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Uses: Primary systemic carnitine deficiency Acute and chronic treatment of inborn errors of metabolism resulting in secondary carnitine deficiency Prevention and treatment of carnitine deficiency in end stage renal disease patients undergoing dialysis (IV form only) Usual Pediatric Dose for Carnitine Deficiency METABOLIC DISORDERS: Oral solution or tablets: Initial dose: 50 mg/kg/day orally in evenly divided doses Maintenance dose: 50 to 100 mg/kg/day orally in evenly divided doses Maximum dose: 3 grams per day 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

Role of genetics and inherited genetic susceptibility loci in the pathogenesis has been proven

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For instance, unimolecular doses of the dual-receptor agonist tirzepatide has effects that closely resemble dulaglutide, a pure GLP-1RA

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