Classical maple syrup urine disease and brain development: Principles of management and formula design
Abstract
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 on a 20-year experience managing 79 patients, we designed a study formula to (1) optimize transport of seven amino acids (Tyr, Trp, His, Met, Thr,...
Paper Details
Title
Classical maple syrup urine disease and brain development: Principles of management and formula design
Published Date
Apr 1, 2010
Volume
99
Issue
4
Pages
333 - 345
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