| Tyrosine supplementation for phenylketonuria. | |
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MedLine Citation:
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PMID: 20687067 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Phenylketonuria is an inherited disease for which the main treatment is the dietary restriction of the amino acid phenylalanine. The diet has to be initiated in the neonatal period to prevent or reduce mental handicap. However, the diet is very restrictive and unpalatable and can be difficult to follow. A deficiency of the amino acid tyrosine has been suggested as a cause of some of the neuropsychological problems exhibited in phenylketonuria. Therefore, this review aims to assess the efficacy of tyrosine supplementation for phenylketonuria. OBJECTIVES: To assess the effects of tyrosine supplementation alongside or instead of a phenylalanine-restricted diet for people with phenylketonuria, who commenced on diet at diagnosis and either continued on the diet or relaxed the diet later in life. To assess the evidence that tyrosine supplementation alongside, or instead of a phenylalanine-restricted diet improves intelligence, neuropsychological performance, growth and nutritional status, mortality rate and quality of life. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register which is comprised of references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. Additional studies were identified from handsearches of the Journal of Inherited Metabolic Disease (from inception in 1978 to 1998). The manufacturers of prescribable dietary products used in the treatment of phenylketonuria were also contacted for further references.Date of the most recent search of the Group's Inborn Errors of Metabolism Trials Register: 09 June 2010. SELECTION CRITERIA: All randomised or quasi-randomised trials investigating the use of tyrosine supplementation versus placebo in people with phenylketonuria in addition to, or instead of, a phenylalanine-restricted diet. People treated for maternal phenylketonuria were excluded. DATA COLLECTION AND ANALYSIS: Two authors independently assessed the trial eligibility, methodological quality and extracted the data. MAIN RESULTS: Six trials were found, of which three trials reporting the results of a total of 56 participants, were suitable for inclusion in the review. The blood tyrosine concentrations were significantly higher in the participants receiving tyrosine supplements than those in the placebo group, mean difference 23.46 (95% confidence interval 12.87 to 34.05). No significant differences were found between any of the other outcomes measured. AUTHORS' CONCLUSIONS: From the available evidence no recommendations can be made about whether tyrosine supplementation should be introduced into routine clinical practice. Further randomised controlled studies are required to provide more evidence. |
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Authors:
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Diana Webster; Joanne Wildgoose |
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Publication Detail:
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Type: Journal Article; Meta-Analysis; Review Date: 2010-08-04 |
Journal Detail:
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Title: Cochrane database of systematic reviews (Online) Volume: - ISSN: 1469-493X ISO Abbreviation: Cochrane Database Syst Rev Publication Date: 2010 |
Date Detail:
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Created Date: 2010-08-05 Completed Date: 2010-08-25 Revised Date: 2010-10-21 |
Medline Journal Info:
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Nlm Unique ID: 100909747 Medline TA: Cochrane Database Syst Rev Country: England |
Other Details:
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Languages: eng Pagination: CD001507 Citation Subset: IM |
Affiliation:
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Nutrition and Dietetic Department, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, UK, BS2 8BJ. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Dietary Supplements* Humans Intelligence / drug effects Neuropsychological Tests Phenylalanine / blood Phenylketonurias / blood, diet therapy, drug therapy* Randomized Controlled Trials as Topic Tyrosine / blood, therapeutic use* |
| Chemical | |
Reg. No./Substance:
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55520-40-6/Tyrosine; 63-91-2/Phenylalanine |
| Comments/Corrections | |
Update Of:
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Cochrane Database Syst Rev. 2000;(2):CD001507
[PMID:
10796799
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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