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Updated: Jun 1, 2025
Measuring the Rate of Lipolysis in Ex Vivo Murine Adipose Tissue and Primary Preadipocytes Differentiated In Vitro
Published on: March 17, 2023
Assessment of Fasting Metabolism With Microdialysis Indicates Earlier Lipolysis in Children With VLCADD Than MCADD
David Olsson1, Charlotte Bieneck Haglind1, Maria Halldin1
1Department of Women's and Children's Health, Unit for Pediatric Endocrinology and Metabolic Disorders, Karolinska Institutet/Karolinska University Hospital, Stockholm, Sweden.
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.
