Table 4. [Pyruvate Dehydrogenase Complex Deficiency: Management by Genetic Cause]. - GeneReviews®

Gene	Treatment	References / GeneReview
DLAT	Ketogenic diet Thiamine (300-1000 mg/day) has been used w/limited success. ¹	Friedman et al [2017]
PDHA1	Fouque et al [2003], DeBrosse et al [2012], Patel et al [2012]
PDHB	Okajima et al [2008]
PDHX	Tajir et al [2012]
PDP1	Bedoyan et al [2019]
DLD	Branched-chain amino acid restriction Dextrose-containing IV fluids Riboflavin (220-400 mg/day) Acetaminophen & ethanol are contraindicated.	See also Dihydrolipoamide Dehydrogenase Deficiency.

From: Primary Pyruvate Dehydrogenase Complex Deficiency Overview

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