{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,6,30]],"date-time":"2024-06-30T01:36:00Z","timestamp":1719711360884},"reference-count":20,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[1995,9,8]],"date-time":"1995-09-08T00:00:00Z","timestamp":810518400000},"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":[[1996,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Classical phenylketonuria (PKU) (McKusick 261600) is an inborn error of metabolism treated by a controlled low\u2010phenylalanine (Phe) diet started as soon as possible in the first days of life. Such a diet can be achieved with vegetable protein and can be considered non\u2010atherogenic because of the reduction of animal products. Thirty patients with PKU were classified into two groups according to their annual mean Phe levels. Their daily protein intake was largely replaced by PKU2 Milupa which contains a mixture of amino acids. The product has no phenylalanine or fat of any kind. Thirty\u2010eight (38) individuals of comparable age were used as controls. Group A (<jats:italic>n<\/jats:italic>=15) had good compliance with the special diet (Phe mean 192\u00b1115 \u00b5mol\/L); group B (<jats:italic>n<\/jats:italic>=15) did not strictly adhere to the diet (Phe mean 595\u00b1263 \u00b5mol\/L). Certain haemostatic components (factors I, VII, VIII, and X, antithrombin III, protein C, and plasminogen) and lipid variables (cholesterol, triglycerides, high\u2010density lipoprotein, low\u2010density lipoprotein, very\u2010low\u2010density lipoprotein) as well as Phe levels were estimated. All the haemostatic factors studied were found within the normal range with the exception of a significant reduction in protein C in both groups of PKU patients. Furthermore, a statistically significant reduction in factor VII and X concentrations was observed in patients on strict diet. Cholesterol and low\u2010density lipoprotein concentrations were significantly lower in PKU children compared to normal controls. It is suggested that even though the special diet of PKU children, especially in group A, is rich in vegetables, the reduced fat intake might have impaired the absorption of vitamin K and its delivery to the site of synthesis of vitamin K\u2010dependent haemostatic factors.<\/jats:p>","DOI":"10.1007\/bf01799833","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T16:38:06Z","timestamp":1119371886000},"page":"603-609","source":"Crossref","is-referenced-by-count":5,"title":["Haemostatic variables in phenylketonuric children under dietary treatment"],"prefix":"10.1002","volume":"19","author":[{"given":"K. H.","family":"Schulpis","sequence":"first","affiliation":[{"name":"Institute of Child Health \u2018Aghia Sophia\u2019 Children's Hospital  Athens 11527 Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Platokouki","sequence":"additional","affiliation":[{"name":"Haemostasis Unit \u2018Aghia Sophia\u2019 Children's Hospital  Athens 11527 Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. D.","family":"Papakonstantinou","sequence":"additional","affiliation":[{"name":"Institute of Child Health \u2018Aghia Sophia\u2019 Children's Hospital  Athens 11527 Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Adamtziki","sequence":"additional","affiliation":[{"name":"Haemostasis Unit \u2018Aghia Sophia\u2019 Children's Hospital  Athens 11527 Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Bargeliotis","sequence":"additional","affiliation":[{"name":"Haemostasis Unit \u2018Aghia Sophia\u2019 Children's Hospital  Athens 11527 Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Aronis","sequence":"additional","affiliation":[{"name":"Haemostasis Unit \u2018Aghia Sophia\u2019 Children's Hospital  Athens 11527 Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1995,9,8]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1016\/S0002-8223(21)09775-3","article-title":"Serum lipids in children with phenylketonuria","volume":"53","author":"Acosta P","year":"1973","journal-title":"J Am Diet Assoc"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(83)80007-9"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/48.3.712"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.281.6246.973"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/36.5.890"},{"key":"e_1_2_1_7_1","first-page":"386","volume-title":"Diets for Sick Children","author":"Francis D","year":"1986"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01811492"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(80)90413-2"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(87)90063-9"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/40.3.468"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(80)91498-1"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(57)90972-8"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9150(86)90174-7"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/58.3.354"},{"key":"e_1_2_1_16_1","volume-title":"The Composition of Food","author":"Paul AA","year":"1980"},{"key":"e_1_2_1_17_1","first-page":"498","article-title":"Diet discontinuation policies and practices of PKU\u2010clinics in the USA","volume":"70","author":"Schuelt V","year":"1980","journal-title":"Am J Dis Child"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/000992288902801007"},{"key":"e_1_2_1_19_1","first-page":"2801","article-title":"Dietary fiber, lipid metabolism and atherosclerosis","volume":"41","author":"Vahouny GV","year":"1982","journal-title":"Fedn Proc"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02312549"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(90)90218-H"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01799833","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01799833","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T11:44:34Z","timestamp":1694778274000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF01799833"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,9,8]]},"references-count":20,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1996,9]]}},"alternative-id":["10.1007\/BF01799833"],"URL":"https:\/\/doi.org\/10.1007\/bf01799833","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1995,9,8]]}}}