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The aim is to normalize homocysteine level.

- Classical homocystinuria and MTHFR deficiency: 

Children: 100-150 mg/kg/day ÷ BID increase weekly by 50 mg/kg increments, as needed. Maximum: 9 grams/day.

Adults: 3 grams twice daily, up to 20 grams/day have been used to control high homocysteine levels. (26-29)

- Cbl C, D, E, F, G defects: < 6 months: 250-500mg twice daily. > 6 months: 75mg/kg/day ÷ BID.(9)

Powder

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Diarrhea, Drug-induced gastrointestinal disturbance, Nausea.

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9. Loche S, Carta D, Muntoni AC, Corda R, Pintor C. Oral administration of arginine enhances the growth hormone response to growth hormone releasing hormone in short children. Acta Paediatr 1993;82(10):883-4

26. Matalon R, Stumpf DA, Michals K, Hart RD, Parks JK, Goodman SI. Lipoamide dehydrogenase deficiency with primary lactic acidosis: favorable response to treatment with oral lipoic acid. J Pediatr 1984;104(1):65-9

27. Craigen WJ. Leigh disease with deficiency of lipoamide dehydrogenase: treatment failure with dichloroacetate. Pediatric neurology 1996;14(1):69-71

28. Brewer GJ, Askari F, Dick RB, et al. Treatment of Wilson's disease with tetrathiomolybdate: V. Control of free copper by tetrathiomolybdate and a comparison with trientine. Translational research : the journal of laboratory and clinical medicine 2009;154(2):70-7 doi: 10.1016/j.trsl.2009.05.002[published Online First: Epub Date]|.

29. Brewer GJ, Askari F, Lorincz MT, et al. Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease. Archives of neurology 2006;63(4):521-7 doi: 10.1001/archneur.63.4.521[published Online First: Epub Date]|.