2014, Number 4
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Ann Hepatol 2014; 13 (4)
Predicting outcome in primary biliary cirrhosis
Lammers WJ, Kowdley KV, van Buuren HR
Language: English
References: 97
Page: 316-326
PDF size: 149.35 Kb.
ABSTRACT
Primary biliary cirrhosis (PBC) is a slowly progressive autoimmune liver disease that may ultimately result in
liver failure and premature death. Predicting outcome is of key importance in clinical management and an
essential requirement for patients counselling and timing of diagnostic and therapeutic interventions. The
following factors are associated with progressive disease and worse outcome: young age at diagnosis, male
gender, histological presence of cirrhosis, accelerated marked ductopenia in relation to the amount of fibrosis,
high serum bilirubin, low serum albumin levels, high serum alkaline phosphatase levels, esophageal
varices, hepatocellular carcinoma (HCC) and lack of biochemical response to ursodeoxycholic acid (UDCA).
The prognostic significance of symptoms at diagnosis is uncertain. UDCA therapy and liver transplantation
have a significant beneficial effect on the outcome of the disease. The Mayo risk score in PBC can be
used for estimating individual prognosis. The Newcastle Varices in PBC Score may be a useful clinical tool
to predict the risk for development of esophageal varices. Male gender, cirrhosis and non-response to
UDCA therapy in particular, are risk factors for development of HCC.
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