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100 mg/mL Usual Adult Dose for: Usual Pediatric Dose for: Additional dosage information: Usual Adult Dose for Carnitine Deficiency METABOLIC DISORDERS: Primary or secondary carnitine deficiency: Oral solution: Initial dose: 1 gram orally daily in evenly spaced divided doses (every 3 to 4 hours) Maintenance dose: 1 to 3 grams orally daily in evenly spaced divided doses (every 3 to 4 hours) Oral tablets: 990 mg orally 1 to 3 times a day, depending on clinical response Intravenous solution: 50 mg/kg as a slow 2 to 3 minute bolus or by infusion, once a day A loading dose may be given in severe metabolic crisis, followed by an equivalent dose every 3 to 4 hours (but not less often than every 6 hours) over the following 24 hours Subsequent daily doses should be in the range of 50 mg/kg or as therapy may require The highest dose administered has been 300 mg/kg

Inject water slowly down the vial wall, swirl gently (do not shake), and refrigerate at 2-8C

Study Design 13

Ethnicity & Disease

Supplementation of butyrate reverses these phenotypes (i.e

10.1016/s1089-8603(02)00124-6 105 WeidnerW.HauckE