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Methionine

Several remethylation defects

40–50 mg/kg per day adjust the dose to maintain upper normal ranges of plasma and CSF methionine (67). However, some investigators argue against its usage in such disorders because it may result in sustained hyperhomocystinaemia (66).

Available in different dosage forms, Capsules, powder and tablets.

PO

Headache, nausea, diarrhea, increased salivation, urinary frequency.

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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