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. 2025 Jun;114(6):1445-1455.
doi: 10.1111/apa.17591. Epub 2025 Jan 21.

Assessment of Fasting Metabolism With Microdialysis Indicates Earlier Lipolysis in Children With VLCADD Than MCADD

David Olsson  1 , Charlotte Bieneck Haglind  1 , Maria Halldin  1 , Svetlana Lajic  1   2 , Anna Nordenström  1   3
Affiliations

Assessment of Fasting Metabolism With Microdialysis Indicates Earlier Lipolysis in Children With VLCADD Than MCADD

David Olsson et al. Acta Paediatr. 2025 Jun.

Abstract

Aim: To investigate fasting metabolism in children with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) and medium-chain acyl-CoA dehydrogenase deficiency (MCADD) using microdialysis technique.

Methods: Twelve patients (7 with VLCADD, 5 with MCADD, mean age 4.9 years, 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-h 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-h intervals in patients with VLCADD.

Results: Children with VLCADD showed signs of lipolysis after a median fasting time of 4.5 h, 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.

Conclusion: Children with VLCADD had a shorter time to initiation of lipolysis during fasting than children with MCADD. Clinical evaluation of fasting metabolism in beta-oxidation disorders should include assessment of lipolysis as an early and important determinant.

Keywords: Beta‐oxidation disorders; MCADD; VLCADD; fasting intolerance; microdialysis.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Study protocol.
FIGURE 2
FIGURE 2
Microdialysis monitoring of glycerol.
FIGURE 3
FIGURE 3
Acylcarnitines, fasting hormones and plasma glucose in patients with VLCADD.
See this image and copyright information in PMC

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