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First , inactivating mutations in GATA1 cause X-linked recessive diseases

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Although no randomized trials exist, the uniformity of observational data has led GeneReviews and European neurometabolic taskforces to issue class I recommendations for immediate high-dose riboflavin upon molecular diagnosis (14)

This pathological state creates an imbalance where ROS and RNS production exceeds the neutralizing ability of intrinsic antioxidant systems, thereby establishing conditions of oxidative stress (Rahman and MacNee, 2000)

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