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Related Experiment Videos

Dicarboxylic aciduria: the response to fasting.

R J Truscott, L Hick, C Pullin

    Clinica Chimica Acta; International Journal of Clinical Chemistry
    |May 16, 1979
    PubMed
    Summary

    A child with Reye's syndrome-like illness excreted excess dicarboxylic acids, particularly adipic and suberic acids. Fasting worsened this excretion, suggesting a fatty acid oxidation defect.

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    Area of Science:

    • Biochemistry
    • Metabolic disorders
    • Pediatric diseases

    Background:

    • Reye's syndrome is a serious condition affecting the brain and liver.
    • Fatty acid oxidation is crucial for energy production, especially during fasting.

    Observation:

    • Urine analysis of a child resembling Reye's syndrome revealed high levels of adipic and suberic acids.
    • Unusual dicarboxylic acids and suberyl glycine were also detected in the patient's urine.

    Findings:

    • Urinary excretion of these dicarboxylic acids significantly increased after fasting for over 10 hours.
    • This suggests a potential impairment in the body's ability to break down fatty acids.

    Implications:

    • The findings point to a possible inborn error of metabolism affecting fatty acid oxidation.

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  • This defect may manifest clinically during prolonged fasting periods in susceptible individuals.