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Increased intestinal clearance of alpha 1-antitrypsin in patients with alpha 1-antitrypsin deficiency

B B Grill, T Tinghitella, C Hillemeier

    Journal of Pediatric Gastroenterology and Nutrition
    |January 1, 1983
    PubMed

    Insights

    Patients with alpha 1-antitrypsin (alpha 1-AT) deficiency show increased intestinal clearance of alpha 1-AT. However, this elevated fecal loss is not the primary cause of low serum alpha 1-AT levels in these individuals.

    Area of Science:

    • Gastroenterology
    • Pulmonology
    • Genetics

    Background:

    • Alpha 1-antitrypsin (alpha 1-AT) deficiency is characterized by low serum levels of alpha 1-AT.
    • Enteric losses may contribute to reduced serum alpha 1-AT concentrations.

    Purpose of the Study:

    • To investigate the intestinal clearance of alpha 1-AT in patients with alpha 1-AT deficiency.
    • To determine if increased fecal alpha 1-AT losses contribute to low serum levels.

    Main Methods:

    • Measured intestinal clearance of alpha 1-AT (C-alpha 1-AT) in alpha 1-AT deficient patients, controls with gastrointestinal disorders, and patients with liver disease.
    • Quantified alpha 1-AT in stool and serum using radial immunodiffusion.
    • Calculated the percentage of daily alpha 1-AT turnover attributed to stool losses.

    Main Results:

    • Patients with alpha 1-AT deficiency exhibited significantly higher C-alpha 1-AT compared to controls.
    • Increased C-alpha 1-AT in deficiency patients was not linked to liver disease.
    • Fecal losses accounted for a similar percentage of daily alpha 1-AT turnover across all groups.

    Conclusions:

    • Alpha 1-AT deficiency is associated with increased fecal clearance of alpha 1-AT.
    • Increased intestinal losses of alpha 1-AT are not a major factor in the pathogenesis of low serum levels in alpha 1-AT deficiency.

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