{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T22:12:08Z","timestamp":1792015928746,"version":"4.4.0"},"reference-count":37,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T00:00:00Z","timestamp":1723593600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Spanish Society for the Study of Inborn Errors of Metabolism (AECOM)"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Treatment of fatty acid oxidation disorders is based on dietary, pharmacological and metabolic decompensation measures. It is essential to provide the patient with sufficient glucose to prevent lipolysis and to avoid the use of fatty acids as fuel as far as possible. Dietary management consists of preventing periods of fasting and restricting fat intake by increasing carbohydrate intake, while maintaining an adequate and uninterrupted caloric intake. In long-chain deficits, long-chain triglyceride restriction should be 10% of total energy, with linoleic acid and linolenic acid intake of 3\u20134% and 0.5\u20131% (5\/1\u201310\/1 ratio), with medium-chain triglyceride supplementation at 10\u201325% of total energy (total MCT+LCT ratio = 20\u201335%). Trihepatnoin is a new therapeutic option with a good safety and efficacy profile. Patients at risk of rhabdomyolysis should ingest MCT or carbohydrates or a combination of both 20 min before exercise. In medium- and short-chain deficits, dietary modifications are not advised (except during exacerbations), with MCT contraindicated and slow sugars recommended 20 min before any significant physical exertion. Parents should be alerted to the need to increase the amount and frequency of carbohydrate intake in stressful situations. The main measure in emergency hospital treatment is the administration of IV glucose. The use of carnitine remains controversial and new therapeutic options are under investigation.<\/jats:p>","DOI":"10.3390\/nu16162707","type":"journal-article","created":{"date-parts":[[2024,8,15]],"date-time":"2024-08-15T03:49:36Z","timestamp":1723693776000},"page":"2707","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Nutritional Management of Patients with Fatty Acid Oxidation Disorders"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6052-5894","authenticated-orcid":false,"given":"Luis","family":"Pe\u00f1a-Quintana","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Unit, Insular Materno-Infantil University Hospital Complex, Asociaci\u00f3n Canaria de Investigaci\u00f3n Pedi\u00e1trica, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de la Fisiopatolog\u00eda de la Obesidad y Nutrici\u00f3n ISCIII, University of Las Palmas de Gran Canaria, 35016 Las Palmas de Gran Canaria, Spain"},{"name":"Asociaci\u00f3n Espa\u00f1ola para el Estudio de los Errores Cong\u00e9nitos del Metabolismo (AECOM), 28221 Majadahonda, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Correcher-Medina","sequence":"additional","affiliation":[{"name":"Asociaci\u00f3n Espa\u00f1ola para el Estudio de los Errores Cong\u00e9nitos del Metabolismo (AECOM), 28221 Majadahonda, Spain"},{"name":"Metabolic and Nutrition Unit, Hospital Universitari i Polit\u00e8cnic la Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,8,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/s11154-018-9448-1","article-title":"Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle","volume":"19","author":"Knottnerus","year":"2018","journal-title":"Rev. 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