| Management of maple syrup urine disease in Canada. Committee for improvement of Hereditary Disease Management. | |
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MedLine Citation:
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PMID: 1032577 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Nine patients with classic maple syrup urine disease (MSUD) and four with variant forms are under care at five treatment centres in the network affiliated with the National Food Distribution Centre for the Treatment of Hereditary Metabolic Diseases (the "Food Bank"). Diagnosis was made by clinicians and not from mass screening programs. MSUD requires complex emergency treatment to prevent severe neurologic damage, but effective management is compatible with normal growth and development. Long-term treatment requires continuous monitoring of the response to diets restricted in branched-chain amino acids; semisynthetic diet products free of branched-chain amino acids, provided by the Food Bank, are essential. Centralized treatment programs reduce the cost of treatment and maximize the potential benefits. The leucine requirement for adequate somatic growth during infancy in MSUD was found to be 200 to 600 mg/d; this range is lower than that estimated for infants with an intact leucine catabolic outflow pathway. The requirements for isoleucine and valine in infancy were also found to be lower than published values for normal infants. |
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Authors:
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Canadian Medical Association journal Volume: 115 ISSN: 0008-4409 ISO Abbreviation: Can Med Assoc J Publication Date: 1976 Nov |
Date Detail:
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Created Date: 1978-07-24 Completed Date: 1978-07-24 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 0414110 Medline TA: Can Med Assoc J Country: CANADA |
Other Details:
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Languages: eng Pagination: 1005-13 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Canada Health Facilities Humans Infant Leucine Maple Syrup Urine Disease / diagnosis, diet therapy* Nutritional Requirements |
| Chemical | |
Reg. No./Substance:
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61-90-5/Leucine |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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