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Notably, as a result of (skewed) X chromosome inactivation, heterozygous females may be susceptible to the same pathophysiologic phenotype as their hemizygous male counterparts.30,93,156 G6PD-deficient neonates who have coinherited a mutation in the uridine-diphosphate-glucuronosyl-transferase-1 (UGT1A1) promoter are particularly at risk for developing neonatal jaundice and even kernicterus.94,205 A small proportion of G6PD-deficient individuals display the phenotype of chronic nonspherocytic hemolytic anemia

Buescher AV, Cidav Z, Knapp M, Mandell DS

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