{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T01:56:18Z","timestamp":1767923778529,"version":"3.49.0"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1997,8,1]],"date-time":"1997-08-01T00:00:00Z","timestamp":870393600000},"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":[[1997,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A new amino acid formulation and a variety of treatment products incorporating it were evaluated for long\u2010term safety, efficacy, and acceptance in 25 subjects with phenylketonuria over a period of 5 years. Palatability of the treatment was improved by reducing the required intake of amino acids, reformulating the mixture to have better taste, and providing vitamins and minerals as tablets. The hypotheses were that these strategies would improve compliance and metabolic control and maintain nutritional status in subjects. Compliance with treatment was determined from mean reported intakes (4\u2010day diet records) and from mean \u2018received\u2019 intakes using receipts of treatment products actually shipped to individuals upon request. Mean amino acid intakes prescribed were significantly reduced from study entry to end, from 1.2 g\/kg to 0.7 g\/kg (p &lt; 0.001). Reported intakes were similarly reduced from 1.3 g\/kg to 0.7 g\/kg (p &lt; 0.001). While actually \u2018received\u2019 intakes of amino acid formula were also significantly reduced (p &lt; 0.001), intakes by this measure were much lower than either prescribed or reported, 0.9 g\/kg at entry and 0.4 g\/kg at the end of the study, suggesting that acceptance of the treatment (usage of products), even when made more palatable, is below clinical expectations. In spite of these findings, mean serum proteins and minerals, height and weight were not significantly reduced during the study, supporting the safety of lowered intakes of amino acids and of nutritionally incomplete products. While the increase in mean serum phenylalanine concentration from 0.38 to 0.48 mmol\/L was significant (p &lt; 0.03), this mean rise of 0.1 mmol\/L during a corresponding mean age increase of 4.2 years (from 6.9 to 11.1 years) is lower than in other recent reports from longitudinal studies of outcomes during this age range in subjects treated with traditional products. These data support the safety and efficacy of a more palatable and flexible approach to treatment.<\/jats:p>","DOI":"10.1023\/a:1005337126669","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T21:48:34Z","timestamp":1040507314000},"page":"486-498","source":"Crossref","is-referenced-by-count":32,"title":["Treatment products and approaches for phenylketonuria: improved palatability and flexibility demonstrate safety, efficacy and acceptance in US clinical trials"],"prefix":"10.1002","volume":"20","author":[{"given":"A. P.","family":"Prince","sequence":"first","affiliation":[{"name":"Department of Chemistry Portland State University  USA"},{"name":"Divisions of Pediatrics\/Medical and Molecular Genetics, School of Medicine Oregon Health Sciences University  Portland Oregon USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. P.","family":"McMurry","sequence":"additional","affiliation":[{"name":"Department of Chemistry Portland State University  USA"},{"name":"Divisions of Pediatrics\/Medical and Molecular Genetics, School of Medicine Oregon Health Sciences University  Portland Oregon USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N. R. M.","family":"Buist","sequence":"additional","affiliation":[{"name":"Department of Chemistry Portland State University  USA"},{"name":"Divisions of Pediatrics\/Medical and Molecular Genetics, School of Medicine Oregon Health Sciences University  Portland Oregon USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1997,8]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"1","volume-title":"Ross Metabolic System Nutrition Support Protocols","author":"Acosta PB","year":"1993"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1177\/0148607181005005406"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0026-0495(73)90069-3"},{"key":"e_1_2_1_5_1","first-page":"35","article-title":"Intellectual development in 12\u2010year\u2010old children treated for phenylketonuria","volume":"145","author":"Azen CG","year":"1991","journal-title":"Am J Dis Child"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1080\/07315724.1993.10718348"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800019"},{"key":"e_1_2_1_8_1","first-page":"224","volume-title":"Diets for Sick Children","author":"Francis DEM","year":"1987"},{"key":"e_1_2_1_9_1","first-page":"23","volume-title":"Compliance in Health Care","author":"Gordis L","year":"1979"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.32.3.338"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/32.3.607"},{"key":"e_1_2_1_12_1","volume-title":"Development of Medical Foods for Rare Diseases: Proceedings of a Workshop","year":"1991"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800008"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/38.3.394"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.68.3.426"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1080\/03014469100001662"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/55.5.997"},{"key":"e_1_2_1_18_1","volume-title":"Recommended Dietary Allowances","year":"1989"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/32.9.1660"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00496411"},{"key":"e_1_2_1_21_1","first-page":"1015","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Scriver CR","year":"1995"},{"key":"e_1_2_1_22_1","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/S0002-8223(21)06859-0","article-title":"Trace element status of children with PKU and normal children","volume":"88","author":"Stepnick\u2010Gropper S","year":"1988","journal-title":"J Am Diet Assoc"},{"key":"e_1_2_1_23_1","first-page":"53","article-title":"Rapid determination of selenium in nutritional supplements by a flameless atomic absorption technique using a novel sample preparation","volume":"2","author":"Thompson DD","year":"1981","journal-title":"Atom Spectrosc"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005337126669","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005337126669","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T04:31:36Z","timestamp":1694752296000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005337126669"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,8]]},"references-count":22,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1997,8]]}},"alternative-id":["10.1023\/A:1005337126669"],"URL":"https:\/\/doi.org\/10.1023\/a:1005337126669","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1997,8]]}}}