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Glycine

-Isovaleric acidemia -HMG-CoA lyase deficiency.
- May be used in 3-Methylcrotonyl glycinuria

250 mg/kg/day (150 - 300 mg/kg/day)  in 4 divided doses (8, 9, 81, 82).

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PO

Blurred vision, temporary blindness, chest pain, hypotension, bradycardia, EKG changes, coma, confusion, seizures, dyspnea, electrolyte disturbance: hyponatremia, hyperkalemia, hemolytic anemia, nausea, vomiting, thrombocytopenia.

4

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

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

81. Harmatz P, Giugliani R, Schwartz I, et al. Enzyme replacement therapy for mucopolysaccharidosis VI: a phase 3, randomized, double-blind, placebo-controlled, multinational study of recombinant human N-acetylgalactosamine 4-sulfatase (recombinant human arylsulfatase B or rhASB) and follow-on, open-label extension study. J Pediatr 2006;148(4):533-39 doi: 10.1016/j.jpeds.2005.12.014[published Online First: Epub Date]|.

82. Harmatz P, Kramer WG, Hopwood JJ, Simon J, Butensky E, Swiedler SJ. Pharmacokinetic profile of recombinant human N-acetylgalactosamine 4-sulphatase enzyme replacement therapy in patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): a phase I/II study. Acta Paediatr Suppl 2005;94(447):61-8; discussion 57

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