{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T08:46:42Z","timestamp":1788425202763,"version":"build-2803163510"},"reference-count":12,"publisher":"Wiley","issue":"S3","license":[{"start":{"date-parts":[[2010,3,19]],"date-time":"2010-03-19T00:00:00Z","timestamp":1268956800000},"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":[[2010,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>At the Kennedy Centre for Phenylketonuria, Denmark, large neutral amino acids (LNAAs) are being used to treat adult and adolescent patients who are nonadherent to dietary treatment for phenylketonuria (PKU). At the start of treatment, a patient must undergo dietary analysis and regular blood sampling to measure plasma amino acid (AA) concentrations. The aim of this analysis and treatment is that the patient receives 25\u201330% of the daily protein requirement from LNAA supplementation and the remaining 70\u201375% from natural, low\u2010phenylalanine proteins (although some patients have difficulties in maintaining this level of protein intake). Patients are therefore able to follow a more \u201cnormal\u201d diet than those adhering to a PKU diet with AA supplementation (in which only 20% of the daily protein requirement is provided from the diet and 80% from AA supplementation). LNAAs have also been used to treat older patients with untreated\/late\u2010diagnosed PKU who show profound intellectual, psychological, and behavioral impairments. Treatment with LNAAs has been shown to improve measures of concentration and awareness of external stimuli in some of these patients and thus enhance their socialization, emotionality, frustration tolerance, and mood.<\/jats:p>","DOI":"10.1007\/s10545-010-9069-7","type":"journal-article","created":{"date-parts":[[2010,3,18]],"date-time":"2010-03-18T05:52:21Z","timestamp":1268891541000},"page":"187-190","source":"Crossref","is-referenced-by-count":11,"title":["Large neutral amino acids in daily practice"],"prefix":"10.1002","volume":"33","author":[{"given":"Kirsten Ki\u00e6r","family":"Ahring","sequence":"first","affiliation":[{"name":"Kennedy Institute Centre for PKU Gl. Landevej 7 Glostrup 2600 Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,3,19]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1976.00500100018008"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0552-8"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799701"},{"key":"e_1_2_8_5_1","first-page":"55","article-title":"Fasting serum phenylalanine in untreated institutionalised patients with phenylketonuria","volume":"21","author":"G\u00fcttler F","year":"1977","journal-title":"J Ment Defic Res"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2005.tb02078.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(85)90250-8"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1987.tb10521.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-006-0395-8"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI5017"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20526"},{"key":"e_1_2_8_12_1","first-page":"1667","volume-title":"The metabolic and molecular bases of inherited disease","author":"Scriver CR","year":"2001"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1987.tb01039.x"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-010-9069-7","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T02:21:27Z","timestamp":1694744487000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-010-9069-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,3,19]]},"references-count":12,"journal-issue":{"issue":"S3","published-print":{"date-parts":[[2010,12]]}},"alternative-id":["10.1007\/s10545-010-9069-7"],"URL":"https:\/\/doi.org\/10.1007\/s10545-010-9069-7","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,3,19]]}}}