{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T10:59:58Z","timestamp":1790593198460,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T00:00:00Z","timestamp":1787443200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T00:00:00Z","timestamp":1787443200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100007373","name":"Stiftelsen Sven Jerrings Fond","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007373","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006741","name":"Stiftelsen Frimurare Barnhuset i Stockholm","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006741","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009810","name":"Wera Ekstr\u00f6ms Stiftelse f\u00f6r Pediatrisk Forskning","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009810","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003514","name":"Kommunfullm\u00e4ktige, Stockholms Stad","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003514","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JIMD Reports"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Fasting\u2010induced cellular energy deficiency and hypoglycemia are well\u2010known complications of beta\u2010oxidation disorders. The changes in energy homeostasis during fasting are under hormonal regulation. This aspect of fasting metabolism in beta\u2010oxidation disorders is not fully characterized. This clinical study investigates hormonal and substrate profiles during fasting and after a standardized meal in patients with beta\u2010oxidation disorders compared with age\u2010matched healthy controls. Children with beta\u2010oxidation disorders (4 LCHADD, 5 VLCADD, 6 MCADD, median age 8.5, 3\u201313\u2009years) and healthy age\u2010matched controls (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200912, median age 9.5, 5\u201313\u2009years) were subjected to a 4\u2010h daytime fast. Blood samples for glucose, ketones, amino acids, nonesterified fatty acids (NEFA), acylcarnitines, and a panel of metabolically active hormones were analyzed after a 4\u2010h fast and 60\u2009min after intake of the standardized meal. Patients with a beta\u2010oxidation disorder displayed a normal glycemic response to fasting. Most hormonal changes were similar to the changes observed in controls. However, children with LCHADD had higher insulin levels and BMI but, interestingly, relatively low HbA1c compared to children with VLCADD and MCADD. Our findings regarding a possible increased risk of insulin resistance in patients with LCHADD need to be verified in larger cohorts.\n                  <\/jats:p>","DOI":"10.1002\/jmd2.70120","type":"journal-article","created":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T01:50:26Z","timestamp":1787536226000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Hormonal Responses to a Short Daytime Fast in Children With Beta\u2010Oxidation Disorders"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7334-3053","authenticated-orcid":false,"given":"David","family":"Olsson","sequence":"first","affiliation":[{"name":"Department of Women's and Children's Health, Unit for Pediatric Endocrinology Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Pediatric Endocrinology and Inborn Errors of Metabolism, Astrid Lindgren Children's Hospital Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Halldin","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health, Unit for Pediatric Endocrinology Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Pediatric Endocrinology and Inborn Errors of Metabolism, Astrid Lindgren Children's Hospital Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte Bieneck","family":"Haglind","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health, Unit for Pediatric Endocrinology Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Pediatric Endocrinology and Inborn Errors of Metabolism, Astrid Lindgren Children's Hospital Karolinska University Hospital  Stockholm 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