Natural history of XXXX and prognostic value of cholangiography in a Dutch population.

@entity1 @entity991 @entity409 @entity29 @entity1463 @entity854 @entity201 @entity204
BACKGROUND: Median survival of @entity1 with @entity991 ( @entity991 ) has been estimated to be 12 years. Cholangiography is the gold standard for diagnosis but is rarely used in estimating prognosis. AIMS: To assess the natural history of Dutch @entity991 @entity1 and to evaluate the prognostic value of a cholangiographic classification system. @entity1 : A total of 174 @entity1 with established @entity991 attending a university hospital and three teaching hospitals from 1970 to 1999. METHODS: Charts were reviewed for validity and time of diagnosis, concurrent @entity29 , interventions, liver transplantation, occurrence of @entity201 , and @entity204 . Follow up data were obtained from the charts and from the attending clinician or family physician. Median follow up was 76 months (range 1-300). The earliest available cholangiography was scored using a radiological classification system for the severity of @entity1463 , developed in our institution. Survival curves were computed by the Kaplan-Meier method. Cholangiographic staging was used to construct a prognostic model, applying Cox proportional hazards analysis. RESULTS: The estimated median survival from time of diagnosis to @entity204 from @entity409 or liver transplantation was 18 years. @entity201 was found in 18 (10%) @entity1 . Fourteen @entity1 (8%) underwent liver transplantation. Cholangiographic scoring was inversely correlated with survival. A combination of intrahepatic and extrahepatic scoring, together with age at endoscopic retrograde cholangiopancreatography, proved strongly predictive of survival. CONCLUSIONS: The observed survival was considerably better than reported in earlier series from Sweden, the UK, and the USA. Classification and staging of @entity854 has prognostic value.
