{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T14:29:31Z","timestamp":1787581771856,"version":"build-2736575974"},"reference-count":36,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,1,21]],"date-time":"2025-01-21T00:00:00Z","timestamp":1737417600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,1,21]],"date-time":"2025-01-21T00:00:00Z","timestamp":1737417600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100003514","name":"Kommunfullm\u00e4ktige, Stockholms Stad","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003514","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\/501100007373","name":"Stiftelsen Sven Jerrings Fond","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007373","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To investigate fasting metabolism in children with very long\u2010chain acyl\u2010CoA dehydrogenase deficiency (VLCADD) and medium\u2010chain acyl\u2010CoA dehydrogenase deficiency (MCADD) using microdialysis technique.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Twelve patients (7 with VLCADD, 5 with MCADD, mean age 4.9\u2009years, 10\/12 diagnosed via newborn screening) were recruited for investigation in connection to clinical fasting examinations at the Karolinska University Hospital (between 2015 and 2024). Patients were subjected to a 9\u2010h night fast after a standard late evening meal. Analysis of glucose, glycerol, lactate, and pyruvate was conducted by continuous microdialysis. Fasting hormones and acylcarnitines were analysed in blood samples at 1\u2010h intervals in patients with VLCADD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Children with VLCADD showed signs of lipolysis after a median fasting time of 4.5\u2009h, whereas patients with MCADD showed no significant increase in lipolysis during the fast. A shorter time to initiation of lipolysis tended to correlate with a lower residual enzyme activity in patients with VLCADD. All patients maintained euglycemia during fasting.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Children with VLCADD had a shorter time to initiation of lipolysis during fasting than children with MCADD. Clinical evaluation of fasting metabolism in beta\u2010oxidation disorders should include assessment of lipolysis as an early and important determinant.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/apa.17591","type":"journal-article","created":{"date-parts":[[2025,1,21]],"date-time":"2025-01-21T22:53:37Z","timestamp":1737500017000},"page":"1445-1455","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Assessment of Fasting Metabolism With Microdialysis Indicates Earlier Lipolysis in Children With\n                    <scp>VLCADD<\/scp>\n                    Than\n                    <scp>MCADD<\/scp>"],"prefix":"10.1111","volume":"114","author":[{"given":"David","family":"Olsson","sequence":"first","affiliation":[{"name":"Department of Women's and Children's Health, Unit for Pediatric Endocrinology and Metabolic Disorders Karolinska Institutet\/Karolinska University Hospital  Stockholm 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Sciences, Sahlgrenska Academy, University of Gothenburg, Pediatric Endocrinology Unit Sahlgrenska University Hospital  Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Nordenstr\u00f6m","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health, Unit for Pediatric Endocrinology and Metabolic Disorders Karolinska Institutet\/Karolinska University Hospital  Stockholm Sweden"},{"name":"Center for Inherited Metabolic Diseases CMMS, Karolinska University Hospital  Stockholm 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