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)

A clinicopathologic study of congenital hepatic fibrosis in 21 patients confirms a strong association with autosomal recessive renal polycystic disease. The liver specimens were subclassified into two groups according to the severity of fibrosis, showing typical hepatic abnormalities in young infants (mean age 0.3 years) and increased hepatic fibrosis in older patients (mean age 19.6 yr) (p less than 0.02). Apparent progression to perilobular fibrosis with parenchymal nodularity occasionally resembled cirrhosis when the nodules had a regenerative appearance because of rounded contours and inapparent central veins. Progression of fibrosis was observed in second biopsy specimens from 2 cases, but not in that of a 3rd, suggesting that factors other than the heritable disorder itself may be responsible for evolving morphology. Identifiable factors that may have contributed to increased fibrosis included localized intrahepatic biliary obstruction and biliary sepsis with suppuration. A factor possibly contributing to the pathogenesis of biliary sepsis was intrahepatic biliary ectasia, i.e., Caroli's disease, which appears to be one morphologic expression of CHF. This study shows that the hepatic abnormality evolves over time and that it may be altered by secondary complications.
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PMID:Congenital hepatic fibrosis: evolving morphology. 322 22

Cirrhotic dogs without intrahepatic hypertension (IHH) never retain sodium or expand plasma volume. To test the hypothesis that IHH may cause urinary sodium retention early in cirrhosis, we prepared cirrhotic dogs (chronic biliary obstruction) who underwent simultaneous hepatic denervation with end-side portacaval anastomoses. Such animals, along with appropriate controls and unanesthetized were studied by balance techniques. In the experimental group, plasma volume never increased, and sodium retention did not occur until 2 days prior to the appearance of detectable ascites. In a sham-denervated group, plasma volume increased by 10% and sodium retention occurred on the average 8.4 days prior to ascites. When the portal veins were left intact, the sham-denervated group showed the usual magnitude of plasma volume expansion observed in cirrhotic dogs (18.3%) with a 7-day delay between sodium retention and ascites appearance. Those dogs with hepatic denervation demonstrated a 9.2-day delay with 12.6% expansion of plasma volume. When ascites was mobilized with a peritoneovenous valve, and dogs were subjected to a high salt diet (130 meq/day), denervated dogs excreted the load normally, whereas sham-denervated dogs retained sodium and developed anasarca. We conclude that in cirrhotic dogs with IHH, liver denervation prevents early non-volume-related sodium retention.
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PMID:Hepatic denervation alters first-phase urinary sodium excretion in dogs with cirrhosis. 366 18

Portal fibrosis is considered to be pivotal in the pathogenesis of portal hypertension associated with extrahepatic biliary obstruction. The histological features, however, include diffuse hepatocyte hyperplasia as well as portal fibrosis, but not cirrhosis, and it is possible that the contribution of hepatocyte hyperplasia in the initiation of portal hypertension is equally important. If so, we hypothesised that patients with biliary obstruction and a coincident condition such as liver atrophy, or hepatic resection, with the potential of accelerating the hepatocyte proliferation caused by biliary obstruction itself, might be expected to develop portal hypertension earlier than patients with biliary obstruction alone. To examine this concept we studied 10 patients with postcholecystectomy bile duct stricture, portal hypertension and liver atrophy, or hepatic resection (group I) and compared them with nine patients with postcholecystectomy stricture and portal hypertension, but no atrophy or resection (group II). Portal hypertension was diagnosed a mean 28 months (range 18-48 months) after cholecystectomy in group I compared with 62 months (range 36-100 months) for patients in group II (p less than 0.005 Mann-Whitney test). Thus hepatocyte hyperplasia may be an important part of the mechanism underlying the development of portal hypertension in chronic biliary disease.
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PMID:Role of liver atrophy, hepatic resection and hepatocyte hyperplasia in the development of portal hypertension in biliary disease. 366 54

Acquired dysfibrinogenemia was documented in a 4-year-old child with obstructive jaundice of 1-month duration, secondary to a choledochal cyst involving the distal common bile duct. It was characterized by decreased thrombin coagulable protein with elevated immunoassayable fibrinogen resulting in abnormal thrombin and reptilase times. The liver morphology was compatible with extrahepatic obstruction, without evidence of cirrhosis or hepatocyte abnormality. All the coagulation abnormalities promptly resolved after surgical correction of the obstruction. Dysfibrinogenemia has been associated with serious liver disease in adults, including tumors, chronic active hepatitis, and cirrhosis, but never with isolated obstructive jaundice. This report documents a case of acquired dysfibrinogenemia due to extra-hepatic biliary obstruction and also emphasizes the importance of the consideration of this disorder in coagulation abnormalities associated with hepatobiliary disease.
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PMID:Dysfibrinogenemia in obstructive liver disease. 368 83

Plasma lipoproteins from two female patients--patient A, 4 wk and patient B, 19 months--were examined prior to and at 1 and 5 wk after surgical correction of biliary obstruction due to choledochal cyst. The findings were correlated with standard indices of hepatic function, namely SGPT, GGTP, 5'nucleotidase, serum bile salts, and total and conjugated bilirubin. Prior to surgery in both patients plasma cholesterol, phospholipid, and triglyceride were elevated; cholesterol esters were low; high-density lipoprotein (HDL) cholesterol and apolipoprotein A-I, the major protein constituent of HDL, were subnormal; most of the plasma lipids were contained in the low-density lipoprotein density region; lipoprotein-X was present. Patient B had had a relatively brief obstruction and suffered little secondary hepatic injury. One week after surgery, plasma lipid concentrations returned to normal; apolipoprotein A-I increased in the HDL density region and a concomitant rise in cholesterol esters to near normal, 65%, was observed; plasma lipids were contained predominantly in HDL; hepatic function improved markedly. Patient A had had intrauterine obstruction and suffered major hepatic injury with cirrhosis. One week after surgery, plasma lipid concentrations, cholesterol esters, low-density lipoprotein lipid predominance, and hepatic function remained essentially unchanged. Five weeks after surgery, the lipoprotein levels and composition and hepatic function were near normal. In conclusion, children with biliary obstruction have lipoprotein abnormalities similar to those seen in adult patients. These alterations are rapidly reversible with surgical relief and may be used as prognostic indicators of outcome.
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PMID:Correctable plasma lipoprotein abnormalities in infants with choledochal cysts. 392 Jun 38

In order to investigate the effect of extra-hepatic biliary obstruction on bilirubin transport, bile flow was re-established after 5, 10, 15 and 28 days when the mean (+/- SEM) plasma bilirubin concentrations of the four groups of five rats were 226 +/- 17.9, 201 +/- 22.4, 178 +/- 8.1 and 145 +/- 3.8 mumol/l, respectively. After 12 h of bile drainage in the conscious animal, the mean plasma bilirubin concentrations of the four groups had decreased by 88, 86, 73 and 46%, respectively. The bile flow rate increased with the duration of the obstruction and was maximal in the 28 day obstructed rats, although the bilirubin output in these animals was significantly less than in the other groups. Histological evidence of biliary obstruction was apparent in the livers of the 5 and 10 day obstructed animals. After 15 days of obstruction cirrhosis developed. In conclusion, the disposal of endogenous bilirubin in the bile duct obstructed rat appears to be influenced by the development of cirrhosis.
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PMID:Effect of prolonged bile duct obstruction in the rat on hepatic transport of bilirubin. 397 63

Two siblings with intrahepatic cholestatic cirrhosis and their brother, who had a potentially related disease at the time of accidental death, are presented. The onset of disease occurred during adolescence in all 3 cases. The initial sign was mild jaundice or portal hypertension. There was no abnormality in the countenance, cardiovascular system, or vertebral column. Except for the brother who died from an accident, jaundice gradually increased. Death followed due to cirrhosis. Liver biopsy specimens of these 2 patients showed diminution of interlobular bile ducts with no significant cholangitis. At autopsy, the livers of the 2 patients showed biliary cirrhosis without extrahepatic biliary obstruction. In both cases there was an accessory lobe on the right hepatic lobe. Histologically, septal bile ducts showed pronounced papillary proliferations of the epithelium; there was also a decrease in the number of small interlobular bile ducts. Excess copper accumulation in the liver was ascertained. It is suggested that the disease in the 2 autopsied cases is intrahepatic cholestatic cirrhosis due to hypoplasia of the intrahepatic biliary trees.
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PMID:Familial intrahepatic cholestatic cirrhosis in young adults. 400 5

Sixty cases of choledochal cysts in children are reviewed. The series comprises 55 cases of Alonso-Lej. Type 1 cysts, two cases whose cysts were infraduodenal and retropancreatic in position, and three cases of choledochal cysts with both proximal and distal atresia of bile ducts. The following five types of operative procedures were employed for the 58 cases: (1) Choledochocystoduodenostomy in 12; (2) Partial excision, choledochorraphy and choledocho-duodenostomy in six; (3) Cyst excision, portal dissection and portojejunostomy in three; (4) Choledochocystojejunostomy Roux-en-Y in 20; and (5) Cyst excision with hepaticojejunostomy Roux-en-Y in 17 cases. Two patients did not receive definitive surgical treatment. Early complications included six cases of leakage, of which one died; and four cases of cholangitis and septicemia, resulting in fatality in all. The overall operative mortality was 5/58 (8.6%). This review, though retrospective in nature, further supports the more recent trend that radical cyst excision with hepaticojejunostomy is the treatment of choice. This procedure carried no mortality and low morbidity. All 17 patients treated in this manner are well and free from jaundice and recurrent cholangitis. Cyst excision eliminates the reservoir for bile stasis, biliary obstruction, cholangitis, and biliary cirrhosis. It also removes the possibility of malignant change in the cyst and spontaneous rupture.
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PMID:Surgical management of choledochal cysts: a review of 60 cases. 404 73

Biliary obstruction is an important complication of chronic calcifying pancreatitis. In this study, liver biopsies were examined to determine the nature and severity of hepatic complications in 23 such cases. The most striking changes were portal tract expansion due to oedema and fibrosis, with proliferation of bile ducts. Although common, these changes were not severe, and no patient had developed secondary biliary cirrhosis. Other features of note were intrahepatic cholestasis, iron overload (56.5%), copper-associated protein stained with the orcein technique (34.7%) and mild fatty change or perivenular sclerosis in 13%. It is concluded that no serious, irreversible pathological changes occurred in the liver despite clinically marked biliary obstruction.
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PMID:The liver in biliary obstruction due to chronic pancreatitis. 405 70

The presence of large amounts of copper in the liver in primary biliary cirrhosis has been confirmed; a similar increase is not found in cases of long-standing extrahepatic biliary obstruction. The serum caeruloplasmin levels are raised in primary and secondary biliary cirrhosis, but this increase does not appear to be related to the degree of increase of hepatic copper. The manganese content of the liver is slightly raised in both these conditions.The reason for the increased metal content in these circumstances cannot yet be determined, and the effects are not yet understood.
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PMID:Copper and manganese concentrations in biliary cirrhosis of liver. 566 88


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