{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T04:59:40Z","timestamp":1770267580532,"version":"3.49.0"},"reference-count":54,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T00:00:00Z","timestamp":1618531200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010887","name":"Vitaflo","doi-asserted-by":"publisher","award":["001"],"award-info":[{"award-number":["001"]}],"id":[{"id":"10.13039\/100010887","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Protein quality and quantity are important factors in determining lean body (muscle) mass (LBM). In phenylketonuria (PKU), protein substitutes provide most of the nitrogen, either as amino acids (AA) or glycomacropeptide with supplementary amino acids (CGMP-AA). Body composition and growth are important indicators of long-term health. In a 3-year prospective study comparing the impact of AA and CGMP-AA on body composition and growth in PKU, 48 children were recruited. N = 19 (median age 11.1 years, range 5\u201315 years) took AA only, n = 16 (median age 7.3 years, range 5\u201315 years) took a combination of CGMP-AA and AA, (CGMP50) and 13 children (median age 9.2 years, range 5\u201316 years) took CGMP-AA only (CGMP100). A dual energy X-ray absorptiometry (DXA) scan at enrolment and 36 months measured LBM, % body fat (%BF) and fat mass (FM). Height was measured at enrolment, 12, 24 and 36 months. No correlation or statistically significant differences (after adjusting for age, gender, puberty and phenylalanine blood concentrations) were found between the three groups for LBM, %BF, FM and height. The change in height z scores, (AA 0, CGMP50 +0.4 and CGMP100 +0.7) showed a trend that children in the CGMP100 group were taller, had improved LBM with decreased FM and % BF but this was not statistically significant. There appeared to be no advantage of CGMP-AA compared to AA on body composition after 3-years of follow-up. Although statistically significant differences were not reached, a trend towards improved body composition was observed with CGMP-AA when it provided the entire protein substitute requirement.<\/jats:p>","DOI":"10.3390\/nu13041323","type":"journal-article","created":{"date-parts":[[2021,4,19]],"date-time":"2021-04-19T06:35:53Z","timestamp":1618814153000},"page":"1323","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Growth and Body Composition in PKU Children\u2014A Three-Year Prospective Study Comparing the Effects of L-Amino Acid to Glycomacropeptide Protein Substitutes"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2579-8699","authenticated-orcid":false,"given":"Anne","family":"Daly","sequence":"first","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]},{"given":"Wolfgang","family":"H\u00f6gler","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Adolescent Medicine, Johannes Kepler University, Kepler University Hospital, Krankenhausstra\u00dfe 26-30, 4020 Linz, Austria"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4328-6304","authenticated-orcid":false,"given":"Nicola","family":"Crabtree","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]},{"given":"Nick","family":"Shaw","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7654-3621","authenticated-orcid":false,"given":"Sharon","family":"Evans","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]},{"given":"Alex","family":"Pinto","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]},{"given":"Richard","family":"Jackson","sequence":"additional","affiliation":[{"name":"Liverpool Clinical Trials Centre, University of Liverpool, Brownlow Hill, Liverpool L69 3GL, UK"}]},{"given":"Boyd J.","family":"Strauss","sequence":"additional","affiliation":[{"name":"School of Medical Sciences, Faculty of Biology, Medicine and Health Sciences, University of Manchester, Manchester M13 9PL, UK"},{"name":"School of Clinical Sciences, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, VIC 3800, Australia"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8953-2403","authenticated-orcid":false,"given":"Gisela","family":"Wilcox","sequence":"additional","affiliation":[{"name":"School of Medical Sciences, Faculty of Biology, Medicine and Health Sciences, University of Manchester, Manchester M13 9PL, UK"},{"name":"The Mark Holland Metabolic Unit, Salford Royal Foundation NHS Trust, Ladywell NW2, Salford, Manchester M6 8HD, UK"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4977-8345","authenticated-orcid":false,"given":"J\u00falio C.","family":"Rocha","sequence":"additional","affiliation":[{"name":"Nutrition and Metabolism, NOVA Medical School, Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, 1169-056 Lisboa, Portugal"},{"name":"Centre for Health and Technology and Services Research (CINTESIS), 4200-450 Porto, Portugal"}]},{"given":"Catherine","family":"Ashmore","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]},{"given":"Anita","family":"MacDonald","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK"}]}],"member":"1968","published-online":{"date-parts":[[2021,4,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1016\/j.diabres.2010.04.012","article-title":"The magnitude of association between overweight and obesity and the risk of diabetes: A meta-analysis of prospective cohort studies","volume":"89","author":"Abdullah","year":"2010","journal-title":"Diabetes Res. 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