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Bearn-Kunkel-Slater syndrome

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Obsolete term for "lupoide hepatitis" as course of an autoimmune chronic hepatitis seen in young women. It is characterized by liver cirrhosis with extreme hypergammaglobulinemia and an increase of plasma cells. Insidious onset as amenorrhea, acne, hirsutism, moon facies, and obesity, with consecutive hepatomegaly and splenomegaly. Hypergammaglobulineamia, painful joints, spider telangiectasia, esophageal varices, fever, respiratory and cardiovascular disorders, and liver pain are the late symptoms. Onset usually at puberty, but it may affect anyone from childhood to old age. Etiology unknown. Some writers consider this and Waldenström's hepatitis to be the same entity.

Bibliography

  • A. G. Bearn, H. G. Künkel, R. J. Slater:
    The problem of chronic liver disease in young women.
    American Journal of Medicine, New York, 1956, 21: 3-15.

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