{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T19:06:58Z","timestamp":1790276818535,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,2,11]],"date-time":"2019-02-11T00:00:00Z","timestamp":1549843200000},"content-version":"vor","delay-in-days":41,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Patients with very long chain acyl\u2010CoA dehydrogenase deficiency (VLCADD), a long chain fatty acid oxidation disorder, are traditionally treated with a long chain triglyceride (LCT) restricted and medium chain triglyceride (MCT) supplemented diet. Introduction of VLCADD in newborn screening (NBS) programs has led to the identification of asymptomatic newborns with VLCADD, who may have a more attenuated phenotype and may not need dietary adjustments.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To define dietary strategies for individuals with VLCADD based on the predicted phenotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>We evaluated long\u2010term dietary histories of a cohort of individuals diagnosed with VLCADD identified before the introduction of VLCADD in NBS and their beta\u2010oxidation (LC\u2010FAO) flux score (rate of oleate oxidation) in cultured skin fibroblasts in relation to the clinical outcome. Based on these results a dietary strategy is proposed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixteen individuals with VLCADD were included. One had an LC\u2010FAO flux score\u2009&gt;90%, was not on a restricted diet and is asymptomatic to date. Four patients had an LC\u2010FAO flux score\u2009&lt;10%, and significant VLCADD related symptoms despite the use of strict diets including LCT restriction, MCT supplementation and nocturnal gastric drip feeding. Patients with an LC\u2010FAO flux score between 10 and 90% (<jats:italic>n<\/jats:italic> =\u200911) showed a more heterogeneous phenotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study shows that a strict diet cannot prevent poor clinical outcome in severely affected patients and that the LC\u2010FAO flux is a good predictor of clinical outcome in individuals with VLCADD identified before its introduction in NBS. Hereby, we propose an individualized dietary strategy based on the LC\u2010FAO flux score.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jimd.12037","type":"journal-article","created":{"date-parts":[[2019,2,11]],"date-time":"2019-02-11T08:08:54Z","timestamp":1549872534000},"page":"159-168","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Proposal for an individualized dietary strategy in patients with very long\u2010chain acyl\u2010CoA dehydrogenase deficiency"],"prefix":"10.1002","volume":"42","author":[{"given":"Jeannette C.","family":"Bleeker","sequence":"first","affiliation":[{"name":"Department of Metabolic Diseases, Dutch Fatty Acid Oxidation Expertise Center Wilhelmina Children\u2019s Hospital (UMCU), University Medical Center Utrecht, Internal Mail KE 04.306.0, PO Box 85090 3508 AB  Utrecht Netherlands"},{"name":"Laboratory Genetic Metabolic Diseases Academic Medical Center  Amsterdam Netherlands"},{"name":"Department of Pediatrics, Academic Medical Center University of Amsterdam  Amsterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene L.","family":"Kok","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Dutch Fatty Acid Oxidation Expertise Center Wilhelmina Children\u2019s Hospital (UMCU), University Medical Center Utrecht, Internal Mail KE 04.306.0, PO Box 85090 3508 AB  Utrecht Netherlands"},{"name":"Department of Internal Medicine and Dermatology, Dietetics University Medical Center Utrecht  Utrecht Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sacha","family":"Ferdinandusse","sequence":"additional","affiliation":[{"name":"Laboratory Genetic Metabolic Diseases Academic Medical Center  Amsterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maaike","family":"de Vries","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Radboud University Medical Center  Nijmegen Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry G.J.","family":"Derks","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases Beatrix Children\u2019s Hospital, University Medical Center Groningen  Groningen Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margot F.","family":"Mulder","sequence":"additional","affiliation":[{"name":"Department of Pediatrics VU University Medical Center Amsterdam  Amsterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monique","family":"Williams","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Erasmus MC\u2010Sophia  Rotterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estela Rubio","family":"Gozalbo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Laboratory Genetic Metabolic Diseases Maastricht 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