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Characteristics of the diet include high\u2010carbohydrate and low\u2010fat content with added probiotics. However, because of insufficient evidenced\u2010based research, clinicians are unable to prescribe or endorse this diet. Our aim was to assess the tolerability of an adapted version of the traditional amino acid diet in children with SMA type I.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Children with SMA type I were recruited if they were enterally fed and experienced at least one gastrointestinal symptom (reflux, vomiting, constipation, and\/or diarrhea). Children were transitioned to an amino acid formula (Neocate Syneo\u2010Nutricia) for 8 weeks. Feeding tolerance was measured weekly by telephone consultation to monitor reflux, vomiting, stool consistency, and frequency.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen children were recruited, the mean age was 4.1 years (\u00b11.2\u2009SD), and 64% of participants were female. The mean resting energy expenditure determined by indirect calorimetry was 51.5\u2009kcal\/kg (\u00b17\u2009SD). The most common gastrointestinal complaint before switching to the amino acid formula was constipation, which was reported in 12 of 14 (85%) patients, of which 10 of the 12 (83%) children required daily stool softeners\/laxatives to help regulate bowel function. After 8 weeks on the amino acid formula, 10 out of 12 (83%) children stopped or reduced constipation medication.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Children with SMA type I who display gastrointestinal symptoms such as constipation and reflux may benefit from an amino acid formula that is fortified with probiotics.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ncp.10940","type":"journal-article","created":{"date-parts":[[2022,12,12]],"date-time":"2022-12-12T05:44:45Z","timestamp":1670823885000},"page":"871-880","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Open\u2010labelled study to monitor the effect of an amino acid formula on symptom management in children with spinal muscular atrophy type I: The SMAAF pilot study"],"prefix":"10.1002","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8625-9264","authenticated-orcid":false,"given":"Graeme","family":"O'Connor","sequence":"first","affiliation":[{"name":"Dietetic Department Great Ormond Street Hospital Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa","family":"Edel","sequence":"additional","affiliation":[{"name":"Physiotherapy Department Great Ormond Street Hospital Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"Raquq","sequence":"additional","affiliation":[{"name":"Developmental Neuroscience Research &amp; Teaching Department Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, NHS Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melissa","family":"Bowerman","sequence":"additional","affiliation":[{"name":"School of Medicine Keele University Keele Staffordshire UK"},{"name":"Wolfson Centre for Inherited Neuromuscular Disease Oswestry Shropshire UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Agnieszka","family":"Szmurlo","sequence":"additional","affiliation":[{"name":"Dietetic Department Great Ormond Street Hospital Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zoe","family":"Simpson","sequence":"additional","affiliation":[{"name":"Dietetic Department Great Ormond Street Hospital Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isobel","family":"Hardy","sequence":"additional","affiliation":[{"name":"Dietetic Department Great Ormond Street Hospital Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary","family":"Fewtrell","sequence":"additional","affiliation":[{"name":"Population, Policy &amp; Practice Department UCL Great Ormond Street Institute of Child Health, Faculty of Population Health Sciences London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Baranello","sequence":"additional","affiliation":[{"name":"Developmental Neuroscience Research &amp; Teaching Department Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, NHS Foundation Trust London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,12,12]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0671-8"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ncl.2015.07.004"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2020.01.003"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25533"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1706198"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.12.047"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2021.01.008"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu12123842"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.50855"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23582"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2018.04.024"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.3389\/fncel.2014.00025"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2012.01.004"},{"issue":"6","key":"e_1_2_9_15_1","first-page":"348","article-title":"Spinal muscular atrophy\u2014summary for nutritional care: the consensus statement for standard of care in spinal muscular atrophy. 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