{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T22:03:50Z","timestamp":1791669830245,"version":"4.3.4"},"reference-count":47,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2020,10,1]],"date-time":"2020-10-01T00:00:00Z","timestamp":1601510400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,10,1]],"date-time":"2020-10-01T00:00:00Z","timestamp":1601510400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100011641","name":"Muscular Dystrophy","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100011641","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000765","name":"University College London","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","journal.chestnet.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Chest"],"published-print":{"date-parts":[[2020,10]]},"DOI":"10.1016\/j.chest.2020.04.043","type":"journal-article","created":{"date-parts":[[2020,5,7]],"date-time":"2020-05-07T03:41:59Z","timestamp":1588822919000},"page":"1606-1616","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":48,"title":["Cardiorespiratory Progression Over 5 Years and Role of Corticosteroids in Duchenne Muscular Dystrophy"],"prefix":"10.1016","volume":"158","author":[{"given":"Federica","family":"Trucco","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joana P.","family":"Domingos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chee Geap","family":"Tay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah","family":"Ridout","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kate","family":"Maresh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pinki","family":"Munot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Sarkozy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Robb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosaline","family":"Quinlivan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mollie","family":"Riley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Burch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Fenton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin","family":"Wallis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francois","family":"Abel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adnan Y.","family":"Manzur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Muntoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib1","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1016\/S1474-4422(18)30025-5","article-title":"Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management","volume":"17","author":"Birnkrant","year":"2018","journal-title":"Lancet Neurol"},{"issue":"18","key":"10.1016\/j.chest.2020.04.043_bib2","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1161\/CIRCULATIONAHA.114.015151","article-title":"Contemporary cardiac issues in Duchenne muscular dystrophy. Working Group of the National Heart, Lung, and Blood Institute in collaboration with Parent Project Muscular Dystrophy","volume":"131","author":"McNally","year":"2015","journal-title":"Circulation"},{"issue":"5","key":"10.1016\/j.chest.2020.04.043_bib3","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1212\/WNL.0000000000002337","article-title":"Practice guideline update summary: corticosteroid treatment of Duchenne muscular dystrophy: report of the Guideline Development Subcommittee of the American Academy of Neurology","volume":"86","author":"Gloss","year":"2016","journal-title":"Neurology"},{"issue":"10119","key":"10.1016\/j.chest.2020.04.043_bib4","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/S0140-6736(17)32160-8","article-title":"Long-term effects of glucocorticoids on function, quality of life, and survival in patients with Duchenne muscular dystrophy: a prospective cohort study","volume":"391","author":"McDonald","year":"2018","journal-title":"Lancet"},{"issue":"9","key":"10.1016\/j.chest.2020.04.043_bib5","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1016\/j.jacc.2012.12.008","article-title":"All-cause mortality and cardiovascular outcomes with prophylactic steroid therapy in Duchenne muscular dystrophy","volume":"61","author":"Schram","year":"2013","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib6","doi-asserted-by":"crossref","first-page":"1080","DOI":"10.1016\/j.jpeds.2013.05.060","article-title":"Oral corticosteroids and onset of cardiomyopathy in Duchenne muscular dystrophy","volume":"163","author":"Barber","year":"2013","journal-title":"J\u00a0Pediatr"},{"issue":"5","key":"10.1016\/j.chest.2020.04.043_bib7","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1016\/j.nmd.2008.03.002","article-title":"Corticosteroid treatment retards development of ventricular dysfunction in Duchenne muscular dystrophy","volume":"18","author":"Markham","year":"2008","journal-title":"Neuromuscul Disord"},{"issue":"6","key":"10.1016\/j.chest.2020.04.043_bib8","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1007\/s00246-005-0909-4","article-title":"Steroid therapy and cardiac function in Duchenne muscular dystrophy","volume":"26","author":"Markham","year":"2005","journal-title":"Pediatr Cardiol"},{"issue":"9979","key":"10.1016\/j.chest.2020.04.043_bib9","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1016\/S0140-6736(15)60025-3","article-title":"Efficacy of idebenone on respiratory function in patients with Duchenne muscular dystrophy not using glucocorticoids (DELOS): a double-blind randomised placebo-controlled phase 3 trial","volume":"385","author":"Buyse","year":"2015","journal-title":"Lancet"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib10","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1002\/mus.25089","article-title":"Clinical trial readiness in non-ambulatory boys and men with duchenne muscular dystrophy: MDA-DMD network follow-up","volume":"54","author":"Connolly","year":"2016","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.chest.2020.04.043_bib11","unstructured":"National Institutes of Health Clinical Center. Phase III study of Idebenone in Duchenne muscular dystrophy (DMD) (DELOS). NCT01027884. ClinicalTrials.gov. Bethesda, MD: National Institutes of Health; 2007. http:\/\/clinicaltrials.gov\/ct2\/show\/NCT01027884. Updated October 19, 2015."},{"issue":"5","key":"10.1016\/j.chest.2020.04.043_bib12","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1002\/ppul.23172","article-title":"Characterization of pulmonary function in Duchenne muscular dystrophy","volume":"50","author":"Mayer","year":"2015","journal-title":"Pediatr Pulmonol"},{"issue":"1","key":"10.1016\/j.chest.2020.04.043_bib13","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1002\/mus.23808","volume":"48","author":"Henricson","year":"2013","journal-title":"Muscle Nerve"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib14","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/mus.24346","article-title":"Outcome reliability in non-ambulatory boys\/men with Duchenne muscular dystrophy","volume":"51","author":"Connolly","year":"2015","journal-title":"Muscle Nerve"},{"issue":"11","key":"10.1016\/j.chest.2020.04.043_bib15","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1016\/j.nmd.2018.07.004","article-title":"Longitudinal pulmonary function testing outcome measures in Duchenne muscular dystrophy: long-term natural history with and without glucocorticoids","volume":"28","author":"McDonald","year":"2018","journal-title":"Neuromuscul Disord"},{"issue":"12","key":"10.1016\/j.chest.2020.04.043_bib16","doi-asserted-by":"crossref","DOI":"10.1136\/bmjopen-2018-022572","article-title":"Randomised placebo-controlled trial of combination ACE inhibitor and beta-blocker therapy to prevent cardiomyopathy in children with Duchenne muscular dystrophy? (DMD Heart Protection Study): a protocol study","volume":"8","author":"Bourke","year":"2018","journal-title":"BMJ Open"},{"key":"10.1016\/j.chest.2020.04.043_bib17","unstructured":"EU Clinical Trials Register. A double-blind randomised multi-centre, placebo-controlled trial of combined ACE-inhibitor and beta-blocker therapy in preventing the development of cardiomyopathy in genetically characterised males with DMD without echo-detectable left ventricular dysfunction (DMD Heart-protection trial). EudraCT Number: 2007-005932-10. EU Clinical trials register. https:\/\/www.clinicaltrialsregister.eu\/ctr-search\/search?query=2007-005932-10."},{"issue":"10","key":"10.1016\/j.chest.2020.04.043_bib18","doi-asserted-by":"crossref","first-page":"251","DOI":"10.3390\/genes8100251","article-title":"Dystrophin Dp116: a yet to be investigated product of the duchenne muscular dystrophy gene","volume":"8","author":"Matsuo","year":"2017","journal-title":"Genes (Basel)"},{"issue":"1","key":"10.1016\/j.chest.2020.04.043_bib19","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCGEN.117.001782","article-title":"Cardiac dysfunction in Duchenne muscular dystrophy is less frequent in patients with mutations in the dystrophin Dp116 coding region than in other regions","volume":"11","author":"Yamamoto","year":"2018","journal-title":"Circ Genom Precis Med"},{"issue":"1","key":"10.1016\/j.chest.2020.04.043_bib20","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/ng0593-77","article-title":"An alternative dystrophin trascript specific to peripheral nerve","volume":"4","author":"Byers","year":"1993","journal-title":"Nat Genet"},{"issue":"1","key":"10.1016\/j.chest.2020.04.043_bib21","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1111\/j.1750-3639.1996.tb00780.x","article-title":"Dystrophin and its isoforms","volume":"6","author":"Sadoulet-Puccio","year":"1996","journal-title":"Brain Pathol"},{"key":"10.1016\/j.chest.2020.04.043_bib22","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1016\/S1474-4422(03)00585-4","article-title":"Dystrophin and mutations: one gene, several proteins, multiple phenotypes","volume":"2","author":"Muntoni","year":"2003","journal-title":"Lancet Neurol"},{"issue":"2","key":"10.1016\/j.chest.2020.04.043_bib23","doi-asserted-by":"crossref","first-page":"1376","DOI":"10.1007\/s12035-017-0405-x","article-title":"Dystrophin Dp71 isoforms are differentially expressed in the mouse brain and retina: report of new alternative splicing and a novel nomenclature for Dp71 isoforms","volume":"55","author":"Aragon","year":"2018","journal-title":"Mol Neurobiol"},{"issue":"1","key":"10.1016\/j.chest.2020.04.043_bib24","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1007\/s12035-011-8218-9","article-title":"Dystrophin Dp71: the smallest but multifunctional product of the Duchenne muscular dystrophy gene","volume":"45","author":"Tadayoni","year":"2012","journal-title":"Mol Neurobiol"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib25","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1212\/WNL.0000000000002891","article-title":"DMD genotypes and loss of ambulation in the CINRG Duchenne Natural History Study","volume":"87","author":"Bello","year":"2016","journal-title":"Neurology"},{"issue":"6","key":"10.1016\/j.chest.2020.04.043_bib26","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0218683","article-title":"Long-term natural history data in Duchenne muscular dystrophy ambulant patients with mutations amenable to skip exons 44, 45, 51 and 53","volume":"14","author":"Brogna","year":"2019","journal-title":"PLoS One"},{"issue":"2","key":"10.1016\/j.chest.2020.04.043_bib27","first-page":"149","article-title":"The NorthStar Ambulatory Assessment in Duchenne muscular dystrophy: considerations for the design of clinical trials","volume":"87","author":"Ricotti","year":"2016","journal-title":"J\u00a0Neurol Neurosurg Psychiatry"},{"issue":"6","key":"10.1016\/j.chest.2020.04.043_bib28","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1136\/jnnp-2012-303902","article-title":"Long-term benefits and adverse effects of intermittent versus daily glucocorticoids in boys with Duchenne muscular dystrophy","volume":"84","author":"Ricotti","year":"2013","journal-title":"J\u00a0Neurol Neurosurg Psychiatry"},{"issue":"2","key":"10.1016\/j.chest.2020.04.043_bib29","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1183\/09031936.05.00034805","article-title":"Standardisation of spirometry","volume":"26","author":"Miller","year":"2005","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.chest.2020.04.043_bib30","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","article-title":"Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations","volume":"40","author":"Quanjer","year":"2012","journal-title":"Eur Respir J"},{"issue":"suppl 1","key":"10.1016\/j.chest.2020.04.043_bib31","doi-asserted-by":"crossref","first-page":"i1","DOI":"10.1136\/thoraxjnl-2012-201964","article-title":"British Thoracic Society guideline for respiratory management of children with neuromuscular weakness","volume":"67","author":"Hull","year":"2012","journal-title":"Thorax"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib32","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1164\/rccm.200307-885ST","article-title":"Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement","volume":"170","author":"Finder","year":"2004","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.chest.2020.04.043_bib33","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.euje.2005.12.014","article-title":"Recommendations for chamber quantification","volume":"7","author":"Lang","year":"2006","journal-title":"Eur J Echocardiogr"},{"issue":"8","key":"10.1016\/j.chest.2020.04.043_bib34","doi-asserted-by":"crossref","first-page":"999","DOI":"10.1016\/j.echo.2015.03.003","article-title":"Feasibility and reproducibility of echocardiographic measures in children with muscular dystrophies","volume":"28","author":"Spurney","year":"2015","journal-title":"J\u00a0Am Soc Echocardiogr"},{"key":"10.1016\/j.chest.2020.04.043_bib35","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1016\/j.jpeds.2016.02.067","article-title":"Corticosteroid treatment and growth patterns in ambulatory males with Duchenne muscular dystrophy","volume":"173","author":"Lamb","year":"2016","journal-title":"J\u00a0Pediatr"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib36","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1016\/j.nmd.2016.12.014","article-title":"Characterization of pulmonary function in 10-18 year old patients with Duchenne muscular dystrophy","volume":"27","author":"Meier","year":"2017","journal-title":"Neuromuscul Disord"},{"issue":"2","key":"10.1016\/j.chest.2020.04.043_bib37","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01418-2017","article-title":"Evolution of respiratory function in Duchenne muscular dystrophy from childhood to adulthood","volume":"51","author":"LoMauro","year":"2018","journal-title":"Eur Respir J"},{"issue":"12","key":"10.1016\/j.chest.2020.04.043_bib38","doi-asserted-by":"crossref","first-page":"1048","DOI":"10.1212\/WNL.0000000000001950","article-title":"Prednisone\/prednisolone and deflazacort regimens in the CINRG Duchenne Natural History Study","volume":"85","author":"Bello","year":"2015","journal-title":"Neurology"},{"issue":"5","key":"10.1016\/j.chest.2020.04.043_bib39","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1002\/mus.26191","article-title":"Deflazacort versus prednisone\/prednisolone for maintaining motor function and delaying loss of ambulation: a post HOC analysis from the ACT DMD trial","volume":"58","author":"Shieh","year":"2018","journal-title":"Muscle Nerve"},{"issue":"1","key":"10.1016\/j.chest.2020.04.043_bib40","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1002\/mus.26736","article-title":"Deflazacort vs\u00a0prednisone treatment for Duchenne muscular dystrophy: a meta-analysis of disease progression rates in recent multicenter clinical trials","volume":"61","author":"McDonald","year":"2020","journal-title":"Muscle Nerve"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib41","doi-asserted-by":"crossref","first-page":"e001338","DOI":"10.1161\/JAHA.114.001338","article-title":"Myocardial fibrosis burden predicts left ventricular ejection fraction and is associated with age and steroid treatment\u00a0duration in duchenne muscular\u00a0dystrophy","volume":"4","author":"Tandon","year":"2015","journal-title":"J\u00a0Am Heart Assoc"},{"issue":"2","key":"10.1016\/j.chest.2020.04.043_bib42","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1002\/mus.24163","article-title":"Cooperative International Neuromuscular Research Group Duchenne Natural History Study demonstrates insufficient diagnosis and treatment of cardiomyopathy in Duchenne muscular dystrophy","volume":"50","author":"Spurney","year":"2014","journal-title":"Muscle Nerve"},{"issue":"4","key":"10.1016\/j.chest.2020.04.043_bib43","doi-asserted-by":"crossref","first-page":"e001896","DOI":"10.1161\/JAHA.115.001896","article-title":"Glucocorticoid therapy for Duchenne cardiomyopathy: a Hobson's choice?","volume":"4","author":"Raman","year":"2015","journal-title":"J\u00a0Am Heart Assoc"},{"issue":"6","key":"10.1016\/j.chest.2020.04.043_bib44","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1001\/jamaneurol.2019.0242","article-title":"Fractures and linear growth in a nationwide cohort of boys with Duchenne muscular dystrophy with and without glucocorticoid treatment: results from the UK NorthStar Database","volume":"76","author":"Joseph","year":"2019","journal-title":"JAMA Neurol"},{"issue":"9","key":"10.1016\/j.chest.2020.04.043_bib45","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1038\/sj.gt.3302686","article-title":"Restoration of all dystrophin protein interactions by functional domains in trans does not rescue dystrophy","volume":"13","author":"Gardner","year":"2006","journal-title":"Gene Ther"},{"issue":"3","key":"10.1016\/j.chest.2020.04.043_bib46","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1016\/j.pediatrneurol.2007.11.001","article-title":"Deflazacort use in Duchenne muscular dystrophy: an 8-year follow-up","volume":"38","author":"Houde","year":"2008","journal-title":"Pediatr Neurol"},{"key":"10.1016\/j.chest.2020.04.043_bib47","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.cct.2017.04.008","article-title":"Developing standardized corticosteroid treatment for Duchenne muscular dystrophy","volume":"58","author":"Guglieri","year":"2017","journal-title":"Contemp Clin Trials"}],"container-title":["Chest"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0012369220308692?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0012369220308692?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T08:26:58Z","timestamp":1757924818000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0012369220308692"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10]]},"references-count":47,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2020,10]]}},"alternative-id":["S0012369220308692"],"URL":"https:\/\/doi.org\/10.1016\/j.chest.2020.04.043","relation":{},"ISSN":["0012-3692"],"issn-type":[{"value":"0012-3692","type":"print"}],"subject":[],"published":{"date-parts":[[2020,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cardiorespiratory Progression Over 5 Years and Role of Corticosteroids in Duchenne Muscular Dystrophy","name":"articletitle","label":"Article Title"},{"value":"Chest","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.chest.2020.04.043","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}