{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,5]],"date-time":"2025-06-05T16:02:49Z","timestamp":1749139369093,"version":"3.40.5"},"reference-count":0,"publisher":"Termedia Sp. z.o.o.","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arch Med Sci"],"published-print":{"date-parts":[[2021,3,21]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Respiratory status is one of the main factors affecting the length of survival in patients with Duchenne muscular dystrophy (DMD) \u2013 the most common, severe, progressive muscular dystrophy.The aim: (1) to assess pulmonary function in DMD patients using the z-score method and (2) to identify factors affecting it, irrespective of the disease progress.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and methods<\/jats:title><jats:p>We evaluated 55 boys (aged 5 \u2013 18 years) with DMD. The spirometry was performed with: forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), peak expiratory flow (PEF)  analysis as absolute values (in litres or litres\/min), % predicted value (%pv) and z-scores [z]. The need of ventilation support, ambulatory status, steroid therapy were collected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>25(45%) subjects were non-ambulatory, 38(69%) used steroid therapy. Mean FVC[z] -2.4\u00b12.2, FEV1[z] -2.0\u00b11.9, PEF[z] -1.5\u00b11.3 value significantly decrease with age (r=-0.62\/-0.65\/-0.55; p&lt;0.001 respectively), after reaching the peak values between 9-12 or 6-9 years of age depending on analysis method (absolute, %pv or z-score). The results fell below normal range (z-score&lt;-1.64) at the age of 9.8\/10.4\/11.6 years and below 80%pv at 10.7\/13.2\/13.2 for FVC\/FEV1\/PEF, respectively. The pulmonary function test results were significantly lower in non-ambulant (p&lt;0.001) and  non-steroid patients (p&lt;0.02).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Analysis of pulmonary function test based on z-score shows that deterioration of pulmonary function in DMD males may appear earlier than we thought measured by %pv and absolute values. Early loss of ambulation, lack of or delayed steroid therapy are risk factors for worse pulmonary outcomes. To confirm these findings cohort longitudinal studies are necessary.<\/jats:p><\/jats:sec>","DOI":"10.5114\/aoms\/119782","type":"journal-article","created":{"date-parts":[[2021,3,22]],"date-time":"2021-03-22T22:03:38Z","timestamp":1616450618000},"source":"Crossref","is-referenced-by-count":5,"title":["\u201cPulmonary dysfunction in children with Duchenne muscular dystrophy may appear earlier than we thought \u2013 analysis using novel methodology based on z-scores.\""],"prefix":"10.5114","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5288-0586","authenticated-orcid":false,"given":"Eliza","family":"Wasilewska","sequence":"first","affiliation":[{"name":"Department of Pulmonology and Allergology, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylwia","family":"Ma\u0142gorzewicz","sequence":"additional","affiliation":[{"name":"Department of Clinical Nutrition, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaros\u0142aw","family":"Meyer-Szary","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology and Congenital Heart Defects, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karolina","family":"Sledzinska","sequence":"additional","affiliation":[{"name":"Department of General Nursery, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marek","family":"Niedoszytko","sequence":"additional","affiliation":[{"name":"Department of Pulmonology and Allergology, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ewa","family":"Jassem","sequence":"additional","affiliation":[{"name":"Department of Pulmonology and Allergology, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jolanta","family":"Wierzba","sequence":"additional","affiliation":[{"name":"Department of General Nursery, Medical University of Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3061","published-online":{"date-parts":[[2021,3,21]]},"container-title":["Archives of Medical Science"],"original-title":[],"link":[{"URL":"https:\/\/www.archivesofmedicalscience.com\/pdf-119782-62980","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.archivesofmedicalscience.com\/pdf-119782-62980","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,29]],"date-time":"2021-10-29T14:18:53Z","timestamp":1635517133000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.archivesofmedicalscience.com\/-Pulmonary-dysfunction-in-children-with-Duchenne-muscular-dystrophy-may-appear-earlier,119782,0,2.html"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,21]]},"references-count":0,"alternative-id":["119782"],"URL":"https:\/\/doi.org\/10.5114\/aoms\/119782","relation":{},"ISSN":["1734-1922","1896-9151"],"issn-type":[{"type":"print","value":"1734-1922"},{"type":"electronic","value":"1896-9151"}],"subject":[],"published":{"date-parts":[[2021,3,21]]}}}