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Riboflavin

Glutaric aciduria (GA1), Multiple acyl Co A dehydrogenase deficiency (MAD), Mitochondrial complex 1 deficiency.

GA1:  There is no firm evidence that riboflavin improves the neurological outcome of GA (100). However, responsiveness to 100-150 mg/day divided in 2-3 doses have been demonstrated in few patients (154, 155).

MAD: 100-400mg/day in 2-3 divided doses (156, 157).

SCAD: 10 mg/kg/ day, divided into three doses with a maximum of 150 mg/day (158).

Mitochondrial complex 1 deficiency: 3-20 mg/kg/day divided in 3 doses (9, 159).

25 mg, 50 mg, 100 mg tablet

400 mg capsule

PO

Urine discoloration

4

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

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]|.

154. Picker JD, Levy HL. Homocystinuria Caused by Cystathionine Beta-Synthase Deficiency. 1993 doi: NBK1524 [bookaccession][published Online First: Epub Date]|.

155. Stockler S, Plecko B, Gospe SM, Jr., et al. Pyridoxine dependent epilepsy and antiquitin deficiency: clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Mol Genet Metab 2011;104(1-2):48-60 doi: 10.1016/j.ymgme.2011.05.014[published Online First: Epub Date]|.

156. Ohkubo Y, Ueta A, Ito T, et al. Vitamin B6-responsive ornithine aminotransferase deficiency with a novel mutation G237D. The Tohoku journal of experimental medicine 2005;205(4):335-42

157. Bobrowski AE, Langman CB. The primary hyperoxalurias. Seminars in nephrology 2008;28(2):152-62 doi: 10.1016/j.semnephrol.2008.01.008[published Online First: Epub Date]|.

158. Monico CG, Rossetti S, Olson JB, Milliner DS. Pyridoxine effect in type I primary hyperoxaluria is associated with the most common mutant allele. Kidney international 2005;67(5):1704-9 doi: 10.1111/j.1523-1755.2005.00267.x[published Online First: Epub Date]|.

159. Clayton PT. B6-responsive disorders: a model of vitamin dependency. J Inherit Metab Dis 2006;29(2-3):317-26 doi: 10.1007/s10545-005-

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