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Vitamin C

Glutathione Synthetase deficiency (GS).

Hawkinsinuria, Tyrosinemia type III and Transient tyrosinemia of newborn.

GS deficiency: 100mg/kg/day (182-184).

Others: 200-1000mg/day.

100 mg tablet

100 mg/mL drops

1g effervescent tablet

PO

Nephrolithiasis, diarrhea, iron overload.

4

182. Santa KM. Treatment options for mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Pharmacotherapy 2010;30(11):1179-96 doi: 10.1592/phco.30.11.1179[published Online First: Epub Date]|.

183. Trevisson E, DiMauro S, Navas P, Salviati L. Coenzyme Q deficiency in muscle. Current opinion in neurology 2011;24(5):449-56 doi: 10.1097/WCO.0b013e32834ab528[published Online First: Epub Date]|.

184. Dimauro S, Rustin P. A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases. Biochimica et biophysica acta 2009;1792(12):1159-67 doi: 10.1016/j.bbadis.2008.10.015[published Online First: Epub Date]|.

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