Maple Syrup Urine Disease falls under the Other category.

Maple Syrup Urine Disease

•Last Updated: September 29, 2022

MSUD (maple syrup urine disease) is a genetic disorder. The body cannot metabolize branched-chain amino acids, resulting in sweet-smelling urine. Neurological symptoms emerge in the first 48 hours of life; if MSUD is left untreated (through diet), death occurs in weeks to months.

Maple Syrup Urine Disease falls under the Other category.

Last Updated:September 29, 2022

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A ketogenic diet for dihydrolipoamide dehydrogenase deficiency

In this case series on children with the rare genetic disease dihydrolipoamide dehydrogenase deficiency, a ketogenic diet did not seem to mitigate brain abnormalities or benefit quality of life.