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Maple Syrup Urine Disease (MSUD)

Anesthesia Implications

Clinically reviewed by anesthesia providers · Updated

Avoid AcidosisAvoid Prolonged FastingHyponatremiaImmunocompromiseIncreased ICPOptimize ElectrolytesOptimize HydrationSeizuresAvoid Hypotonic FluidsCerebral Edema

Pathophysiology

Maple syrup urine disease (MSUD) is an autosomal recessive defect of the branched-chain alpha-ketoacid dehydrogenase (BCKAD) complex, the mitochondrial enzyme that breaks down leucine, isoleucine,...

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