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Those affected by FAOD are unable to convert fatty acids into tricarboxylic acid cycle intermediates such as acetyl-coenzyme A, resulting in decreased adenosine triphosphate and glucose for use as energy in a variety of high-energy\u2013requiring organ systems. Signs and symptoms may manifest in infants but often also appear in adolescents or adults during times of increased metabolic demand, such as fasting, physiologic stress, and prolonged exercise. Patients with FAOD present with a highly heterogeneous clinical spectrum. The most common clinical presentations include hypoketotic hypoglycemia, liver dysfunction, cardiomyopathy, rhabdomyolysis, and skeletal myopathy, as well as peripheral neuropathy and retinopathy in some subtypes. Despite efforts to detect FAOD through newborn screening and manage patients early, symptom onset can be sudden and serious, even resulting in death. Therefore, it is critical to identify quickly and accurately the key signs and symptoms of patients with FAOD to manage metabolic decompensations and prevent serious comorbidities.<\/jats:p>","DOI":"10.1007\/s11154-020-09568-3","type":"journal-article","created":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T00:52:55Z","timestamp":1594428775000},"page":"479-493","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":106,"title":["Clinical manifestations and management of fatty acid oxidation disorders"],"prefix":"10.1007","volume":"21","author":[{"suffix":"2nd","given":"J. 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Dr. Merritt reports consulting fees and honoraria from Sanofi Genzyme, Horizon Pharma, Asklepios Biopharmaceutical, Inc.EM has participated in advisory boards for Vitaflo, Nutricia, and Ajinomoto Cambrooke; and has received speaking honoraria from Vitaflo and MedEd.AJ is an employee and shareholder of Ultragenyx Pharmaceutical Inc.BH does not have any conflicts of interests to report.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}