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  1. Maple Syrup Urine Disease - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK1319
    ... maintains plasma leucine concentration within the normal range (reference mean ±2 SDs). In persons with classic MSUD, in vivo oxidation and urinary losses of branched-chain amino acids (BCAAs) are negligible [ Schadewaldt et al 1999b , Levy ...
  2. Amino Acid Metabolism Disorders 
    https://medlineplus.gov/aminoacidmetabolismdisorders.html
    ... deficiency: MedlinePlus Genetics (National Library of Medicine) ... (National Institutes of Health) Journal Articles References and abstracts from MEDLINE/PubMed (National Library of ...
  3. Primary Pyruvate Dehydrogenase Complex Deficiency Overview - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK571223
    ... Genetic Cause View in own window Gene Treatment References / GeneReview DLAT ... Branched-chain amino acid restriction Dextrose-containing IV fluids Riboflavin (220-400 ...
  4. Dihydrolipoamide Dehydrogenase Deficiency - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK220444
    ... of significant hyperleucinosis, protein intake should consist of branched-chain amino acid (BCAA)-free powder formula with 2-3 g/ ... presentation only occasionally have biochemical evidence of dysfunctional branched chain amino acid (BCAA) metabolism (i.e., elevations of allo-isoleucine ...
  5. References - Cirrhosis in Over 16s - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK385212
    ... Pedretti G, Pelosi G, Sacchini D, Zeneroli ML. Branched chain amino acid enriched solutions in the treatment of hepatic encephalopathy: ... Ceci F, Muscaritoli M, et al. Use of branched chain amino acids for treating hepatic encephalopathy: clinical experiences. Gut. 1986; ...
  6. References - Cirrhosis in Over 16s - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK385202
    ... Nielsen B, Koretz RL, Gluud LL, Gluud C. Branched-chain amino acids for hepatic encephalopathy. Cochrane Database of Systematic Reviews. ... Cordoba J, Marchesini G, Borre M, et al. Branched-chain amino acids for people with hepatic encephalopathy. Cochrane Database of ...
  7. References for Appendixes C–G - Nutrition as Prevention for Improved Cancer Health Outcomes - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK592520
    ... WC, Fan ST, et al. Long-term oral branched chain amino acids in patients undergoing chemoembolization for hepatocellular carcinoma: a ... Lu CY, et al. Randomized, controlled study of branched chain amino acid-enriched total parenteral nutrition in malnourished patients with ...
  8. Nonketotic Hyperglycinemia - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK1357
    ... several other amino acids in the CSF (particularly branched-chain amino acids). Note that on brain MRI the diffusion restriction ... Tada K. Effects of the metabolites of the branched-chain amino acids and cysteamine on the glycine cleavage system. Biochem ...
  9. Executive Summary - Nutrition as Prevention for Improved Cancer Health Outcomes - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK592543
    ... Lu CY, et al. Randomized, controlled study of branched chain amino acid-enriched total parenteral nutrition in malnourished patients with ... K, et al. A snack enriched with oral branched-chain amino acids prevents a fall in albumin in patients with ...
  10. Ornithine Transcarbamylase Deficiency - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK154378
    ... be possibly associated with reduced or borderline plasma branched-chain amino acid concentrations. Liver transplant appears to have a beneficial ... causes menstrual dysfunction & body odor, & appears to deplete branched chain amino acids; sodium benzoate causes hypokalemia due to ↑ renal losses ...
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