{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T20:26:15Z","timestamp":1792009575946,"version":"4.4.0"},"reference-count":23,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,5,7]],"date-time":"2025-05-07T00:00:00Z","timestamp":1746576000000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JIMD Reports"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>The current standard diet for long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency (LCHADD) in the first months of life includes a special formula low in long\u2010chain triglycerides (LCT) and enriched in medium\u2010chain triglycerides (MCT). It involves the interruption of breastfeeding, withholding its nutritional and nonnutritional benefits. We describe the clinical case of a late preterm with 36\u2009weeks gestational age diagnosed with LCHADD through newborn screening (NBS) who developed necrotizing enterocolitis (NEC) and sepsis due to <jats:styled-content style=\"fixed-case\"><jats:italic>Escherichia coli<\/jats:italic><\/jats:styled-content> (<jats:styled-content style=\"fixed-case\"><jats:italic>E. coli<\/jats:italic><\/jats:styled-content>) at 7\u2009days of life. During hospital admission, the patient was fed skimmed breast milk supplemented with MCT oil and a low\u2010fat MCT\u2010enriched formula. Because the family wished to continue pumping milk after discharge, they were trained to defat milk using a non\u2010refrigerated benchtop centrifuge. At home, a similar feeding regime was followed for 4\u2009months. Hospital and home\u2010produced skimmed breast milk met the dietary treatment requirement of &lt;\u20091.0\u2009g\/dL of fat content. Growth and development during the first 5\u2009months of life were normal, with an improved serum acylcarnitine profile and no decompensation. In this report, we demonstrated that breast milk defatting is a safe and feasible option for patients with LCHADD during hospital admission and at home, providing the benefits of human milk in these patients. This approach could influence dietary management guidelines for metabolic disorders or expand breast milk feeding options for medically complex infants.<\/jats:p>","DOI":"10.1002\/jmd2.70018","type":"journal-article","created":{"date-parts":[[2025,5,7]],"date-time":"2025-05-07T11:09:45Z","timestamp":1746616185000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Nutrition With Skimmed Breast Milk in an Infant With Long Chain 3\u2010Hydroxyacyl\u2010<scp>coA<\/scp> Dehydrogenase Deficiency"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4045-4846","authenticated-orcid":false,"given":"Clara","family":"Alonso\u2010Diaz","sequence":"first","affiliation":[{"name":"Neonatology Department Research Institut i+12. 12 de Octubre Hospital. Universidad Complutense  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Escuder\u2010Vieco","sequence":"additional","affiliation":[{"name":"Aladina\u2010MGU Regional Mother's Milk Bank Research Institut i+12. 12 de Octubre Hospital  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Quijada\u2010Fraile","sequence":"additional","affiliation":[{"name":"Enfermedades Mitocondriales\u2010Metab\u00f3licas Hereditarias Research Institut i+12. 12 de Octubre Hospital  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delia","family":"Barrio\u2010Carreras","sequence":"additional","affiliation":[{"name":"Enfermedades Mitocondriales\u2010Metab\u00f3licas Hereditarias Research Institut i+12. 12 de Octubre Hospital  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"P\u00e9rez\u2010Mohand","sequence":"additional","affiliation":[{"name":"Enfermedades Mitocondriales\u2010Metab\u00f3licas Hereditarias Research Institut i+12. 12 de Octubre Hospital  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Mart\u00edn\u2010Hern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Enfermedades Mitocondriales\u2010Metab\u00f3licas Hereditarias Research Institut i+12. 12 de Octubre Hospital  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen Rosa","family":"Pallas\u2010Alonso","sequence":"additional","affiliation":[{"name":"Neonatology Department Research Institut i+12. 12 de Octubre Hospital. 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