| Reassessment of phenylalanine tolerance in adults with phenylketonuria is needed as body mass changes. | |
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MedLine Citation:
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PMID: 19747868 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Lifelong treatment of phenylketonuria (PKU) includes a phenylalanine (phe) restricted diet that provides sufficient phe for growth and maintenance plus phe-free amino acid formula to meet requirements for protein, energy and micronutrients. Phe tolerance (mg phe/kg body weight/day) is the amount of phe those with PKU can consume and maintain acceptable blood phe levels; it requires individual assessment because of varying phenylalanine hydroxylase activity. The objective was to reassess phe tolerance in eight adults with PKU considering phe requirements, blood phe levels, genotype and phe tolerance at 5 years of age. Subjects had not received a personalized assessment of phe tolerance in several years, and five subjects were overweight, body mass index (BMI) 25-28. With the guidance of a metabolic dietitian, seven subjects increased phe tolerance (by 15-173%) without significantly increasing blood phe concentration. Increased phe tolerance was associated with both improved dietary compliance and inadequate phe intake at the onset of the protocol compared with current requirements. Improved dietary compliance reflected increased consumption of protein equivalents from amino acid formula and increased frequency of formula intake, from 2.2 to 3 times per day. Predictors of higher final phe tolerance following reassessment included being male and having a lower BMI (R(2)=0.588). This suggests that the rising trend of overweight and obesity may affect assessment of phe tolerance in adults. Therefore, interaction with the metabolic dietitian to reassess phe tolerance in relation to body mass is essential throughout adulthood to insure adequate intake of phe to support protein synthesis and prevent catabolism. |
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Authors:
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Erin L MacLeod; Sally T Gleason; Sandra C van Calcar; Denise M Ney |
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Publication Detail:
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Type: Clinical Trial; Journal Article; Research Support, N.I.H., Extramural Date: 2009-08-08 |
Journal Detail:
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Title: Molecular genetics and metabolism Volume: 98 ISSN: 1096-7206 ISO Abbreviation: Mol. Genet. Metab. Publication Date: 2009 Dec |
Date Detail:
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Created Date: 2009-11-09 Completed Date: 2010-02-03 Revised Date: 2011-03-03 |
Medline Journal Info:
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Nlm Unique ID: 9805456 Medline TA: Mol Genet Metab Country: United States |
Other Details:
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Languages: eng Pagination: 331-7 Citation Subset: IM |
Affiliation:
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Department of Nutritional Sciences, 1415 Linden Drive, University of Wisconsin, Madison, WI 53705, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adaptation, Physiological* Adult Body Weight* Diet Female Genotype Humans Male Phenylalanine / administration & dosage, blood* Phenylketonurias / blood*, diet therapy, genetics, physiopathology* Young Adult |
| Grant Support | |
ID/Acronym/Agency:
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P30-HD-03352/HD/NICHD NIH HHS; R03 DK071534-01/DK/NIDDK NIH HHS; R03 DK071534-02/DK/NIDDK NIH HHS; R03 DK071534-02S1/DK/NIDDK NIH HHS; R03-DK-071534/DK/NIDDK NIH HHS |
| Chemical | |
Reg. No./Substance:
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63-91-2/Phenylalanine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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