{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T04:15:16Z","timestamp":1791951316039,"version":"4.3.4"},"reference-count":44,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,8,6]],"date-time":"2022-08-06T00:00:00Z","timestamp":1659744000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006447","name":"Universit\u00e4t Z\u00fcrich","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006447","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Prognostic factors in Duchenne muscular dystrophy (DMD) predict the disease course and may help individualize patient care. The aim was to summarize the evidence on prognostic factors that may support treatment decisions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We searched six databases for prospective studies that each included \u226550 DMD patients with a minimum follow\u2010up of 1 y. Primary outcomes were age at loss of ambulation (LoA), pulmonary function (forced vital capacity percent of predicted, FVC%p), and heart failure.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Out of 5074 references, 59 studies were analyzed. Corticosteroid use was associated with a delayed LoA (pooled effect hazard ratio [HR] 0.42, 95% confidence interval [CI] 0.23\u20130.75, I2 94%), better pulmonary function tests (higher peak FVC%, prolonged time with FVC%p\u2009&gt;\u200950%, and reduced need for assisted ventilation) and delayed cardiomyopathy. Longer corticosteroid treatment was associated with later LoA (&gt;1\u2009y compared to &lt;1\u2009y; pooled HR: 0.50, 95% CI 0.27\u20130.90) and early treatment start (aged &lt;5\u2009y) may be associated with early cardiomyopathy and higher fracture risk. Genotype appeared to be an independent driver of LoA in some studies. Higher baseline physical function tests (e.g., 6\u2010minute walk test) were associated with delayed LoA. Left ventricular dysfunction and FVC &lt;1 L increased and the use of angiotensin\u2010converting enzyme (ACE) inhibitors reduced the risk of heart failure and death. Fusion surgery in scoliosis may potentially preserve pulmonary function.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Prognostic factors that may inform clinical decisions include age at corticosteroid treatment initiation and treatment duration, ACE\u2010inhibitor use, baseline physical function tests, pulmonary function, and cardiac dysfunction.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27682","type":"journal-article","created":{"date-parts":[[2022,7,21]],"date-time":"2022-07-21T08:18:36Z","timestamp":1658391516000},"page":"462-470","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Prognostic factors, disease course, and treatment efficacy in Duchenne muscular dystrophy: A systematic review and meta\u2010analysis"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9842-4749","authenticated-orcid":false,"given":"Fabio J.","family":"Weber","sequence":"first","affiliation":[{"name":"Sleep Disorders Center and Pulmonary Division University Hospital Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsogyal D.","family":"Latshang","sequence":"additional","affiliation":[{"name":"Sleep Disorders Center and Pulmonary Division University Hospital Zurich  Zurich Switzerland"},{"name":"Sleep Disorders Center and Pulmonary Division Kantonsspital Graubuenden  Chur Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel R.","family":"Blum","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine University Hospital Bern, University of Bern  Bern Switzerland"},{"name":"Institute of Primary Health Care (BIHAM) University of Bern  Bern Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malcolm","family":"Kohler","sequence":"additional","affiliation":[{"name":"Sleep Disorders Center and Pulmonary Division University Hospital Zurich  Zurich Switzerland"},{"name":"Zurich Center for Interdisciplinary Sleep Research University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria M.","family":"Wertli","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine University Hospital Bern, University of Bern  Bern Switzerland"},{"name":"Department of Internal Medicine Cantonal Hospital Baden  Baden Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,8,6]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073810371002"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70271-6"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/0009922810378738"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(02)00140-2"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10654-020-00613-8"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30024-3"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30025-5"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30026-7"},{"issue":"10","key":"e_1_2_13_10_1","first-page":"783","article-title":"[changes in clinical condition and causes of death of inpatients with Duchenne muscular dystrophy in Japan from 1999 to 2012]. 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