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Changes in pediatric plasma acylcarnitines upon fasting for refined interpretation of metabolic stress

Willemijn J van Rijt1, Rixt M van der Ende1, Catharina M L Volker-Touw2

  • 1University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Division of Metabolic Diseases, Groningen, The Netherlands.

Molecular Genetics and Metabolism
|July 8, 2019
PubMed

Insights

Pediatric fasting intolerance is serious, but age-specific plasma acylcarnitine reference values are lacking. This study establishes age-dependent reference ranges for acylcarnitines during fasting in children, aiding diagnosis.

Area of Science:

  • Biochemistry
  • Pediatric Metabolism
  • Clinical Diagnostics

Background:

  • Childhood fasting intolerance poses life-threatening risks, often linked to inborn errors of metabolism.
  • Plasma acylcarnitines are crucial biomarkers for fatty acid oxidation and diagnosing fasting intolerance.
  • Lack of established pediatric reference values for plasma acylcarnitines during fasting hinders accurate interpretation.

Purpose of the Study:

  • To establish age-dependent reference values for plasma acylcarnitines in children undergoing supervised fasting.
  • To analyze changes in metabolic parameters and acylcarnitine profiles during fasting across different pediatric age groups.
  • To provide data supporting the interpretation of fasting intolerance in children.

Main Methods:

  • Retrospective analysis of 48 supervised pediatric fasting studies (01/2005-09/2012).
  • Children were grouped by age: ≤24 months (A), 25-84 months (B), and ≥85 months (C).
  • Median and 2.5th-97.5th percentiles of metabolic parameters and acylcarnitines were determined at the start and end of fasting.

Main Results:

  • Hypoglycemia occurred in 21% of participants.
  • Older children (group C) had higher end-glucose and lower end-ketone concentrations.
  • Fasting altered acylcarnitine profiles, with significant increases in C2, C6, C12, C14, and C16, and decreases in free and C3-carnitine, with age-dependent differences observed.

Conclusions:

  • Fasting counter-regulatory mechanisms and acylcarnitine changes are age-dependent in children.
  • Established reference values improve the interpretation of individual fasting responses.
  • Data may aid in assessing safe fasting durations and treatment efficacy for pediatric patients.
Abstract

Keywords:
AcylcarnitineFasting intoleranceHypoglycemiaInborn error of metabolismMetabolic stressTandem mass spectrometry

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