Maple Syrup Urine Disease.
Strauss KA, Puffenberger EG, Carson VJ.
Strauss KA, et al.
2006 Jan 30 [updated 2020 Apr 23]. In: Adam MP, Bick S, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2026.
2006 Jan 30 [updated 2020 Apr 23]. In: Adam MP, Bick S, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2026.
PMID: 20301495
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MANAGEMENT: Treatment of manifestations: Treatment consists of dietary leucine restriction, BCAA-free medical foods, judicious supplementation with isoleucine and valine, and frequent clinical and biochemical monitoring. ...Acute metabolic decompensation is corrected by tr …
MANAGEMENT: Treatment of manifestations: Treatment consists of dietary leucine restriction, BCAA-free medical foods, judicious supplement …