| Nutritional issues in treating phenylketonuria. | |
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MedLine Citation:
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PMID: 20151202 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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A phenylalanine (Phe)-restricted diet is the mainstay of phenylketonuria (PKU) treatment, and, in recent years, the nutritional management of PKU has become more complex in order to optimize patients' growth, development and diet compliance. Dietary restriction of Phe creates a diet similar to a vegan diet, and many of the nutritional concerns and questions applicable to vegans who wish to avoid animal products are also relevant to patients with PKU. Owing to their nutritional characteristics, breast milk and breastfeeding should be given greater consideration as a useful food in patients with PKU and in those with other inborn errors of metabolism. Further key issues for consideration include the quality of the available amino acid substitutes, the neurotrophic and neuroprotective effects of added long-chain polyunsaturated fatty acids (e.g. docosahexaenoic acid), micronutrient deficiencies, bone disease and antioxidant status. Long-term dietary guidance and monitoring of the nutritional status of patients with PKU should be part of a follow-up programme that continues for life. |
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Authors:
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François Feillet; Carlo Agostoni |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-02-12 |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: 33 ISSN: 1573-2665 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 2010 Dec |
Date Detail:
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Created Date: 2010-11-29 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: Netherlands |
Other Details:
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Languages: eng Pagination: 659-64 Citation Subset: IM |
Affiliation:
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Centre de référence des maladies héréditaires du métabolisme de Nancy, INSERM U 954, Hôpital d'Enfants, CHU Brabois, Allée du Morvan, Vandoeuvre les Nancy 54500, France. f.feillet@chu-nancy.fr |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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