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Pyridoxine

Pyridoxine responsive pyridoxine responsive cystathionine β synthase deficiency (CBS), pyridoxine dependent epilepsy (PDE), pyridoxine responsive Ornithine Aminotransferase Deficiency ( OAT), primary hyperoxaluria type 1(PH1).

CBS:  200 mg/day or the lowest dose that produces the maximum biochemical benefit (i.e., lowest plasma homocysteine and methionine concentrations), as determined by measurement of total homocysteine and amino acid levels, should be given (17, 148).

PDE: 100 mg IV, additional doses may be administered over the course of 30 min while observing for both a clinical and possible electrographic response. If IV administration of pyridoxine is not possible for a first trial, pyridoxine is given orally/enterally with 30 mg/kg/day. Long term treatment; there are no clear-cut dosing recommendations, generally 15-30 mg/kg/day have been used in infants or up to 200 mg/day in neonates and 500 mg/day in adults (149).

OAT deficiency: 300-600mg/day (8, 17, 150).

PH1: 5-10 mg/kg/day. Monitor oxalate and glycolate excretion and titrate the dose accordingly (151, 152).

Tablet 25 mg, 40 mg, 50 mg, 100 mg, 250 mg, and 500 mg

Liquid oral: 200 mg/5 mL

PO or IV (PDE)

PO in others

Decreased folic acid,  paresthesia, somnolence.

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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.

17. Chien YH, Hwu WL. A review of treatment of Pompe disease in infants. Biologics : targets & therapy 2007;1(3):195-201

148. Pronicka E, Rowinska E, Bentkowski Z, Zawadzki J, Holme E, Lindstedt S. Treatment of two children with hereditary tyrosinaemia type I and long-standing renal disease with a 4-hydroxyphenylpyruvate dioxygenase inhibitor (NTBC). J Inherit Metab Dis 1996;19(2):234-8

149. Ostman-Smith I, Brown G, Johnson A, Land JM. Dilated cardiomyopathy due to type II X-linked 3-methylglutaconic aciduria: successful treatment with pantothenic acid. British heart journal 1994;72(4):349-53

150. Cox DW, Roberts E. Wilson Disease. 1993 doi: NBK1512 [bookaccession][published Online First: Epub Date]|.

151. Brewer GJ. Novel therapeutic approaches to the treatment of Wilson's disease. Expert opinion on pharmacotherapy 2006;7(3):317-24 doi: 10.1517/14656566.7.3.317[published Online First: Epub Date]|.

152. Product information for Cuprimine®. Secondary Product information for Cuprimine®. http://www.cuprimine.com/pdfs/Cuprimine_PI.pdf.

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