Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Portland experience with patients requiring proctocolectomy for chronic ulcerative colitis in association with primary sclerosing cholangitis has been reported. Nineteen patients had conventional ileostomy reconstruction, 5 of whom had development of stomal varices with recurrent hemorrhage due to cirrhosis and portal hypertension. When this combination of conditions exists, the therapeutic options must be carefully weighed. Perhaps ileal pouch to anal anastomosis should be considered when proctocolectomy becomes mandatory. Ileostomy is presently a contraindication to liver transplantation. In patients with ileostomies, control of hemorrhage by local measures, including ileostomy revisions, proved to be of only temporary value, yet it should be the preferred management of patients with a severely limited life expectancy. Successful shunts directed at portal decompression have always prevented further ileostomy hemorrhage, however, they have commonly accelerated liver failure and thus death. Furthermore, such shunts seriously complicate liver transplantation if it is considered.
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PMID:Exigent ileostomy hemorrhage. A complication of proctocolectomy in patients with chronic ulcerative colitis and primary sclerosing cholangitis. 316 Feb 51

We report 12 cases of primary sclerosing cholangitis (confirmed by cholangiography) in which the serum alkaline phosphatase activity was normal. The enzyme activity remained normal during follow-up in 7 cases and fluctuated in 5 cases (it returned to normal in 4). The presence of advanced histologic stage (fibrosis/cirrhosis) with marked cholangiographic changes in 4 patients establishes that cirrhotic-stage primary sclerosing cholangitis can occur without a concomitant increase in serum alkaline phosphatase activity. Therefore, primary sclerosing cholangitis may exist in an occult state without symptoms or increase in serum alkaline phosphatase activity. Our findings suggest that primary sclerosing cholangitis may be more prevalent than realized, especially in patients who have inflammatory bowel disease. A normal value for serum alkaline phosphatase activity should not preclude further investigation for primary sclerosing cholangitis in patients with inflammatory bowel disease when symptoms or signs suggest liver disease.
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PMID:Primary sclerosing cholangitis with normal serum alkaline phosphatase activity. 316 3

A rare autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver is described. The patient, a 62-year-old female at the time of autopsy, presented with prolonged cholestasis of about 9 years duration. Initial (at 53 years) and second (at 59 years) liver biopsies disclosed fibrous enlargement of the portal tracts with loss of interlobular bile ducts, lymphoplasmacytic infiltration, a few epithelioid granulomas, piecemeal necrosis, atypical ductular proliferation and deposition of copper granules. Hypergammaglobulinemia with elevated IgM was also noted. These clinicopathological features resembled primary biliary cirrhosis. However, no florid duct lesions were found, and absence of antimitochondrial antibodies and cholangiographic demonstration of a beaded biliary tree favored a diagnosis of primary sclerosing cholangitis. The autopsied liver disclosed sclerosis and cholangioectases of the intra- and extrahepatic biliary tree in addition to biliary cirrhosis. The histology of the biliary tree disclosed nonspecific fibrosing inflammation in the extra- and intrahepatic biliary tree. Other autopsy findings included chronic thyroiditis, sialoadenitis and pancreatitis.
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PMID:An autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver. 338 52

A variety of biliary and hepatocellular diseases occur with increased incidence in patients with inflammatory bowel disease. These include fatty infiltration of the liver, cholelithiasis, pericholangitis-primary sclerosing cholangitis, cirrhosis, chronic active hepatitis, liver abscess, amyloidosis, granulomatous hepatitis, and bile duct carcinoma. Radiography is essential in accurate diagnosis.
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PMID:Hepatobiliary complications of inflammatory bowel disease. 354 68

Hepatobiliary diseases are certainly not very frequent extraintestinal complications of chronic intestinal inflammatory diseases, however, they are an important prognostic factor. 2% of patients with ulcerative colitis develop liver cirrhosis but 10% of those die as a direct result of liver failure. Other associated severe hepatobiliary diseases include primary sclerosing cholangitis, carcinoma of the bile duct and amyloidosis. The present review attempts to divide the associated hepatobiliary diseases into three groups. 1. those that are the result of therapy. 2. those that are the result of the pathophysiological mechanisms of the underlying disease and 3. those of unknown etiological origin. This division might serve not only for a better understanding of the various mechanisms but should have some impact on therapeutic regimens.
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PMID:[Hepatobiliary diseases in Crohn disease and ulcerative colitis]. 356 Nov 41

Percutaneous cholecystography was performed on 13 children who had biliary system abnormalities: two had biliary hypoplasia, five had sclerosing cholangitis, three had cirrhosis, two had distal choledochal obstruction, and one had an obstructed portoenterostomy. In 12 patients transcholecystic cholangiography showed, without significant complications, the intra-and extrahepatic bile ducts. In one patient with primary sclerosing cholangitis, the intrahepatic bile ducts were not opacified satisfactorily; dilatation of the gallbladder required surgical drainage. The transcholecystic technique is indicated when the intrahepatic bile ducts are either mildly dilated or not dilated.
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PMID:Percutaneous cholecystography in children. 368 43

Review of liver biopsy specimens, autopsy specimens, and clinical records of 107 patients with chronic ulcerative colitis and hepatobiliary diseases showed "pericholangitis" (defined as small-duct primary sclerosing cholangitis) in 37 (35%), primary sclerosing cholangitis (defined as large-duct primary sclerosing cholangitis) in 18 (17%), chronic active hepatitis in 14 (13%), cryptogenic cirrhosis in 12 (11%), and miscellaneous lesions including malignancies in 26 (24%). Documented cirrhosis was present or developed in 37 patients (35%). The spectrum of histologic features of small-duct primary sclerosing cholangitis was indistinguishable from that of confirmed large-duct primary sclerosing cholangitis. In 6 of the 18 patients who eventually developed the large-duct disease, biopsy evidence 1 to 12 years earlier had shown small-duct primary sclerosing cholangitis. Thus, small-duct and large-duct primary sclerosing cholangitis seem to be components of a disease spectrum.
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PMID:Pericholangitis in chronic ulcerative colitis: primary sclerosing cholangitis of the small bile ducts? 398 11

Primary sclerosing cholangitis is a chronic, cholestatic syndrome characterized by fibrosing inflammation of the bile ducts that may lead to cirrhosis and death from liver failure. Previous reports have suggested abnormal hepatic copper metabolism in this disease. Therefore, in 70 patients, we prospectively determined the levels of hepatic copper, serum copper, and serum ceruloplasmin, and the rate of urinary copper excretion to assess the diagnostic and prognostic usefulness of these tests. Virtually all patients had at least one abnormal copper test. Hepatic copper levels were elevated in 87% of patients [292 +/- 38 micrograms/g dry wt (mean +/- SE)] and 24-h urinary copper levels in 64% of patients [135 +/- 15 micrograms/24 h (mean +/- SE)] to values comparable to those seen in Wilson's disease or primary biliary cirrhosis. In advanced histologic stages of primary sclerosing cholangitis, progressively higher mean levels of hepatic and urinary copper were found. In the liver, mean copper content (in micrograms per gram dry weight) in disease stages I and II was 147 +/- 36 (mean +/- SE); in stage III (fibrosis), 302 +/- 68; and in stage IV (cirrhosis), 379 +/- 69. In the urine, mean copper excretion (in micrograms per 24 h) in stages I and II was 72 +/- 14 (mean +/- SE); in stage III, 100 +/- 14; and in stage IV, 207 +/- 30. Higher hepatic and urinary copper levels at initial evaluation were associated with decreased survival during a median follow-up period of 2.6 yr: patients with hepatic copper greater than 250 micrograms/g dry wt and urinary copper excretion greater than 200 micrograms/24 h at initial evaluation had an 18-mo survival of less than 60%. We conclude that abnormal copper metabolism is a universal feature of primary sclerosing cholangitis, that hepatic copper accumulates and urinary copper excretion increases as the disease progresses, and that the hepatic copper concentration and the 24-h urinary copper determination are useful prognostic indicators in this disease.
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PMID:Abnormalities in tests of copper metabolism in primary sclerosing cholangitis. 400 18

Hepatobiliary carcinomas were found in eight patients with chronic ulcerative colitis (CUC) and primary sclerosing cholangitis (large-duct PSC; five cases) or "pericholangitis" (small-duct PSC; three cases). The tumors were extrahepatic in five cases and intrahepatic in two; in one case the neoplasm affected both liver and gallbladder. The tumors in seven patients were glandular and, sometimes, cystic and papillary; in the remaining patient a combined hepatocellular carcinoma and cholangiocarcinoma was found. The latter tumor seemed to arise from regenerative nodules in secondary biliary cirrhosis complicating PSC. The presence of carcinoma in situ in areas of fibrous cholangitis, the multicentric origin of the tumor, the presence of tumor-free large-duct PSC or small-duct PSC (pericholangitis) at a distance from the carcinomatous areas, and the documentation, in some cases, of long-standing inflammatory hepatobiliary disease prior to the discovery of the tumors would seem to confirm the clinical impression that carcinomas may develop in pre-existing PSC. The appearance of hepatobiliary carcinomas in patients with classic PSC and in patients with pericholangitis supports previous evidence indicating that cholangiographically diagnosed large-duct PSC and histologically diagnosed small-duct PSC (pericholangitis) are manifestations of a shared condition that could be named PSC syndrome. The findings of the present study indicate that the PSC syndrome predisposes patients for the development of bile duct carcinoma. Most patients with CUC and bile duct carcinoma seem to have PSC prior to the development of the hepatobiliary tumor.
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PMID:Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. 400 48

Primary sclerosing cholangitis is now being recognized as the underlying cause of liver disease with increasing frequency. This is due to the widespread use of cholangiography especially endoscopic retrograde cholangiopancreatography. Hepatic histology shows a spectrum of changes and is diagnostic in only a minority of patients. Several distinct clinical presentations of primary sclerosing cholangitis are now recognized. While most patients present with a cholestatic illness and acute cholangitis, a proportion simulate chronic active hepatitis, cryptogenic cirrhosis or primary biliary cirrhosis. Some patients are entirely asymptomatic. Primary sclerosing cholangitis is associated with inflammatory bowel disease (predominantly ulcerative colitis) and a variety of fibrosing diseases. Approximately 3 percent of patients with ulcerative colitis develop primary sclerosing cholangitis. The prognosis of primary sclerosing cholangitis is variable; it can no longer be regarded as an invariably progressive cholestatic disease. Patients may remain asymptomatic or enjoy remissions lasting many years. Treatment for the disease remains unsatisfactory despite attempts with numerous medical and surgical measures.
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PMID:Primary sclerosing cholangitis. 635 17


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