{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T14:30:06Z","timestamp":1782138606549,"version":"3.54.5"},"reference-count":10,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2004,11,25]],"date-time":"2004-11-25T00:00:00Z","timestamp":1101340800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2005,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p><jats:bold>Aim<\/jats:bold>.The aim was to study the efficacy and acceptability of MSUD Express in adolescent and adult patients with maple syrup urine disease (MSUD). <jats:bold>Background<\/jats:bold>. There are difficulties associated with current protein substitutes designed for adolescents and adults with MSUD. This can affect their metabolic control. MSUD Express is a new low\u2010volume, nutritionally complete protein substitute, free from leucine, isoleucine and valine and designed specifically for older patients with MSUD. <jats:bold>Methods<\/jats:bold>. Four patients with MSUD were included in the trial (aged 16\u201341 years). Product dosage was 4\u00d7 25 g sachets per day, providing 1264 kJ and 72 g amino acids: more than adequately meeting requirements for all micronutrients. <jats:bold>Results<\/jats:bold>. At the start of the study, the mean (range) of the previous eight leucine concentrations (on MSUD Aid III) was 564 \u03bcmol\/L (430\u2013817 \u03bcmol\/L) and during the study (on MSUD Express) was 382 \u03bcmol\/L (181\u2013603 \u03bcmol\/L). The average percentage fall in mean leucine concentrations was 32%. In patients 2, 3 and 4, low leucine levels resulted in an increase in the natural protein allowance per day. MSUD Express was rated as \u2018excellent\u2019 or \u2018good\u2019 by all patients on the basis of appearance, taste, smell and texture. All patients found the product \u2018very easy\u2019 to prepare and found it \u2018easy\u2019 or \u2018very easy\u2019 to take outside the home. <jats:bold>Conclusion<\/jats:bold>. All patients thought the preparation of MSUD Express was \u2018very easy\u2019. Metabolic control (mean leucine concentrations) improved in all of the patients. In three patients this meant that an increase in the amount of natural protein in the diet was possible. All patients elected to stay on MSUD Express after the trial period. MSUD Express appears to be an effective and acceptable product, offering an alternative to current protein substitutes available for older people with MSUD.<\/jats:p>","DOI":"10.1007\/s10545-005-0061-6","type":"journal-article","created":{"date-parts":[[2005,9,7]],"date-time":"2005-09-07T19:05:23Z","timestamp":1126119923000},"page":"665-672","source":"Crossref","is-referenced-by-count":5,"title":["A new protein substitute for adolescents and adults with maple syrup urine disease (MSUD)"],"prefix":"10.1002","volume":"28","author":[{"given":"P.","family":"Hallam","sequence":"first","affiliation":[{"name":"The Charles Dent Metabolic Unit, The National Hospital for Neurology {&amp;} Neurosurgery University College Hospitals NHS Trust  London UK"},{"name":"The Charles Dent Metabolic Unit The National Hospital for Neurology and Neurosurgery  Queen Square London WC1N 3BG"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Lilburn","sequence":"additional","affiliation":[{"name":"The Charles Dent Metabolic Unit, The National Hospital for Neurology {&amp;} Neurosurgery University College Hospitals NHS Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. J.","family":"Lee","sequence":"additional","affiliation":[{"name":"The Charles Dent Metabolic Unit, The National Hospital for Neurology {&amp;} Neurosurgery University College Hospitals NHS Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,11,25]]},"reference":[{"issue":"6","key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1542\/peds.68.6.856","article-title":"Outcome of early and long\u2010term management of classical maple syrup urine disease","volume":"68","author":"Clow CL","year":"1981","journal-title":"Pediatrics"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/9780470690369.ch15"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)81037-6"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000028784.11080.9b"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.109.6.999"},{"key":"e_1_2_1_7_1","first-page":"138","volume-title":"Atlas of Metabolic Diseases","author":"Nyhan WL","year":"1998"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-662-04285-4_16"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.68.3.426"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1442-200X.1988.tb02531.x"},{"key":"e_1_2_1_11_1","unstructured":"Van WykK(2000)Specialist dietitian\u2014metabolic disease.Maple Syrup Urine Disease\u2014Practical Dietary Management. Great Ormond Street Hospital for Children NHS Trust in SHS Inborn Error Review Series No.10.."}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-005-0061-6","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-005-0061-6","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T01:11:54Z","timestamp":1694740314000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-005-0061-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,11,25]]},"references-count":10,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2005,9]]}},"alternative-id":["10.1007\/s10545-005-0061-6"],"URL":"https:\/\/doi.org\/10.1007\/s10545-005-0061-6","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,11,25]]}}}