📘 Disorders of Mitochondrial Fatty Acid β-Oxidation

Nelson Textbook of Pediatrics 22nd Ed — Section 106.1. MCAD, VLCAD, LCHAD, carnitine cycle defects, electron transfer flavoprotein (ETF) deficiencies. Hypoketotic hypoglycemia, cardiomyopathy, rhabdomyolysis, newborn screening with acylcarnitines. Treatment: IV glucose, carnitine, triheptanoin, avoid fasting.