{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T19:57:49Z","timestamp":1769198269723,"version":"3.49.0"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2007,6,21]],"date-time":"2007-06-21T00:00:00Z","timestamp":1182384000000},"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":[[2007,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Lysinuric protein intolerance (LPI) is a rare autosomal recessive disorder characterized by defective transport of cationic amino acids. Poor intestinal absorption and increased renal loss of arginine, ornithine and lysine lead to low plasma concentrations of these amino acids and, subsequently, to impaired urea cycle function. The patients therefore have decreased nitrogen tolerance, which may lead to hyperammonaemia after ingestion of normal amounts of dietary protein. As a protective mechanism, most patients develop strong aversion to protein\u2010rich foods early in life. Oral supplementation with citrulline, which is absorbed normally and metabolized to arginine and ornithine, improves protein tolerance to some extent, as do sodium benzoate and sodium phenylbutyrate also used by some patients. Despite effective prevention of hyperammonaemia, the patients still consume a very restricted diet, which may be deficient in energy, essential amino acids and some vitamins and minerals. To investigate the potential nutritional problems of patients with lysinuric protein intolerance, 77 three\u2010 to four\u2010day food records of 28 Finnish LPI patients aged 1.5\u201361 years were analysed. The data suggest that the patients are clearly at risk for many nutritional deficiencies, which may contribute to their symptoms. Their diet is highly deficient in calcium, vitamin D, iron and zinc. Individualized nutritional supplementation accompanied by regular monitoring of dietary intake is therefore an essential part of the treatment of LPI.<\/jats:p>","DOI":"10.1007\/s10545-007-0558-2","type":"journal-article","created":{"date-parts":[[2007,6,21]],"date-time":"2007-06-21T00:16:51Z","timestamp":1182385011000},"page":"716-721","source":"Crossref","is-referenced-by-count":29,"title":["Nutrient intake in lysinuric protein intolerance"],"prefix":"10.1002","volume":"30","author":[{"given":"L. M.","family":"Tanner","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Turku Kiinamyllynkatu 4\u20138 Turku 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"N\u00e4nt\u00f6\u2010Salonen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Turku Kiinamyllynkatu 4\u20138 Turku 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Venetoklis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Turku University Hospital Turku Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Kotilainen","sequence":"additional","affiliation":[{"name":"Research Centre of Applied and Preventive Cardiovascular Medicine University of Turku Turku Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Niinikoski","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Turku Kiinamyllynkatu 4\u20138 Turku 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Huoponen","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics University of Turku Turku Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"O.","family":"Simell","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Turku Kiinamyllynkatu 4\u20138 Turku 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,6,21]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198501313120506"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01955906"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.HYP.29.4.913"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0308-8146(96)00077-5"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1983.03340030030023"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/68.4.859"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/72.1.168"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/69.3.516"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00710296"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.104.4.1176"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0046-8177(94)90150-3"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(65)92446-3"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(80)91679-7"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.21.6.519"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(80)80422-7"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/0026-0495(81)90211-0"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)11683-7"},{"key":"e_1_2_1_19_1","first-page":"4933","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Simell O","year":"2001"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(75)90358-7"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00191519"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.metabol.2005.08.016"},{"key":"e_1_2_1_23_1","doi-asserted-by":"publisher","DOI":"10.1210\/edrv-15-1-80"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/6809"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-007-0558-2","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-007-0558-2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T05:01:34Z","timestamp":1694754094000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-007-0558-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,6,21]]},"references-count":23,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2007,10]]}},"alternative-id":["10.1007\/s10545-007-0558-2"],"URL":"https:\/\/doi.org\/10.1007\/s10545-007-0558-2","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,6,21]]}}}