Books > Medicine > Clinical & internal medicine > Hepatology
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Primary Biliary Cirrhosis - From Pathogenesis to Clinical Treatment (Hardcover, 1998 ed.)
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Primary Biliary Cirrhosis - From Pathogenesis to Clinical Treatment (Hardcover, 1998 ed.)
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The condition of prolonged obstructive jaundice with patent bile
ducts was first described in 1851 by Addison and Gull of Guy's
Hospital, London. The term primary biliary cirrhosis (PBC) was
defined in 1950 by Ahrens and colleagues of the Rockefeller
Institute, New York. The condition was considered rare but this
changed in 1965 with the discovery of a definitive diagnostic serum
mitochondrial antibody test and the recognition that a raised serum
alkaline phosphatase value, often discovered incidentally, could be
a diagnostic pointer. If the diagnosis is made earlier, the end
stages are rarely reached as death is replaced by liver
transplantation. On November 6th 1997, in Chicago, an International
Faculty discussed in depth the clinical features, pathogenesis and
treatment of PBC, no longer considered a rare disease. The course
of PBC is long, but some 18 years after the discovery of a positive
mitochondrial antibody test in a symptom free patient with normal
serum biochemistry, 83% will have developed abnormal tests and 76%
will be symptomatic. Identification of those who will progress
rapidly is difficult. The serum antimitochondrial profile may be
useful but this is a very specialist technique. Mathematical
prognostic models are useful in therapeutic trials and in the
selection and timing of patients for liver transplantation but have
limited value in individual patients. An increasing serum bilirubin
level remains the most important indicator of rapid progression.
Its value however can be negated by the use of ursodeoxycholic acid
which has a bilirubin-lowering effect."
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