Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mild liver dysfunction is a well-known complication of HAI, but it has been thought to be transient and reversible in most cases. In the case, of metastatic liver disease, in particular, HAI has been performed safely because liver function is normal for the most part. We encountered 2 cases of irreversible severe liver dysfunction and esophageal varices after hepatectomy for metastatic liver tumor from colorectal cancer. They were treated with postoperative adjuvant HAI. Biliary enzyme as alkaline phosphatase elevated, and dilated intrahepatic bile ducts were observed in both patients. Fibrosis of Glissonean sheath, dilatation of intrahepatic bile ducts and intrahepatic biliary stones were observed at autopsy in both patients. One of the patients had obstruction of portal trunk. It must not be forgotten that such complications can occur even in a case with non-cirrhotic liver.
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PMID:[Two cases of esophageal varices complication after hepatic arterial infusion chemotherapy (HAI) for metastatic liver tumor]. 132 19

A total of 832 patients with portal hypertension resulting from different etiology was studied by ultrasonograph as a screening test. In 17 of the 832 patients, cavernous transformation of the portal vein was detected by means of ultrasonography. We have prospectively studied these 17 patients, and the diagnosis of cavernous transformation was confirmed by portography in all patients. To evaluate how much biliary tract has been affected from cavernous transformation of the portal vein, and to explain the cause of mildly increased alkaline phosphatase and serum bilirubin levels, endoscopic retrograde cholangiopancreatography (ERCP) was performed in 16 of the 17 patients. There were narrowing, irregularity, undulation and nodular extrinsic defects resulting from compression of thrombosis of the portal vein and the collateral vessels, mimicking cholangiocarcinoma spreading along the common bile duct on the extrahepatic biliary tract in all 16 patients who underwent ERCP. Similar ERCP findings were not found in six patients with portal hypertension due to liver cirrhosis. The ultrasonographic, portographic, and ERCP findings corresponded to surgical findings in six patients who had undergone splenectomy for either hypersplenism or bleeding from esophageal varices. The results indicate that cavernous transformation of the portal vein cause the above-mentioned radiographic findings that we propose to call "pseudo-cholangiocarcinoma sign."
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PMID:Bile duct varices or "pseudo-cholangiocarcinoma sign" in portal hypertension due to cavernous transformation of the portal vein. 144 45

Three patients with symptomatic, noncirrhotic primary biliary cirrhosis who had no evidence of esophageal varices on esophagogastroduodenoscopy and who were treated with ursodeoxycholic acid, 15 mg.kg-1.day-1, for a period of 1-2 years are reported. Initially, all three patients showed improvement in symptoms of fatigue and pruritus, and there was marked improvement or normalization in serum levels of bilirubin, alkaline phosphatase, and alanine aminotransferase. However, after 1-2 years, all three patients progressed histologically to cirrhosis on follow-up liver biopsy, and all had esophageal variceal bleeding documented by esophagogastroduodenoscopy. These three patients represent examples of ursodeoxycholic acid treatment failure despite improvements in symptoms and biochemical liver test results.
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PMID:Progression of primary biliary cirrhosis with ursodeoxycholic acid therapy. 829 25

Congenital hepatic fibrosis (CHF) is a recognized cause of portal hypertension with oesophageal varices, gastro-intestinal haemorrhage and cholangitis in children without significant impairment of hepatic or renal function. This report describes the varied clinical presentation of CHF as seen at King Faisal Specialist Hospital and Research Centre (KFSH & RC) and emphasizes the clinical patterns that should enable a pediatrician to consider the diagnosis. Fourteen children with CHF were diagnosed between 1981 and 1988. The age at presentation ranged from 1.8-14 years (mean: 7.5 years); clinical manifestations at diagnosis were splenomegaly (12), hepatomegaly (11), failure to thrive (10), marked abdominal distention (4), and fever (4). Liver function tests were normal except for high alkaline phosphatase. Eight patients had polycystic kidneys confirmed on ultrasound examination. Upper gastro-intestinal endoscopy showed oesophageal varices of variable severity in all eight patients examined. Splenoportography revealed splenic vein occlusion in one patient. One patient died within days of admission with convulsions, coma, and aspiration pneumonia. One patient was lost to follow-up. The remaining 12 patients are all alive and receive regular follow-up. Two patients required splenorenal shunt. In view of the prevalence of consanguinity in Saudi Arabia, the diagnosis of CHF should be considered in children with hepatomegaly despite normal liver function tests, and particularly in those with renal abnormalities and/or evidence of portal hypertension.
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PMID:Congenital hepatic fibrosis in Saudi Arabia. 178 58

Among 30 consecutive patients diagnosed with primary biliary cirrhosis (PBC) in Taiwan, 27 were females and the median age of symptom onset was 54.5 years. Most had similar clinical manifestations to those reported in the Western countries, but ascites and oesophageal varices as commonly found at the late stages of cirrhosis of liver were noted in nine patients (30%) and 13 patients (43%) respectively. Only one patient was asymptomatic. Hyperbilirubinaemia was noted in 21 patients (70%) and hypoalbuminaemia in 8 patients (27%). All patients had elevated serum alkaline phosphatase and alanine aminotransferase and 28 (93%) had antimitochondrial antibodies. Ten out of 21 patients (48%) were positive in antinuclear antibodies, of which most were of speckled type. Sixteen out of 18 patients (89%) had elevated serum IgM levels. Interestingly, only one of 26 patients (3.8%) was positive for hepatitis B surface antigen, in contrast to its high prevalence (15%) in the Taiwan population. Special associated diseases, including systemic lupus erythematosus, scleroderma, malignant lymphoma and hepatocellular carcinoma, were each noted in one patient respectively. Eight patients had a history of gallstones before the diagnosis of PBC. The mean follow-up period was 23.6 +/- 19.8 months, and nine patients died during that period. In conclusion, the clinical manifestations of PBC in Taiwan are similar to those in Western countries, but most of our cases were at later stages.
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PMID:Primary biliary cirrhosis in Taiwan. 212 28

The clinical and pathological findings of five adult cases of idiopathic nonsyndromatic paucity of interlobular bile ducts are reported. Patients were 18-32 years old at the onset of the disease; four presented with pruritus and/or jaundice and one with bleeding of the esophageal varices. Two patients were siblings. Serum alkaline phosphatase counts ranged from 1 to 16 times the upper normal value, and total bilirubin counts ranged from 0.6 to 8.8 mg/dL (10 to 150 mumol/L). Initial liver biopsy showed portal and periportal fibrosis with cholangiolar proliferation and reduction in the number of interlobular bile ducts. Antimitochondrial antibodies were absent, and bile ducts were normal after opacification. The patients were observed for 3-11 years. Repeated liver biopsies in the five patients showed progression of the lesions, with development of biliary type cirrhosis in four. Two of the four patients with cirrhosis died of hepatic failure 3 and 11 years after onset of the disease. In the two other cases, liver transplantation was performed successfully. These cases suggest that chronic cholestasis with marked ductopenia resembling the nonsyndromatic paucity described in infancy and childhood may reveal itself at an adult age. This disorder, possibly familial, may rapidly progress to severe and even fatal liver disease and could be a new indication for liver transplantation.
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PMID:Idiopathic biliary ductopenia in adults: a report of five cases. 222 98

Autoimmune chronic active hepatitis (ACAH) is an infrequent modality of chronic hepatitis (CH) with clinical and laboratory findings of an autoimmune disorder. Clinical and pathological findings of 7 cases are presented; all were females with ages between 7 and 24 years. Main symptoms and signs were weight loss, malaise, arthralgias, fever, menstrual disturbances, hepatosplenomegaly, jaundice ascites and esophageal varices. Aminotransferases were elevated in all cases, and bilirubin, alkaline phosphatase and gammaglobulins were found to be raised in six. Antinuclear antibodies were positive in 5 cases, smooth muscle antibodies in 3, and antimitochondrial antibodies were detected in one. Morphological changes were those of chronic active hepatitis with variable degrees of fibrosis. Plasma cells were conspicuous. All patients received steroid treatment (Prednisone). It is concluded that the diagnosis of ACAH can be based on clinical and immunological criteria provided other causes of CH such as viral hepatitis, are ruled out.
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PMID:[Autoimmune chronic hepatitis: clinico-pathologic spectrum in 7 cases]. 251 21

A retrospective study was made of the clinical features, especially oesophageal varices, of 93 patients with PBC. The 5 year survival rate of asymptomatic PBC patients was 88.7% and that of symptomatic PBC was 43.7%. The 5 year survival rate of the group with oesophageal varices was 44.0% and that of the group without varices was 68.8%. The 5 year survival rate of the patients with high-risk varices was 39.1% and of those without high-risk varices was 67.9%. Management of variceal bleeding in PBC patients was considered very difficult. In prognostic study, the patients with the prophylactic therapy were better than the patients with emergency or elective therapy. The antiM8 (a subtype of antimitochondrial antibody) positive patients had poor prognosis compared with antiM8 negative patients. Therefore, it was concluded from these data that some kind of treatment was necessary for patients with high-risk varices. In particular, it was considered necessary to monitor closely the patients whose serum alkaline phosphatase levels had remained high.
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PMID:Immunological response and oesophageal varices in PBC. 251 87

The effect of proctocolectomy on the primary sclerosing cholangitis that frequently is associated with chronic ulcerative colitis in patients with both conditions is unknown. We have studied prospectively the progression of clinical, biochemical, cholangiographic, and hepatic histologic features in 45 patients with both primary sclerosing cholangitis and chronic ulcerative colitis to compare these variables in the 20 patients who had undergone proctocolectomy with the 25 who had not. The two groups were similar initially with regard to clinical, biochemical, cholangiographic, and hepatic histologic findings. All patients were followed for a minimum of 1 yr and overall duration of follow-up was similar in both groups (4.1 vs. 3.9 yr). Clinically, new onset of hepatomegaly, splenomegaly, esophageal varices, and ascites did not differ in patients with and without proctocolectomy. Biochemically, the serial changes in bilirubin, alkaline phosphatase, aspartate aminotransferase, prothrombin time, and albumin were similar. Histologic progression on liver biopsy did not differ between groups, nor did changes on serial cholangiograms. Proctocolectomy also had no effect on survival. We conclude that proctocolectomy for chronic ulcerative colitis has no beneficial effect on the primary sclerosing cholangitis in patients with both diseases.
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PMID:Effect of proctocolectomy for chronic ulcerative colitis on the natural history of primary sclerosing cholangitis. 291 41

The aim of this paper is to evaluate the relationships among the increase of serum bile acids (SBA) and other common liver function tests in subjects with liver cirrhosis. Our results show that SBA levels are well-correlated with the seriousness of the disease (classified according to Child's criteria), and with the presence of ascites, of oesophageal varices, of hepatic encephalopathy and with the gamma-globulin level. SBA also appear to be well-correlated with total bilirubinemia, and, at a lower extent, with cholesterolemia and albuminemia; no significant linear correlation was found among SBA and cholestasis (alkaline phosphatase, gamma-glutamyl-transpeptidase) or cytolysis (transaminases) indexes. In conclusion, the SBA increase in liver cirrhosis without evidence of cholestasis (as in our patients) seems to be related to liver cell reuptake disturbances and to the presence of porto-systemic shunts, with consequent alterations in entero-hepatic bile salt recirculation.
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PMID:[Serum bile acids in cirrhosis: correlation with liver function parameters and with the severity of the disease]. 367 66


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