Maple syrup urine disease diet

The goal of diet therapy is to maintain branched chain amino acids (BCAA), particularly leucine, within normal limits as studies have shown a . Maple syrup urine disease;; Nutrition therapy/diet therapy;; Practice guidelines;; Inborn amino acidopathies;; Inborn errors of metabolism; . Maple syrup urine disease (MSUD), which is also known as branched-chain ketoaciduria, branched-chain alpha-keto acid dehydrogenase deficiency, . Maple syrup urine disease (MSUD) is an aminoacidopathy secondary to an. The mainstay in the treatment of MSUD is dietary restriction of . People with MSUD have problems breaking down certain amino acids found in protein. Lifelong treatment with the MSUD diet is necessary. Bloxam HR, Day MG, Gibbs NK, Woolf LI. An inborn defect in the metabolism of tyrosine in infants on a normal diet.

Maple Syrup Urine Disease (MSUD). Maple syrup urine disease (MSUD) is a rare genetic disorder characterized by. The disorder can be successfully managed through a specialized diet.