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Primary sclerosing cholangitis

Thirty six consecutive patients with primary sclerosing cholangitis (PSC), 20 males median age 42 years, were studied in order to define prognostic variables and determine the influence of surgery on outcome. Presentation was usually with insidious cholestasis or recurrent cholangitis. Twenty six pa...

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Main Author: Lemmer, Eric Richard
Format: Thesis
Language:English
Published: Department of Medicine 2017
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access_status_str Open Access
author Lemmer, Eric Richard
author_browse Lemmer, Eric Richard
author_facet Lemmer, Eric Richard
author_sort Lemmer, Eric Richard
collection Thesis
description Thirty six consecutive patients with primary sclerosing cholangitis (PSC), 20 males median age 42 years, were studied in order to define prognostic variables and determine the influence of surgery on outcome. Presentation was usually with insidious cholestasis or recurrent cholangitis. Twenty six patients (72 per cent) had associated inflammatory bowel disease (ulcerative colitis 20, Crohn's disease 2, unclassified 4). Thirty two patients were followed prospectively for up to nine years. Twenty three remained either stable or had slowly progressive disease. Of the remaining nine patients, seven died (five from end-stage liver failure and two from cholangiocarcinoma) and two patients underwent liver transplantation. Actuarial survival at five years was 52 per cent. A raised serum bilirubin concentration was the only variable at presentation that independently predicted a poor outcome. Cholangiograms were available for detailed assessment in thirty PSC patients. Neither the extent of biliary involvement nor the presence of surgical correctable ("dominant") strictures in the extrahepatic ductal system were of prognostic importance. Six patients who developed obstructive jaundice associated with advanced liver disease underwent surgical drainage operations for dominant biliary strictures, but this did not seem to prevent progression of the disease. Two patients who progressed to end-stage liver disease went on to liver transplantation and were alive with functioning grafts at seven and fourteen months respectively. Nine patients with asymptomatic PSC were followed prospectively for up to twelve years. None of these patients developed overt liver disease but serum bilirubin levels became mildly elevated in two patients. It is concluded that symptomatic PSC is a progressive disease with a poor prognosis. Patients with advanced liver disease due to PSC should be considered directly for liver transplantation. In contrast, asymptomatic PSC patients may remain symptom-free for many years.
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provenance_str_mv Harvested via OAI-PMH from UCTD — University of Cape Town Open Access Repository
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spelling oai:open.uct.ac.za:11427/25576 Primary sclerosing cholangitis Lemmer, Eric Richard Bile ducts - Radiography Thirty six consecutive patients with primary sclerosing cholangitis (PSC), 20 males median age 42 years, were studied in order to define prognostic variables and determine the influence of surgery on outcome. Presentation was usually with insidious cholestasis or recurrent cholangitis. Twenty six patients (72 per cent) had associated inflammatory bowel disease (ulcerative colitis 20, Crohn's disease 2, unclassified 4). Thirty two patients were followed prospectively for up to nine years. Twenty three remained either stable or had slowly progressive disease. Of the remaining nine patients, seven died (five from end-stage liver failure and two from cholangiocarcinoma) and two patients underwent liver transplantation. Actuarial survival at five years was 52 per cent. A raised serum bilirubin concentration was the only variable at presentation that independently predicted a poor outcome. Cholangiograms were available for detailed assessment in thirty PSC patients. Neither the extent of biliary involvement nor the presence of surgical correctable ("dominant") strictures in the extrahepatic ductal system were of prognostic importance. Six patients who developed obstructive jaundice associated with advanced liver disease underwent surgical drainage operations for dominant biliary strictures, but this did not seem to prevent progression of the disease. Two patients who progressed to end-stage liver disease went on to liver transplantation and were alive with functioning grafts at seven and fourteen months respectively. Nine patients with asymptomatic PSC were followed prospectively for up to twelve years. None of these patients developed overt liver disease but serum bilirubin levels became mildly elevated in two patients. It is concluded that symptomatic PSC is a progressive disease with a poor prognosis. Patients with advanced liver disease due to PSC should be considered directly for liver transplantation. In contrast, asymptomatic PSC patients may remain symptom-free for many years. 2017-10-11T10:54:30Z 2017-10-11T10:54:30Z 1993 2017-07-12T11:31:52Z Master Thesis Masters MMed http://hdl.handle.net/11427/25576 eng application/pdf Department of Medicine Faculty of Health Sciences University of Cape Town
spellingShingle Bile ducts - Radiography
Lemmer, Eric Richard
Primary sclerosing cholangitis
thesis_degree_str Master's
title Primary sclerosing cholangitis
title_full Primary sclerosing cholangitis
title_fullStr Primary sclerosing cholangitis
title_full_unstemmed Primary sclerosing cholangitis
title_short Primary sclerosing cholangitis
title_sort primary sclerosing cholangitis
topic Bile ducts - Radiography
url http://hdl.handle.net/11427/25576
work_keys_str_mv AT lemmerericrichard primarysclerosingcholangitis