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L-Lysine-HCL

LPI

10 - 40 mg/kg/day in 3 divided doses.(8, 107, 111, 112) or 0.05-0.5 mmol/kg, three times per day. Monitor plasma lysine level (113).

Powder

PO

Fanconi's syndrome, renal failure.

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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.

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]|.

111. Berry GT, Steiner RD. Long-term management of patients with urea cycle disorders. J Pediatr 2001;138(1 Suppl):S56-60; discussion S60-1 doi: a111837 [pii][published Online First: Epub Date]|.

112. Carpenter TO, Levy HL, Holtrop ME, Shih VE, Anast CS. Lysinuric protein intolerance presenting as childhood osteoporosis. Clinical and skeletal response to citrulline therapy. N Engl J Med 1985;312(5):290-4 doi: 10.1056/NEJM198501313120506[published Online First: Epub Date]|.

113. Sebastio G, Sperandeo MP, Andria G. Lysinuric protein intolerance: reviewing concepts on a multisystem disease. American journal of medical genetics. Part C, Seminars in medical genetics 2011;157(1):54-62 doi: 10.1002/ajmg.c.30287[published Online First: Epub Date]|.

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