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 familial type of cholangiodysplastic pseudocirrhosis is presented. This chronic liver disease is caused by malformation of the intrahepatic bile ducts. The female infant was 5 months old when the diagnosis was established. The liver biopsy was studied by light and electron microscopy. Electron microscopic examination revealed active proliferation of ductual cells and progression of fibrogenesis, findings consistent with the rapid and fatal course of the disease. In the case presented an acute cholangitis occurred, but after healing the progression of the original process led to hepatic insufficiency. It is suggested that cholangiodysplastic pseudocirrhosis is a chronic, progressive liver disease the course of which might be hastened by the complication of cholangitis; the process itself causes liver cirrhosis without inflammation.
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PMID:Cholangiodysplastic pseudocirrhosis: light and electron microscopic examination. 81 Sep 46

The prognosis of 97 patients with congenital biliary atresia treated by hepatic portoenterostomy was studied. In 37 of 97 patients (38%), good bile excretion was observed after surgery, and jaundice cleared. Sixteen of the 37 patients developed ascending cholangitis; 11, esophageal varices; and nine died. Seven died of ascending cholangitis and two of hepatic failure. Fourteen patients had an uneventful course. In the remaining 60 patients, bile excretion was scanty, and the disease progressed to biliary cirrhosis; 52 died. Causes of death were hepatic failure (53%), intracranial hemorrhage (16%), rupture of esophageal varices (12%), severe dehydration (9%), and postoperative complications (9%). The age at death ranged from 2 to 52 months, with an average at 16 months.
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PMID:Congenital biliary atresia. Analysis of 97 cases with reference to prognosis after hepatic portoenterostomy. 82 37

On the serum of 3790 patients (2131 women and 1659 male), aged from 3 days to 84 years, the RAC has been performed at the same time with the Mc Lagan's Kunkel's and Takata's reactions. The test of sulfobromophthalein's excretion has been performed in 246 of these patients. Between RAC and T.D., disagreement has been observed in 111 cases (- 2,9%). RAC positive and T.D. negative occurred in 68 cases; T.D. positive and RAC negative in the other 43. Repetition of the RAC on 100 sera (after complement inactivation at 56 degrees C for 30 min; after permanence at 4 degrees C for 7 days; after congealment at -18 degrees C) has always given the same results obtained on fresh sera. The complement, therefore, has no way influence on the RAC. The study of disagreeing cases has shown the RAC become positive for: 1) an apperance in serum of abnormal lipoproteins (chloestasis, cholangitis, some dislipemias), 2) a rise in gamma-M (biliary cirrhosis, macroglobulinaemia, lymphatic chronic leukema); 3) 1 and 2. It is always necessary to perform T.D. and RAC on the same serum, because these two test express different modification of the serum-proteins picture. Their agreement, or their disagreement, is very important in every single case, particularly in the diagnostic of jaundice and of bilary ducts pathology.
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PMID:[On the chloranilic acid reaction (RAC) proposed by Closs (author's transl)]. 82 64

After a short historical retrospect and a comment on the nomenclature and on the notion of chronic hepatitis and liver cirrhosis the diagnostic criteria and immunopathological peculiarities of virus-induced HBsAg-positive, non-virus-induced autoimmune, drug-induced and finally cryptogenic chronically progressing liver diseases are discussed. Immunoserology and immunohistology are nowadays to be regarded as the most important enrichments in the diagnostic spectre for the differentiation of chronic inflammatory liver diseases. In order to complete the diagnostic programme and to understand the pathogenesis of cryptogenic chronic hepatitides as soon as possible an establishment of the hepatitis-A- and C-serology is necessary. Apart from a further analysis of the group of the non-B-hepatitides the diagnostic use of other markers of virus hepatitides will be able to adopt a definite attitude to the unclarified question of virus-induced autoimmunopathies in liver diseases. The primary biliary cirrhosis with the morphologic findings of a chronically destructing, non-purulent cholangitis is an immunologically conditioned liver diseases of unknown etiology, which in contrast to the autoimmune chronic active hepatitides and liver cirrhoses is not to be influenced in the course by therapeutic measures.
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PMID:[Immunopathological aspects of liver cirrhosis]. 85 42

Liver biopsy was done at the time of operation in 125 consecutive upper abdominal procedures to assess the incidence of unsuspected or undiagnosed hepatic abnormalities. Specifically excluded were hepatic lesions unexpectedly identified at laparotomy. Sixty-seven percent of the liver biopsy specimens were abnormal, the most frequent findings being fatty metamorphosis, cholestasis, triaditis, fibrosis, inflammatory infiltrate, cholangitis, cirrhosis, and hepatitis. The most frequent operation performed was cholecystectomy. In 63 patients with chronic cholecystitis, there was a 51% incidence of abnormal liver histology, while in nine patients with acute cholecystitis, the incidence was 78%. In 83% of all other operations, abnormal liver biopsy specimens were identified. Bile leakage, hemorrhage, and infection did not occur in this series, despite inclusion of patients with severe biliary obstruction, abnormal clotting factors, and intra-abdominal sepsis. New techniques of histochemical enzyme analysis and electron microscopy are expected to enhance the clinical correlation of occult hepatic lesions. We conclude that liver biopsy in a safe, informative adjunct to all upper abdominal procedures.
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PMID:'Routine' liver biopsy in upper abdominal surgery. 88 45

Sclerosing diseases of the biliary system encompass a spectrum ranging from primary sclerosing cholangitis (usually of the extrahepatic biliary tree) to primary biliary cirrhosis of the intrahepatic bile canaliculi. In a study of 35 patients with primary intra- and extrahepatic biliary sclerosis, age of onset, sex distribution, symptomatology, associated diseases, radiographic abnormalities and chemical profile were considered. The difficulty of differentiating sclerosing cholangitis and biliary cirrhosis from other causes of obstructive jaundice preoperatively was stressed, in addition to points of differential clinical and laboratory findings. The etiology of these entities as well as the possibility that they represent variant clinical manifestations of the same disease process were also considered. Mechanical and pharmacological treatment alternatives that were attempted included drainage procedures, the easiest and most widely used of which was the T-tube. However, this could prove to be a source of infection and should therefore be removed early, inasmuch as cholangitis represents a major cause of morbidity. Steroids have been used with varying effectiveness; subjective improvement was generally attained, although objective improvement has been difficult to document. When choleuretics and cholestyramine were administered, we noted significant palliation. Antibiotics were reserved for treatment of cholangitis. Until the underlying etiology of this rare malignant sclerosing process is found, only symptomatic treatment can be offered.
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PMID:Sclerosing cholangitis and primary biliary cirrhosis--a disease spectrum? 92 53

The elimination of para-aminosalicylic acid (PAS) after the intravenous injection of 20 mg PAS sodium/kg was estimated in patients with liver disease, in uremic patients and in volunteers without damage of the liver or kidneys. The drug was estimated with a colorimetric and fluorometric method. In the volunteers, the half-lives obtained with the fluorometric method were significantly longer than those estimated with the colorimetric method. This is caused by the estimation of more PAS metabolites by the used fluorometric method. In the patients with renal insufficiency (dialysis patients) the elimination rate of unchanged PAS--estimated with the colorimetric method--was not altered, whereas the elimination of PAS and its metabolites extractable by ethyl acetate was markedly slowed in comparison with the results obtained with the volunteers. The clearance of the unchanged PAS was even increased in the uremic patients. The serum protein binding of PAS was lowered significantly in the serum of uremic patients. In patients with liver cirrhosis, acute virus hepatitis and cholangitis the elimination rate of the drug was not altered in comparison with the volunteers. The results show that the dose of PAS in patients with renal insufficiency may not be reduced. The therapeutic level of the drug cannot otherwise be reached in these patients.
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PMID:Elimination of para-aminosalicylic acid in patients with liver disease and renal insufficiency. 92 30

Long strictures of the intrapancreatic portion of the common bile duct were found in 6 patients with chronic pancreatitis. These strictures were responsible for painless obstructive jaundice, recurrent cholangitis, secondary biliary cirrhosis, and chronic abdominal pain difficult to distinguish from that caused by pancreatitis. Endoscopic retrograde cholangiopancreatography and intraoperative cholangiography were invaluable in making the diagnosis and in planning surgical correction. Decompression of the biliary tree by anastomosis of the gallbladder or common duct to the small intestine completely relieved symptoms and allowed liver function to improve significantly. Common duct stricture as a complication of chronic pancreatitis should be considered in the differential diagnosis of extrahepatic biliary obstruction and whenever surgical treatment of chronic pancreatitis is contemplated.
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PMID:Persistent obstructive jaundice, cholangitis, and biliary cirrhosis due to common bile duct stenosis in chronic pancreatitis. 94 56

Transient bacteremia associated with percutaneous liver biopsy was studied by pour-plate blood cultures, which were obtained immediately before and after the procedure and 5, 10, 15, and 30 min later in 89 patients. Part of the liver tissue was also cultured in all patients. Histological diagnoses included hepatitis, cirrhosis, cholangitis, fatty liver, granulomata, metastatic liver disease, lymphoma, and miscellaneous disorders. All blood cultures obtained before liver biopsy were sterile. Bacteremia was demonstrable in 12 patients (13.48%). In most of these patients, blood cultures were positive for as long as 15 min after liver biopsy; all cultures were negative at 30 min. Among the bacteria associated with 12 episodes of bacteremia were Escherichia coli, Klebsiella, Bacteroides, enterococci, diphtheroids, Staphylococcus aureus, alpha-hemolytic Streptococcus, and Streptococcus pneumoniae. The patients with positive liver biopsies had a higher incidence of bacteremia (83.3%) than did the patients whose liver biopsies were sterile (8.r%); this difference is stastically significant (P smaller than 0.01). Thus, liver biopsy can be associated with transient bactermia.
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PMID:Transient bacteremia associated with percutaneous liver biopsy. 109 72

Fourteen patients with "noncorrectable" biliary atresia are living without jaundice for more than 2 yr after hepatic portoenterostomy or its modification. Retardation of physical growth was observed in one of them, and mental retardation in another, both of which seemed irrelevant to biliary atresia. Serial tests for liver function after operation revealed early recovery of serum bilirubin, transminase, and turbidity, and delayed improvement of alkaline phosphatase. Postoperative needle biopsy of the liver disclosed that changes in hepatic parenchyma and ductular proliferation were rapidly improved after successful operation. Improvement of fibrosis of the liver was delayed, and it was not satisfactory in patients whose preoperative changes in the liver were severe or in whom ascending cholangitis had been a frequent complication. Histologic features of hepatic cirrhosis were observed in the liver in three cases, in two of which there had been frequent episodes of cholangitis. Only one of these showed clinical signs of portal hypertension. Functional and morphologic cure can be achieved in "noncorrectable" biliary atresia by hepatic portoenterostomy or its modifications, although varying degree of hepatic fibrosis may remain according to severity of preoperative changes of the liver and postoperative complication of ascending cholangitis.
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PMID:Follow-up studies of long term survivors after hepatic portoenterostomy for "noncorrectible" biliary atresia. 112 98


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