value of efficiently correcting nocturnal desaturations after one month of non-invasive ventilation in amyotrophic lateral sclerosis: a retrospective monocentre observational cohort study.","volume":"14","author":"J Gonzalez-Bermejo","year":"2013","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"pone.0302515.ref028","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1002\/mus.27736","article-title":"Clinical and neurophysiological biomarkers of disease progression in amyotrophic lateral sclerosis","volume":"67","author":"A Hannaford","year":"2023","journal-title":"Muscle Nerve"},{"key":"pone.0302515.ref029","doi-asserted-by":"crossref","first-page":"1564","DOI":"10.1016\/j.clinph.2021.02.394","article-title":"Neurophysiological indices in amyotrophic lateral sclerosis correlate with functional outcome measures, staging and disease progression.","volume":"132","author":"A Barp","year":"2021","journal-title":"Clin Neurophysiol"},{"issue":"1","key":"pone.0302515.ref030","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1186\/1750-1172-4-10","article-title":"Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency","volume":"4","author":"P. Carrat\u00f9","year":"2009","journal-title":"Orphanet Journal of Rare Diseases"},{"issue":"sup1","key":"pone.0302515.ref031","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1080\/21678421.2021.1887263","article-title":"Arterial blood gas analysis: base excess and carbonate are predictive of noninvasive ventilation adaptation and survival in amyotrophic lateral sclerosis.","volume":"22","author":"U Manera","year":"2021","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"4","key":"pone.0302515.ref032","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1155\/2011\/139769","article-title":"Home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline.","volume":"18","author":"DA McKim","year":"2011","journal-title":"Can Respir J."},{"issue":"5","key":"pone.0302515.ref033","doi-asserted-by":"crossref","first-page":"307","DOI":"10.3949\/ccjm.77a.09145","article-title":"Noninvasive positive pressure ventilation: Increasing use in acute care.","volume":"77","author":"LS Aboussouan","year":"2010","journal-title":"Cleveland Clinic Journal of Medicine"},{"key":"pone.0302515.ref034","doi-asserted-by":"crossref","first-page":"220266","DOI":"10.1183\/20734735.0266-2022","article-title":"How to increase noninvasive ventilation effectiveness in bulbar amyotrophic lateral sclerosis patients.","volume":"19","author":"J Sancho","year":"2023","journal-title":"Breathe (Sheff)."},{"key":"pone.0302515.ref035","doi-asserted-by":"crossref","first-page":"1425","DOI":"10.1016\/S0140-6736(96)91680-3","article-title":"Dose-ranging study of riluzole in amyotrophic lateral sclerosis","volume":"347","author":"L Lacomblez","year":"1996","journal-title":"Lancet"},{"key":"pone.0302515.ref036","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.jns.2012.08.011","article-title":"Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: clinical features and survival analysis","volume":"323","author":"R Spataro","year":"2012","journal-title":"J Neurol Sci"},{"key":"pone.0302515.ref037","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1111\/ene.14647","article-title":"Non-invasive and tracheostomy invasive ventilation in amyotrophic lateral sclerosis: utilization and survival rates in a cohort study over 12 years in Germany.","volume":"28","author":"S Spittel","year":"2021","journal-title":"Eur J Neurol"},{"key":"pone.0302515.ref038","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1136\/jnnp.2009.193631","article-title":"Weaning from prolonged invasive ventilation in motor neuron disease: analysis of outcomes and survival","volume":"82","author":"R Chadwick","year":"2011","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"pone.0302515.ref039","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1136\/thx.2011.160481","article-title":"Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year 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