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An inherited metabolic disorder is an increasingly common indication for liver transplantation (LT), accounting for more than 15% of pediatric cases worldwide and up to 30% in some specialist centers. Urea cycle disorders are now the most common indication in…
Defects In Fatty Acid Oxidation Fatty acid oxidation (FAO) provides an important source of energy during fasting and physiologic stress, especially in childhood when glycogen stores are limited. Hepatic FAO produces ketone bodies, which are an important secondary energy source…