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L-serine
- 3-phosphoglycerate dehydrogenase deficiency (3-PGDH) deficiency
-Phosphoserine aminotransferase (PSAT) deficiency
- Phosphoserine phosphatase (PSPH) deficiency
• 3-PGDH:
Infantile form: 500 - 600 mg/kg/day in 3 divided doses
Juvenile form: 100-150 mg /kg/day in 3 divided doses.
• PSAT: 500mg/kg/day
• PSPH: 200 - 300mg/kg/day
However, the doses are varied aiming to normalize CSF serine (114).
Powder
PO
Unknown
4
114. Tanner LM, Nanto-Salonen K, Venetoklis J, et al. Nutrient intake in lysinuric protein intolerance. J Inherit Metab Dis 2007;30(5):716-21 doi: 10.1007/s10545-007-0558-2[published Online First: Epub Date]|.
