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.

Direct Link: https://doi.org/10.1007/s10545-014-9750-3

Journal: Journal of inherited metabolic disease. 2015 Mar;38(2):315-22

Keywords: children, fasting period, fatty acid accumulation, glycerol production, Heart Rate, metabolic decompensation, subcutaneous adipose tissue,

Applications: Heart Rate,

CamNtech Reference: AH15070

Back to Search Results

UK & International customers

CamNtech Ltd.
Manor Farm
Fenstanton
Cambridgeshire
PE28 9JD, UK

US customers

CamNtech Inc.
630 Boerne Stage Airfield,
Boerne,
Texas 78006,
USA

Copyright

© 2020 CamNtech Ltd and CamNtech Inc

Company information

Registered in England No. 2221302
VAT No: GB486 3019 34


Privacy Policy