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Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast
Women’s and Children’s Health, Karolinska Institute, Stockholm, Sweden.
Women’s and Children’s Health, Karolinska Institute, Stockholm, Sweden.
Astrid Lindgren Children’s Hospital, Karolinska University Hospital, Stockholm, Sweden.
Division of Metabolic Diseases, Department of Laboratory Medicine, Karolinska Institute, Stockholm, Sweden.
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2015 (English)In: Journal of Inherited Metabolic Disease, ISSN 0141-8955, E-ISSN 1573-2665, Vol. 38, no 2, 315-322 p.Article in journal (Refereed) Published
Abstract [en]

Children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) have a defect in the degradation of long-chain fatty acids and are at risk of hypoketotic hypoglycemia and insufficient energy production as well as accumulation of toxic fatty acid intermediates. Knowledge on substrate metabolism in children with LCHAD deficiency during fasting is limited. Treatment guidelines differ between centers, both as far as length of fasting periods and need for night feeds are concerned. To increase the understanding of fasting intolerance and improve treatment recommendations, children with LCHAD deficiency were investigated with stable isotope technique, microdialysis, and indirect calometry, in order to assess lipolysis and glucose production during 6 h of fasting. We found an early and increased lipolysis and accumulation of long chain acylcarnitines after 4 h of fasting, albeit no patients developed hypoglycemia. The rate of glycerol production, reflecting lipolysis, averaged 7.7 ± 1.6 µmol/kg/min, which is higher compared to that of peers. The rate of glucose production was normal for age; 19.6 ± 3.4 µmol/kg/min (3.5 ± 0.6 mg/kg/min). Resting energy expenditure was also normal, even though the respiratory quotient was increased indicating mainly glucose oxidation. The results show that lipolysis and accumulation of long chain acylcarnitines occurs before hypoglycemia in fasting children with LCHAD, which may indicate more limited fasting tolerance than previously suggested.

Place, publisher, year, edition, pages
2015. Vol. 38, no 2, 315-322 p.
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Endocrinology and Diabetes
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URN: urn:nbn:se:uu:diva-238291DOI: 10.1007/s10545-014-9750-3ISI: 000350360200013PubMedID: 25141826OAI: oai:DiVA.org:uu-238291DiVA: diva2:770774
Note

Correction in: Journal of Inherited Metabolic Disease, vol. 38, issue 2, pages 377-377.

DOI: 10.1007/s10545-014-9786-4

ISI: 000350360200026

Available from: 2014-12-11 Created: 2014-12-11 Last updated: 2017-12-05Bibliographically approved

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Gustafsson, JanStenlid, Maria Halldin

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