{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T00:56:05Z","timestamp":1790556965517,"version":"4.1.0"},"reference-count":38,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T00:00:00Z","timestamp":1789948800000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Neuroendocrinology"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Female carriers of X\u2010linked adrenoleukodystrophy (X\u2010ALD) frequently develop neurodegenerative phenotypes resembling adrenomyeloneuropathy. However, evidence supporting disease\u2010modifying nutritional strategies remains limited. We evaluated the effects of Aldixyl\u00ae, a derivative of Lorenzo's Oil, and dietary interventions on neurological progression, very\u2010long\u2010chain fatty acids (VLCFAs) levels, adrenal function, and systemic safety in X\u2010ALD women. We conducted a single\u2010center retrospective longitudinal study including ABCD1 heterozygous women followed at Bambino Ges\u00f9 Children's Hospital between 2001 and 2025. Neurological disability was measured by Adult ALD Clinical Score (AACS), Expanded Disability Status Scale (EDSS), and Timed 25\u2010Foot Walk (T25\u2010FW). Outcomes were compared between patients treated with Aldixyl\u00ae plus dietary prescriptions (group A) and those receiving diet\u2010only (group B). Thirty\u2010six patients were included (mean age 41.6\u2009years), 36.1% were neurologically symptomatic at baseline (mean AACS 2.7\u2009\u00b1\u20092.6). Median C26:0 levels were mildly elevated (1.59\u2009\u03bcmol\/L). Group A (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200920) was older and more neurologically impaired at baseline than group B (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200916) (AACS 3.45 vs. 0.38). Over a median follow\u2010up of 69\u2009months, neurological progression was significantly slower in group A according to AACS (LS mean difference \u22122.26) and EDSS (\u22121.03), while T25\u2010FW did not change. Aldixyl\u00ae exposure was associated with sustained VLCFA reduction (\n                    <jats:italic>\u03b2<\/jats:italic>\n                    \u2009=\u2009\u22120.40\u2009\u03bcmol\/L). Adrenal function and metabolic parameters remained stable, although a mild, uncomplicated decrease in platelet count was observed. In female X\u2010ALD carriers, Aldixyl\u00ae is associated with sustained VLCFA reduction and signals suggestive of a more favorable neurological trajectory, while preserving adrenal function and demonstrating an acceptable safety profile. These findings warrant confirmation in prospective, controlled studies.\n                  <\/jats:p>","DOI":"10.1111\/jne.70270","type":"journal-article","created":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T02:06:02Z","timestamp":1790042762000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Effects of combined nutritional interventions on the natural history of X\u2010linked adrenoleukodystrophy in female carriers"],"prefix":"10.1111","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-3803-4350","authenticated-orcid":false,"given":"Davide","family":"Ferrari","sequence":"first","affiliation":[{"name":"Department of Experimental Medicine Sapienza University of Rome  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rita","family":"Spreghini","sequence":"additional","affiliation":[{"name":"Endocrinology and Diabetology Unit' Bambino Ges\u00f9' Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graziamaria","family":"Ubertini","sequence":"additional","affiliation":[{"name":"Endocrinology and Diabetology Unit' Bambino Ges\u00f9' Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9276-4590","authenticated-orcid":false,"given":"Enrico Silvio","family":"Bertini","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Disorders, Department of Neurosciences Bambino Ges\u00f9 Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Aiello","sequence":"additional","affiliation":[{"name":"Laboratory of Medical Genetics, Translational Cytogenomics Research Unit IRCCS Bambino Ges\u00f9 Children Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Petrarca","sequence":"additional","affiliation":[{"name":"Movement Analysis and Robotics Laboratory \u201cBambino Ges\u00f9\u201d Children's Hospital\u2014IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7950-4970","authenticated-orcid":false,"given":"Antonio","family":"Petrucci","sequence":"additional","affiliation":[{"name":"Center for Neuromuscular and Neurological Rare Diseases S. Camillo Forlanini Hospital  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5954-9749","authenticated-orcid":false,"given":"Cristiano","family":"Rizzo","sequence":"additional","affiliation":[{"name":"UOC of Metabolic Diseases Bambino Ges\u00f9 Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9037-5417","authenticated-orcid":false,"given":"Andrea M.","family":"Isidori","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine Sapienza University of Rome  Rome Italy"},{"name":"Centre for Rare Diseases (Endo\u2010ERN Accredited) Policlinico Umberto I  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5647-7519","authenticated-orcid":false,"given":"Marco","family":"Cappa","sequence":"additional","affiliation":[{"name":"Research Unit for Innovative Therapies in 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