| Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria. | |
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MedLine Citation:
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PMID: 2116544 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The biochemical and growth responses to dietary branched-chain amino acid (BCAA) intake were studied in two children; one with a disorder of branched-chain amino acid metabolism, maple syrup urine disease (MSUD) (McKusick 24860), and another with methylmalonic aciduria (MMA) (McKusick 25100). Biochemical control of MSUD focussed on plasma leucine levels while measurement of plasma ammonia levels was used in MMA. From 0 to 2.75 years both patients exhibited five episodes of toxicity. In each case toxicity was associated with dietary indiscretion or infection. The quantity of protein tolerated was always less in the MMA patient and was approximately 1 g/kg/day. From 1 to 2.75 years each patient's growth velocity approximated their predicted growth channel except during periods of toxicity. In both cases leucine intake, which gave normal growth without toxicity, was always judged to be less than the FAO/WHO recommendations. The BCAA intake of the MMA patient was remarkably stable from 0.5 to 2.75 years and at 2 years of age isoleucine and valine intake approximated the FAO/WHO recommendations. From 2 to 2.75 years BCAA intake (mg/kg/day) of the MSUD patient was less than that of the MMA patient and well below FAO/WHO recommendations. Neuromotor development in both patients is normal. |
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Authors:
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H G Parsons; R J Carter; M Unrath; F F Snyder |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: 13 ISSN: 0141-8955 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 1990 |
Date Detail:
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Created Date: 1990-09-12 Completed Date: 1990-09-12 Revised Date: 2007-03-21 |
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: 125-36 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Faculty of Medicine, University of Calgary, Alberta, Canada. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Amino Acid Metabolism, Inborn Errors
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diet therapy*,
metabolism Amino Acids, Branched-Chain / administration & dosage*, metabolism Ammonia / blood Body Weight Child, Preschool Creatinine / urine Dietary Proteins / metabolism Female Food, Formulated Growth Humans Infant Infant, Newborn Isoleucine / blood, metabolism Leucine / blood, metabolism Male Malonates / urine* Maple Syrup Urine Disease / diet therapy*, metabolism Methylmalonic Acid / urine* Nutritional Requirements Valine / blood, metabolism |
| Chemical | |
Reg. No./Substance:
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0/Amino Acids, Branched-Chain; 0/Dietary Proteins; 0/Malonates; 516-05-2/Methylmalonic Acid; 60-27-5/Creatinine; 61-90-5/Leucine; 7004-03-7/Valine; 73-32-5/Isoleucine; 7664-41-7/Ammonia |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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