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[Primary biliary cirrhosis].

Kh Brailski, B Damianov

    Vutreshni Bolesti
    |January 1, 1983
    PubMed
    Summary

    Primary biliary cirrhosis (PBC) diagnosis relies on symptoms like itching and jaundice, with key lab markers including elevated alkaline phosphatase (AP) and antimitochondrial antibodies. Early diagnosis aids management, though PBC prognosis remains variable.

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    Area of Science:

    • Hepatology
    • Gastroenterology
    • Internal Medicine

    Background:

    • Primary biliary cirrhosis (PBC) is a chronic liver disease affecting predominantly women.
    • Common symptoms include pruritus, jaundice, and hepatosplenomegaly.
    • Characteristic laboratory findings involve elevated liver enzymes and specific autoantibodies.

    Purpose of the Study:

    • To report findings from a study on primary biliary cirrhosis patients.
    • To compare diagnostic markers and methods for PBC.
    • To evaluate treatment outcomes and patient survival in PBC.

    Main Methods:

    • Study involved 26 patients with primary biliary cirrhosis (20 female, 6 male, average age 46).
    • Diagnostic evaluation included clinical symptoms, laboratory tests (serum 5'-nucleotidase, AP, LAP, gamma GTP, GOT, cholesterol, cholic acid, antimitochondrial antibodies, IgM), and imaging (percutaneous transhepatic cholangiography, echography, laparoscopy, scintigraphy, liver biopsy).
    • Treatment modalities assessed included cholestrimine, corticosteroids, azathioprine, and surgical intervention.

    Main Results:

    • Elevated serum 5'-nucleotidase, alkaline phosphatase (AP), and antimitochondrial antibodies were significant for early PBC diagnosis.
    • Diagnostic accuracy varied: echography (81%), laparoscopy (73%), scintigraphy (61.53%), and liver biopsy (50%).
    • Treatments showed temporary improvement but disease progression was observed; 9 out of 20 followed patients died within 5 years of diagnosis.

    Conclusions:

    • Early diagnosis of PBC is crucial, aided by specific serological markers and imaging techniques.
    • Prognosis for PBC patients is variable, with a significant mortality rate within five years.
    • Long-term survival is possible, with some patients living over a decade after diagnosis.

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