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L-carnitine

Primary and secondary carnitine deficiency.

Acute crises(carnitine boluses): 100 mg/kg/dose three to four  times daily i.e.(300–400 mg/kg/day) should be given. Urine output should be appropriate prior to dosing (or hemofiltration be ongoing).
Chronic: 100 - 300 mg/kg/day ÷ q8 hr.(8, 99-101)

300 mg/mL Oral liquid

300 mg capsules

500 mg Tablets

200 mg/mL Solution for injection

PO or IV

Diarrhea, nausea, stomach cramps, vomiting.

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8. Centre for Evidence-Based Medicine. Secondary Centre for Evidence-Based Medicine  2009. http://www.cebm.net/index.aspx?o=1025.

99. Charrow J, Andersson HC, Kaplan P, et al. Enzyme replacement therapy and monitoring for children with type 1 Gaucher disease: consensus recommendations. J Pediatr 2004;144(1):112-20 doi: 10.1016/j.jpeds.2003.10.067[published Online First: Epub Date]|.

100. Korman SH, Wexler ID, Gutman A, Rolland MO, Kanno J, Kure S. Treatment from birth of nonketotic hyperglycinemia due to a novel GLDC mutation. Annals of neurology 2006;59(2):411-5 doi: 10.1002/ana.20759[published Online First: Epub Date]|.

101. Lu FL, Wang PJ, Hwu WL, Tsou Yau KI, Wang TR. Neonatal type of nonketotic hyperglycinemia. Pediatric neurology 1999;20(4):295-300

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