{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T17:52:38Z","timestamp":1783101158809,"version":"3.54.6"},"reference-count":102,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,8,11]],"date-time":"2024-08-11T00:00:00Z","timestamp":1723334400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2024,8,11]],"date-time":"2024-08-11T00:00:00Z","timestamp":1723334400000},"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":["Muscle and Nerve"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    The nutritional implications of spinal muscular atrophy (SMA) are profound. Disease modifying therapies (DMT) have improved clinical outcomes. This review describes the impact of DMT on nutrition outcomes. A systematic search strategy was applied across seven databases until May 2023. Eligible studies measured nutrition outcomes in individuals with SMA on DMT (nusinersen, risdiplam or onasemnogene abeparvovec [OA]) compared to untreated comparators. Nutrition outcomes included anthropometry, feeding route, swallowing dysfunction, dietary intake, dietetic intervention, nutritional biochemistry, metabolism, gastrointestinal issues and energy expenditure. Articles retrieved were screened in duplicate, data were extracted and appraised systematically. Sixty three articles from 54 studies were included; 41% (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200922) investigated nusinersen in pediatric participants with SMA type 1. Anthropometry (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200918), feeding route (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200939), and swallowing dysfunction (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200918) were the most commonly reported outcomes. In combined pediatric and adult cohorts, BMI z\u2010score remained stable post nusinersen therapy. The proportion of children with SMA requiring enteral nutrition was stable post nusinersen therapy. Ability to thrive at age 1.5\u2009years was higher in children treated in early infancy with OA compared to historical controls. Significant heterogeneity existed across study population characteristics and outcome measures. Nusinersen may prevent deterioration in some nutrition outcomes; and OA in early infancy may be associated with improved nutrition outcomes. Timing of DMT initiation is an important consideration for future nutrition research. Studies investigating nutrition as a primary outcome of DMT, using consistent outcome measures are required for nutritional management strategies for this cohort to be appropriately tailored.\n                  <\/jats:p>","DOI":"10.1002\/mus.28224","type":"journal-article","created":{"date-parts":[[2024,8,12]],"date-time":"2024-08-12T01:24:00Z","timestamp":1723425840000},"page":"890-902","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Nutrition outcomes of disease modifying therapies in spinal muscular atrophy: A systematic review"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7886-551X","authenticated-orcid":false,"given":"Katie","family":"O'Brien","sequence":"first","affiliation":[{"name":"Department of Nutrition, Dietetics and Food Monash University, Faculty of Medicine Nursing and Health Sciences  Melbourne Australia"},{"name":"Department of Nutrition and Food Services Royal Children's Hospital  Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay","family":"Nguo","sequence":"additional","affiliation":[{"name":"Department of Nutrition, Dietetics and Food Monash University, Faculty of Medicine Nursing and Health Sciences  Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eppie M.","family":"Yiu","sequence":"additional","affiliation":[{"name":"Department of Neurology Royal Children's Hospital  Melbourne Australia"},{"name":"Neurosciences Research, Murdoch Children's Research Institute  Melbourne Australia"},{"name":"Department of Paediatrics Faculty of Medicine Dentistry and Health Sciences, University of Melbourne  Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian R.","family":"Woodcock","sequence":"additional","affiliation":[{"name":"Department of Neurology Royal Children's Hospital  Melbourne Australia"},{"name":"Neurosciences Research, Murdoch Children's Research Institute  Melbourne Australia"},{"name":"Department of Paediatrics Faculty of Medicine Dentistry and Health Sciences, University of Melbourne  Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8566-9341","authenticated-orcid":false,"given":"Natassja","family":"Billich","sequence":"additional","affiliation":[{"name":"Molecular Therapies Research, Murdoch Children's Research Institute  Melbourne Australia"},{"name":"The University of Queensland School of Human Movement and Nutrition Sciences  St Lucia Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoe E.","family":"Davidson","sequence":"additional","affiliation":[{"name":"Department of Nutrition, Dietetics and Food Monash University, Faculty of Medicine Nursing and Health Sciences  Melbourne Australia"},{"name":"Department of Neurology Royal Children's Hospital  Melbourne Australia"},{"name":"Neurosciences Research, Murdoch Children's Research Institute  Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8,11]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90460-3"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)60921-6"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305668"},{"key":"e_1_2_11_5_1","volume-title":"GeneReviews [Internet]","author":"Prior TW","year":"2000"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305788"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073807305673"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(12)70061-3"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.11.005"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2016.05.005"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26823"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2012.04.008"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2009.03.009"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2013.12.006"},{"key":"e_1_2_11_15_1","unstructured":"FDA approves first drug for spinal muscular atrophy.U.S. Food and Drug Administration Web site.https:\/\/www.fda.gov\/news\u2010events\/press\u2010announcements\/fda\u2010approves\u2010first\u2010drug\u2010spinal\u2010muscular\u2010atrophyUpdated March 28 2018. Accessed October 30 2023."},{"key":"e_1_2_11_16_1","unstructured":"Spinraza EPAR product information.European Medicines Agency Web site. Updated October 12 2023. Accessed October 30 2023. Available from:https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/spinraza-epar-product-information_en.pdf"},{"key":"e_1_2_11_17_1","unstructured":"FDA news release. FDA Approves Oral Treatment for Spinal Muscular Atrophy.U.S. Food and Drug Administration Web site.https:\/\/www.fda.gov\/news\u2010events\/press\u2010announcements\/fda\u2010approves\u2010oral\u2010treatment\u2010spinal\u2010muscular\u2010atrophyUpdated August 7 2020. Accessed October 30 2023."},{"key":"e_1_2_11_18_1","unstructured":"An overview of Evrysdi and why it is authorised in the EU.European Medicines Agency Web site.https:\/\/www.ema.europa.eu\/en\/documents\/overview\/evrysdi\u2010epar\u2010medicine\u2010overview_en.pdfUpdated September 22 2023. Accessed October 30 2023."},{"key":"e_1_2_11_19_1","unstructured":"FDA approves innovative gene therapy to treat pediatric patients with spinal muscular atrophy a rare disease and leading genetic cause of infant mortality.U.S. Food and Drug Administration Web site.https:\/\/www.fda.gov\/news\u2010events\/press\u2010announcements\/fda\u2010approves\u2010innovative\u2010gene\u2010therapy\u2010treat\u2010pediatric\u2010patients\u2010spinal\u2010muscular\u2010atrophy\u2010rare\u2010diseaseUpdated May 24 2019. Accessed 30 October 2023."},{"key":"e_1_2_11_20_1","unstructured":"An overview of Zolgensma and why it is authorised in the EU.European Medicines Agency Web site.https:\/\/www.ema.europa.eu\/en\/documents\/overview\/zolgensma\u2010epar\u2010medicine\u2010overview_en.pdfUpdated March 17 2023. 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