{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T14:11:51Z","timestamp":1788271911193,"version":"build-2803163510"},"reference-count":28,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T00:00:00Z","timestamp":1778544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T00:00:00Z","timestamp":1778544000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To develop a functional mobility classification for spinal muscular atrophy (SMA), reflecting changing phenotypes in the era of disease\u2010modifying agents, with associations to hip displacement, scoliosis, and pulmonary function determined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>Patients with SMA and Hammersmith Functional Motor Scale Expanded (HFMSE) assessments were included. The Spinal Muscular Atrophy Functional Classification System (SMAFCS), comprising levels I to IV, was constructed, referencing Functional Mobility Scale groupings. Receiver\u2010operating characteristic analysis determined HFMSE thresholds by SMAFCS. Associations of hip displacement, scoliosis, and pulmonary function were determined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Sixty patients (62% female) were included, with 312 HFMSE assessments (mean 5.3\u00a0[SD 2.7] per patient) over 7\u2009years 6\u2009months\u00a0(SD 3\u2009years 11\u2009months). HFMSE thresholds by SMAFCS level were I, \u226527; II, \u226522, &lt;27; III, \u226510, &lt;22; IV, &lt;10 (area under the curve 0.62\u20130.89;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). HFMSE versus SMAFCS age trajectories were modeled, significant for levels I (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.002), III (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), and IV (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.002) but not level II (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.48). The prevalences of hip displacement and scoliosis by SMAFCS level were respectively I, 61%; II, 50%; III, 92%; IV, 96% (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.008) and I, 42%; II, 50%; III, 85%; IV, 96% (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Pulmonary function (forced vital capacity percent of predicted) worsened by SMAFCS level: I, 96\u00a0(SD 15); II, 83\u00a0(SD 12); III, 75\u00a0(SD 16); IV, 48\u00a0(SD 27) (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>These preliminary data suggest the SMAFCS could be a valuable tool to stratify gross motor function and predict risk of clinical outcomes in SMA. Further work is required to study reliability and responsiveness to change.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/dmcn.70307","type":"journal-article","created":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T04:14:46Z","timestamp":1778645686000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["The Spinal Muscular Atrophy Functional Classification System"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3049-6345","authenticated-orcid":false,"given":"Jason J.","family":"Howard","sequence":"first","affiliation":[{"name":"Division of Orthopaedic Surgery, Department of Surgery IWK Health Centre  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6607-7631","authenticated-orcid":false,"given":"H. Kerr","family":"Graham","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery University of Melbourne, Royal Children's Hospital  Melbourne VIC Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8207-2109","authenticated-orcid":false,"given":"M. Wade","family":"Shrader","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Surgery Nemours Children's Health  Wilmington DE USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"Schroth","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology, University of Wisconsin School of Medicine and Public Health  Madison WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manish","family":"Kumar","sequence":"additional","affiliation":[{"name":"Pediatric Neurology, Neuromuscular Division Nemours Children's Health  Wilmington DE USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sadettin","family":"Ciftci","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics and Traumatology Sel\u00e7uk University  Konya Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,12]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.5435\/JAAOS-20-06-393"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.11.004"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/344767a0"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2011.09.001"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-genom-102319-103602"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-017-0711-7"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/prp2.447"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1706198"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1710504"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/BPO.0000000000002595"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/BPO.0000000000002759"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(06)80015-5"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12883-017-0790-9"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/01241398-200409000-00011"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3109\/01942630903454930"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2007.00603.x"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/09638288.2020.1746845"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.3109\/ort.1980.51.suppl-184.01"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s43390-021-00302-w"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12874-022-01542-8"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/BPO.0000000000002091"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.2106\/00004623-200601000-00015"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/jpc.15707"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamapediatrics.2024.0492"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2007.00764.x"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2018.01.003"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305788"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2013.04.004"}],"container-title":["Developmental Medicine &amp; 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