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Ubiquinone (Coenzyme Q10)

Primary CoQ10 deficiency.

The dosage employed is highly variable, Adult: 200-600 mg QID, Pediatrics: 2-15 mg/kg/day BID (176).

Other used: 30 mg/kg/day (177). As high as 2000 mg/day have been used (178).

50 mg, 100 mg, 200 mg soft gels capsule

PO

Diarrhea, nausea, heartburn, appetite suppression, headache, dizziness, irritability, agitation, mild increase in liver enzymes, skin rash, pruritus, exanthema.

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176. Morton DH, Strauss KA, Robinson DL, Puffenberger EG, Kelley RI. Diagnosis and treatment of maple syrup disease: a study of 36 patients. Pediatrics 2002;109(6):999-1008

177. Pastoris O, Savasta S, Foppa P, Catapano M, Dossena M. Pyruvate dehydrogenase deficiency in a child responsive to thiamine treatment. Acta Paediatr 1996;85(5):625-8

178. Di Rocco M, Lamba LD, Minniti G, Caruso U, Naito E. Outcome of thiamine treatment in a child with Leigh disease due to thiamine-responsive pyruvate dehydrogenase deficiency. European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 2000;4(3):115-7 doi: 10.1053/ejpn.2000.0278[published Online First: Epub Date]|.

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