{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:00:19Z","timestamp":1791302419809,"version":"4.1.0"},"reference-count":42,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,5,6]],"date-time":"2013-05-06T00:00:00Z","timestamp":1367798400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2013,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:label\/><jats:p><jats:italic>Introduction<\/jats:italic>: Glucocorticoid (GC) therapy in Duchenne muscular dystrophy (DMD) has altered disease progression, necessitating contemporary natural history studies. <jats:italic>Methods<\/jats:italic>: The Cooperative Neuromuscular Research Group (CINRG) DMD Natural History Study (DMD\u2010NHS) enrolled 340 DMD males, ages 2\u201328 years. A comprehensive battery of measures was obtained. <jats:italic>Results<\/jats:italic>: A novel composite functional \u201cmilestone\u201d scale scale showed clinically meaningful mobility and upper limb abilities were significantly preserved in GC\u2010treated adolescents\/young adults. Manual muscle test (MMT)\u2010based calculations of global strength showed that those patients &lt;10 years of age treated with steroids declined by 0.4\u00b10.39 MMT unit\/year, compared with \u22120.4\u00b10.39 MMT unit\/year in historical steroid\u2010naive subjects. Pulmonary function tests (PFTs) were relatively preserved in steroid\u2010treated adolescents. The linearity and magnitude of decline in measures were affected by maturational changes and functional status. <jats:italic>Conclusions<\/jats:italic>: In DMD, long\u2010term use of GCs showed reduced strength loss and preserved functional capabilities and PFTs compared with previous natural history studies performed prior to the widespread use of GC therapy. <jats:italic>Muscle Nerve<\/jats:italic>, 2013<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.23808","type":"journal-article","created":{"date-parts":[[2013,2,6]],"date-time":"2013-02-06T15:39:06Z","timestamp":1360165146000},"page":"55-67","source":"Crossref","is-referenced-by-count":183,"title":["The cooperative international neuromuscular research group Duchenne natural history study: Glucocorticoid treatment preserves clinically meaningful functional milestones and reduces rate of disease progression as measured by manual muscle testing and other commonly used clinical trial outcome measures"],"prefix":"10.1002","volume":"48","author":[{"given":"Erik K.","family":"Henricson","sequence":"first","affiliation":[{"name":"Department of Physical Medicine and Rehabilitation School of Medicine University of California Davis 4860 Y Street Suite 3850 Sacramento California 95817 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Ted","family":"Abresch","sequence":"additional","affiliation":[{"name":"Department of Physical Medicine and Rehabilitation School of Medicine University of California Davis 4860 Y Street Suite 3850 Sacramento California 95817 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Avital","family":"Cnaan","sequence":"additional","affiliation":[{"name":"Center for Genetic Medicine Research, Children's National Medical Center Washington DC USA"},{"name":"Departments of Pediatrics, Epidemiology, and Biostatistics, George Washington University Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fengming","family":"Hu","sequence":"additional","affiliation":[{"name":"Center for Genetic Medicine Research, Children's National Medical Center Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tina","family":"Duong","sequence":"additional","affiliation":[{"name":"Center for Genetic Medicine Research, Children's National Medical Center Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrienne","family":"Arrieta","sequence":"additional","affiliation":[{"name":"Center for Genetic Medicine Research, Children's National Medical Center Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jay","family":"Han","sequence":"additional","affiliation":[{"name":"Department of Physical Medicine and Rehabilitation School of Medicine University of California Davis 4860 Y Street Suite 3850 Sacramento California 95817 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana M.","family":"Escolar","sequence":"additional","affiliation":[{"name":"Department of Neurology, Kennedy Krieger Institute Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julaine M.","family":"Florence","sequence":"additional","affiliation":[{"name":"Department of Neurology, Washington University St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula R.","family":"Clemens","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pittsburgh and Department of Veterans Affairs Medical Center Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric P.","family":"Hoffman","sequence":"additional","affiliation":[{"name":"Center for Genetic Medicine Research, Children's National Medical Center Washington DC USA"},{"name":"Department of Integrative Systems Biology, George Washington University Washington, DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig M.","family":"McDonald","sequence":"additional","affiliation":[{"name":"Department of Physical Medicine and Rehabilitation School of Medicine University of California Davis 4860 Y Street Suite 3850 Sacramento California 95817 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the CINRG Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,5,6]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(87)90579-4"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0960-8966(91)90039-U"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-1809.1955.tb01289.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320070107"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1980.tb02293.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00279050"},{"key":"e_1_2_7_8_1","first-page":"588","article-title":"On fatty degeneration of the voluntary muscles","volume":"2","author":"Meryon E","year":"1851","journal-title":"Lancet"},{"key":"e_1_2_7_9_1","volume-title":"Album de photographes pathologiques","author":"Duchenne GBA","year":"1862"},{"key":"e_1_2_7_10_1","volume-title":"Pseudo\u2010hypertrophic muscular paralysis","author":"Gowers W","year":"1879"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1981.tb08442.x"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gaitpost.2008.06.002"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00002060-199509001-00003"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(02)00140-2"},{"key":"e_1_2_7_15_1","first-page":"808","article-title":"Clinical investigation of Duchenne muscular dystrophy","volume":"44","author":"Mendell JR","year":"1987","journal-title":"A methodology for therapeutic trials based on natural history controls. Arch Neurol"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.39.4.475"},{"key":"e_1_2_7_17_1","first-page":"173","volume-title":"Muscular dystrophy research","author":"Angelini C","year":"1991"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198906153202405"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.12.1874"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1991.00530180027012"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1991.00530160047012"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(02)00180-3"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e318227b164"},{"key":"e_1_2_7_24_1","first-page":"XXX","article-title":"The Cooperative International Neuromuscular Research Group Duchenne Natural History Study\u2014a longitudinal investigation in the era of glucocorticoid therapy: design of protocol and the methods used","author":"McDonald CM","year":"2013","journal-title":"Muscle Nerve"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880040304"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1963.03700150043007"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2004.tb00508.x"},{"key":"e_1_2_7_28_1","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1093\/ptj\/65.9.1339","article-title":"Reliability of goniometric measurements in patients with Duchenne muscular dystrophy","volume":"65","author":"Pandya S","year":"1985","journal-title":"Phys Ther"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1097\/00002060-199509001-00002"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880060204"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/ptj\/64.1.41"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1093\/ptj\/72.2.115"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.1070"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20654"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70271-6"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21544"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21808"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2011.03939.x"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2010.06.014"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2009.06.368"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1197\/j.jht.2005.04.003"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.2466\/pms.2000.90.3.823"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1371\/currents.RRN1297"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.23808","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.23808","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T09:18:14Z","timestamp":1696497494000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.23808"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,5,6]]},"references-count":42,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,7]]}},"alternative-id":["10.1002\/mus.23808"],"URL":"https:\/\/doi.org\/10.1002\/mus.23808","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,5,6]]}}}