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L-citrulline

-Carbamoyl phosphate synthetase (CPS) deficiency

-Ornithine transcarbamylase (OTC) deficiency

Lysinuric protein intolerance (LPI)

CPS and OTC deficiency:

170 mg/kg/day or 3.8 gram/m2/day.(102-105)

LPI: 100 mg/kg/day, however, the aim is to keep the citrulline at normal ranges.

In some patients 400 mg/kg/day were used (106-108).

500 mg capsules
Powder

PO

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102. Ohya Y, Ochi N, Mizutani N, Hayakawa C, Watanabe K. Nonketotic hyperglycinemia: treatment with NMDA antagonist and consideration of neuropathogenesis. Pediatric neurology 1991;7(1):65-8

103. Tegtmeyer-Metzdorf H, Roth B, Gunther M, et al. Ketamine and strychnine treatment of an infant with nonketotic hyperglycinaemia. Eur J Pediatr 1995;154(8):649-53

104. Wraith JE, Clarke LA, Beck M, et al. Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human alpha-L-iduronidase (laronidase). J Pediatr 2004;144(5):581-8 doi: 10.1016/j.jpeds.2004.01.046[published Online First: Epub Date]|.

105. Chapman KA, Gropman A, Macleod E, et al. Acute management of propionic acidemia. Mol Genet Metab 2012;105(1):16-25 doi: 10.1016/j.ymgme.2011.09.026[published Online First: Epub Date]|.

106. Kolker S, Christensen E, Leonard JV, et al. Diagnosis and management of glutaric aciduria type I--revised recommendations. J Inherit Metab Dis 2011;34(3):677-94 doi: 10.1007/s10545-011-9289-5[published Online First: Epub Date]|.

107. Sutton VR, Chapman KA, Gropman AL, et al. Chronic management and health supervision of individuals with propionic acidemia. Mol Genet Metab 2012;105(1):26-33 doi: 10.1016/j.ymgme.2011.08.034[published Online First: Epub Date]|.

108. Brusilow SW. Arginine, an indispensable amino acid for patients with inborn errors of urea synthesis. J Clin Invest 1984;74(6):2144-8 doi: 10.1172/JCI111640[published Online First: Epub Date]|.

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