{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T14:09:13Z","timestamp":1793196553254,"version":"4.5.0"},"reference-count":21,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2012,5,18]],"date-time":"2012-05-18T00:00:00Z","timestamp":1337299200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Human Nutrition Diet"],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:sec><jats:label\/><jats:p>Adam S., Champion H., Daly A., Dawson S., Dixon M., Dunlop C., Eardley J., Evans S., Ferguson C., Jankowski C., Lowry S., MacDonald A., Maritz C., Micciche A., Robertson L., Stafford J., Terry A., Thom R., van Wyk K., Webster D., White F.J. &amp; Wildgoose J. on behalf of the British Inherited Metabolic Diseases Group (BIMDG) Dietitian's Group. (2012) Dietary management of urea cycle disorders: UK practice.<jats:italic>J Hum Nutr Diet.<\/jats:italic><jats:bold>25<\/jats:bold>, 398\u2013404<\/jats:p><\/jats:sec><jats:sec><jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background:\u2002<\/jats:bold>There is no published data describing UK dietary management of urea cycle disorders (UCD). The present study describes dietary practices in UK inherited metabolic disorder (IMD) centres.<\/jats:p><jats:p><jats:bold>Methods:\u2002<\/jats:bold>Cross\u2010sectional data from 16 IMD centres were collected by a questionnaire describing the management of UCD patients on prescribed protein\u2010restricted diets.<\/jats:p><jats:p><jats:bold>Results:\u2002<\/jats:bold>One hundred and seventy\u2010five patients [<jats:italic>N<\/jats:italic>\u2010acetylglutamate synthase deficiency,<jats:italic>n<\/jats:italic>\u2003=\u20033; carbamoyl phosphate synthase deficiency (CPS),<jats:italic>n<\/jats:italic>\u2003=\u20038; ornithine transcarbamoylase deficiency (OTC),<jats:italic>n<\/jats:italic>\u2003=\u200375; citrullinaemia,<jats:italic>n<\/jats:italic>\u2003=\u200341; argininosuccinic aciduria (ASA),<jats:italic>n<\/jats:italic>\u2003=\u200336; arginase deficiency,<jats:italic>n<\/jats:italic>\u2003=\u200312] were reported; 70% (<jats:italic>n<\/jats:italic>\u2003=\u2003123) aged 0\u201316\u2003years; 30% (<jats:italic>n<\/jats:italic>\u2003=\u200352) &gt;16\u2003years. Prescribed median protein intake decreased with age (0\u20136\u2003months: 2\u2003g\u2003kg<jats:sup>\u22121<\/jats:sup>\u2003day<jats:sup>\u22121<\/jats:sup>; 7\u201312\u2003months: 1.6\u2003g\u2003kg<jats:sup>\u22121<\/jats:sup>\u2003day<jats:sup>\u22121<\/jats:sup>; 1\u201310\u2003years: 1.3\u2003g\u2003kg<jats:sup>\u22121<\/jats:sup>\u2003day<jats:sup>\u22121<\/jats:sup>; 11\u201316\u2003years: 0.9\u2003g\u2003kg<jats:sup>\u22121<\/jats:sup>\u2003day<jats:sup>\u22121<\/jats:sup>and &gt;16\u2003years: 0.8\u2003g\u2003kg<jats:sup>\u22121<\/jats:sup>\u2003day<jats:sup>\u22121<\/jats:sup>) with little variation between disorders. Adult protein prescription ranged 0.4\u20131.2\u2003g\u2003kg<jats:sup>\u22121<\/jats:sup>\u2003day<jats:sup>\u22121<\/jats:sup>(40\u201360\u2003g\u2003day<jats:sup>\u22121<\/jats:sup>). In the previous 2\u2003years, 30% (<jats:italic>n<\/jats:italic>\u2003=\u200353) were given essential amino acid supplements (EAAs) (CPS,<jats:italic>n<\/jats:italic>\u2003=\u20032; OTC, n\u2003=\u200320; citrullinaemia,<jats:italic>n<\/jats:italic>\u2003=\u200315; ASA,<jats:italic>n<\/jats:italic>\u2003=\u20037; arginase deficiency,<jats:italic>n<\/jats:italic>\u2003=\u20039). EAAs were prescribed for low plasma quantitative essential amino acids (<jats:italic>n<\/jats:italic>\u2003=\u200313 centres); inadequate natural protein intake (<jats:italic>n<\/jats:italic>\u2003=\u200311) and poor metabolic control (<jats:italic>n<\/jats:italic>\u2003=\u20039). From diagnosis, one centre prescribed EAAs for all patients and one centre for severe defects only. Only 3% (<jats:italic>n<\/jats:italic>\u2003=\u20036) were given branch chain amino acid supplements. Enteral feeding tubes were used by 25% (<jats:italic>n<\/jats:italic>\u2003=\u200344) for feeds and 3% (<jats:italic>n<\/jats:italic>\u2003=\u20036) for medications. Oral energy supplements were prescribed in 17% (<jats:italic>n<\/jats:italic>\u2003=\u200330) of cases.<\/jats:p><jats:p><jats:bold>Conclusions:\u2002<\/jats:bold>In the UK, protein restriction based on World Health Organization \u2018safe intakes of protein\u2019, is the principle dietary treatment for UCD. EAA supplements are prescribed mainly on clinical need. Multicentre collaborative research is required to define optimal dietary treatments.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1365-277x.2012.01259.x","type":"journal-article","created":{"date-parts":[[2012,5,18]],"date-time":"2012-05-18T06:09:33Z","timestamp":1337321373000},"page":"398-404","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Dietary management of urea cycle disorders: UK practice"],"prefix":"10.1111","volume":"25","author":[{"given":"S.","family":"Adam","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Champion","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Daly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Dawson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Dixon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Dunlop","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Eardley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Evans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Ferguson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Jankowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Lowry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"MacDonald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Maritz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Micciche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Robertson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Stafford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Terry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Thom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"van Wyk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Webster","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. 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