| Optimising growth in phenylketonuria: Current state of the clinical evidence base. | |
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MedLine Citation:
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PMID: 21959353 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Patients with phenylketonuria (PKU) must follow a strict low-phenylalanine (Phe) diet in order to minimise the potentially disabling neuropsychological sequelae of the disorder. Research in this area has unsurprisingly focussed largely on managing blood Phe concentrations to protect the brain. Protein requirements in dietary management of PKU are met mostly from Phe-free protein substitutes with the intake of natural protein restricted to patient tolerance. Several reports have suggested that growth in early childhood in PKU is sub-optimal, relative to non-PKU control groups or reference populations. We reviewed the literature searching for evidence regarding PKU and growth as well as possible links between dietary management of PKU and growth. The search retrieved only limited evidence on the effect of PKU and its dietary management on growth. Physical development in PKU remains an under-studied aspect of this disorder. |
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Authors:
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Katharina Dokoupil; Hulya Gokmen-Ozel; Anna Maria Lammardo; Kristina Motzfeldt; Martine Robert; Júlio César Rocha; Margreet van Rijn; Kirsten Ahring; Amaya Bélanger-Quintana; Anita Macdonald |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-9-27 |
Journal Detail:
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Title: Clinical nutrition (Edinburgh, Scotland) Volume: - ISSN: 1532-1983 ISO Abbreviation: - Publication Date: 2011 Sep |
Date Detail:
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Created Date: 2011-9-30 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8309603 Medline TA: Clin Nutr Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved. |
Affiliation:
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Department of Metabolism and Nutrition, Dr von Hauner Children's Hospital, University of Munich, Munich, Germany. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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