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Folic acid

Long term supplementation to compensate for the so-called methylfolate trap in remethylation defect.

Variable, 5-30mg/day (66, 67).

1 mg and 5 mg tablets

PO

Bad taste in mouth, large doses: loss of appetite, nausea, irritability, sleep pattern disturbance and confusion.

4

66. Berendzen K, Theriaque DW, Shuster J, Stacpoole PW. Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency. Mitochondrion 2006;6(3):126-35 doi: 10.1016/j.mito.2006.04.001[published Online First: Epub Date]|.

67. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA. Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr 2001;138(3):383-9 doi: 10.1067/mpd.2001.111818[published Online First: Epub Date]|.

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