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Tyrosyluria in marasmus.

P S Dhatt, A S Saini, I Gupta

    The British Journal of Nutrition
    |November 1, 1979
    PubMed
    Summary
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    Marasmus patients show elevated plasma tyrosine and urinary metabolites like p-hydroxyphenyl lactic acid (PHPLA). Ascorbic acid reduced PHPLA, suggesting its role in tyrosyluria due to impaired enzyme activity.

    Area of Science:

    • Biochemistry
    • Pediatric Nutrition
    • Metabolic Disorders

    Background:

    • Marasmus is a severe form of malnutrition.
    • Tyrosinemia, characterized by elevated tyrosine levels, can occur in infants.
    • Urinary metabolites of tyrosine, such as p-hydroxyphenyl lactic acid (PHPLA) and p-hydroxyphenyl acetic acid (PHPAA), are indicators of metabolic status.

    Purpose of the Study:

    • To investigate plasma tyrosine and urinary PHPLA and PHPAA levels in children with marasmus compared to controls.
    • To explore the effect of ascorbic acid administration on these metabolites.
    • To elucidate the biochemical basis of tyrosyluria in marasmus.

    Main Methods:

    • Comparative analysis of plasma and urinary metabolites.
    • Biochemical assays for tyrosine, PHPLA, and PHPAA.

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  • Intervention study with ascorbic acid administration.

    • Main Results:

    • Marasmus patients exhibited significantly higher plasma tyrosine and urinary PHPLA and PHPAA levels than controls.
    • While only 13.3% of marasmus patients had conventional tyrosyluria, 52.3% excreted high levels of PHPAA.
    • Ascorbic acid administration reduced PHPLA excretion but did not affect PHPAA levels.

    Conclusions:

    • Tyrosyluria in marasmus is linked to reduced activity of the hepatic enzyme 4-hydroxyphenyl pyruvate: oxygen oxidoreductase (PHPAA-oxidase), likely due to ascorbic acid deficiency.
    • High PHPAA excretion in marasmus is associated with the child's age and nutritional status and is independent of ascorbic acid.
    • Urinary PHPLA excretion serves as a reliable indicator of tyrosyluria in marasmus.