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Mannose

Congenital disorder of glycosylation type 1b

0.15mg/kg/dose or 1 g/kg/day divided in 5 doses  However, the mannose doses depend on plasma mannose measurement, and the frequency of mannose uptakes are crucial because of the short clearance half-time of mannose (8, 117-119). 

Liquid

PO

Unknown

4

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

117. Rajantie J, Simell O, Rapola J, Perheentupa J. Lysinuric protein intolerance: a two-year trial of dietary supplementation therapy with citrulline and lysine. J Pediatr 1980;97(6):927-32

118. Tanner LM, Nanto-Salonen K, Niinikoski H, Huoponen K, Simell O. Long-term oral lysine supplementation in lysinuric protein intolerance. Metabolism 2007;56(2):185-9 doi: 10.1016/j.metabol.2006.09.011[published Online First: Epub Date]|.

119. Lukkarinen M, Nanto-Salonen K, Pulkki K, Aalto M, Simell O. Oral supplementation corrects plasma lysine concentrations in lysinuric protein intolerance. Metabolism 2003;52(7):935-8

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