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Since the first attempts at treatment using a low-phenylalanine diet and after more than 50 years of research, considerable progress has been made so we are now at a stage where mental retardation caused by high plasma phenylalanine can be prevented. We must, however, be aware of the new challenges we face in managing PKU. These include: maintaining optimal growth by providing enough phenylalanine without jeopardizing the child's psychomotor development; providing an optimal nutritional status that ensures other essential nutrients, such as long chain polyunsaturated fatty acids, are not excluded from the diet; ensuring optimal compliance to the dietary intervention; and considering patients' quality of life. New strategies, such as tetrahydrobiopterin (BH4) supplementation, need to be evaluated with regard to safety, efficacy and expected outcomes in specific types of HPA.<\/jats:p>","DOI":"10.1177\/147323000703500602","type":"journal-article","created":{"date-parts":[[2013,2,27]],"date-time":"2013-02-27T14:22:43Z","timestamp":1361974963000},"page":"742-752","source":"Crossref","is-referenced-by-count":4,"title":["Treating Phenylketonuria: A Single Centre Experience"],"prefix":"10.1177","volume":"35","author":[{"given":"M","family":"Giovannini","sequence":"first","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"Riva","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"Salvatici","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L","family":"Fiori","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Paci","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"Verduci","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C","family":"Agostoni","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, San Paolo Hospital, University of Milan, Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2007,11,1]]},"reference":[{"key":"bibr1-147323000703500602","first-page":"1015","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Scriver CR","year":"1995","edition":"7"},{"key":"bibr2-147323000703500602","doi-asserted-by":"publisher","DOI":"10.1007\/PL00014253"},{"key":"bibr3-147323000703500602","doi-asserted-by":"publisher","DOI":"10.1007\/BF01963563"},{"key":"bibr4-147323000703500602","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1052440"},{"key":"bibr5-147323000703500602","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.306.6870.115"},{"key":"bibr6-147323000703500602","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799703"},{"key":"bibr7-147323000703500602","unstructured":"Agostoni C, Riva E, Valsasina R, Plasma and urine levels of taurine in PKU children and in a normal control population. 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