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However, because not all G6PD-deficient newborns have NNJ ( Table 18-6 ), it is likely that genetic or environmental factors (or both) are involved in addition to G6PD deficiency and that the same factors that are more extreme can also make the NNJ more severe

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L-carnitine is a crucial nutrient that plays an essential role in energy production by transporting fatty acids into the mitochondria to be burned for fuel

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Also, metabolites of VPA -oxidation (including propionic derivatives and 4-en-VPA) inhibit the mitochondrial CPS I, which is the first enzyme necessary for ammonia elimination via the urea cycle in the liver [19, 24]