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Classical maple syrup urine disease and brain development: Principles of management and formula design

Branched-chain ketoacid dehydrogenase deficiency results in complex and volatile metabolic derangements that threaten brain development. Treatment for classical maple syrup urine disease (MSUD) should address this underlying physiology while also protecting children from nutrient deficiencies. Based...

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Bibliografiska uppgifter
Huvudupphovsmän: Strauss, Kevin A., Wardley, Bridget, Robinson, Donna, Hendrickson, Christine, Rider, Nicholas L., Puffenberger, Erik G., Shelmer, Diana, Moser, Ann B., Morton, D. Holmes
Materialtyp: Artigo
Språk:Inglês
Publicerad: 2010
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Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC3671925/
https://ncbi.nlm.nih.gov/pubmed/20061171
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2009.12.007
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