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GS deficiency: 10mg/kg/day (182-184).

50 units/ mL drops

PO

Bleeding, hepatotoxicity, retinal hemorrhage, pulmonary embolism, sepsis.

4

182.        Njalsson R. Glutathione synthetase deficiency. Cellular and molecular life sciences : CMLS. 2005;62:1938-45 doi: 10.1007/s00018-005-5163-7 [published Online First: 2005/07/02].

183.        Njalsson R, Ristoff E, Carlsson K, Winkler A, Larsson A, Norgren S. Genotype, enzyme activity, glutathione level, and clinical phenotype in patients with glutathione synthetase deficiency. Human genetics. 2005;116:384-9 doi: 10.1007/s00439-005-1255-6 [published Online First: 2005/02/18].

184.        Ristoff E, Mayatepek E, Larsson A. Long-term clinical outcome in patients with glutathione synthetase deficiency. J Pediatr. 2001;139:79-