Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient operated upon for acalculous cholecystitis was later found to have Caroli's disease, congenital ectasis of intrahepatic bile ducts. Cholangitis, calculi and abscesses occurred in both lobes of the liver. Sepsis was not controlled despite open drainage, hepatic segmentectomy and ductal lavage with antibiotics and saline. At autopsy the liver contained a dozen unsuspected cavities filled with calculi, bile and pus. Diagnosis of Caroli's disease is best made by operative cholangiography in patients with atypical biliary disease. Management with antibiotics alone is seldom successful. Hepatic resection is better than drainage procedures for unilateral lesions. In patients with bilateral abscesses and no extrahepatic ductal obstruction or dilation, surgical treatment is often ineffective.
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PMID:Caroli's: a surgical challenge. 42 75

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

Major hepatic resections were performed on 138 patients for a variety of conditions. There was one intraoperative death. Including this patient, there were 15 deaths within 30 days of the operation (operative mortality 10.9%). Important postoperative complications were intra-abdominal sepsis (17%), biliary leak (11%), hepatic failure (8%), and hemorrhage (6%). The results of 30 resections for the benign lesions, liver cell adenoma, focal nodular hyperplasia, hemangioma, and cystadenoma showed no operative mortality and low morbidity. Of 26 patients with hepatocellular carcinoma, seven died within a month of operation. The cumulative survival of the 26 at five years was 38%, and of the 19 who survived the procedure, 51%. Poor survival followed resections for cholangiocarcinoma and "mixed tumors." The five-year cumulative survival of 22 patients who had colorectal metastases excised was 31%. Apart from a patient with carcinoid, prolonged survival was rare after resection of other secondaries and after en bloc resections for tumors directly invading the liver. Hepatic resection was of value in the management of some patients with hepatic trauma, Caroli's disease, liver cysts, and intrahepatic stones.
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PMID:Major hepatic resection. A 25-year experience. 629 17

Caroli's disease is a congenital disease of cystic or saccular dilatation of the intrahepatic bile ducts. There are two disease entities: a simple type and a periportal fibrosis type. Frequent complications with the simple type are recurrent cholangitis, liver abscess, intraductal lithiasis, abdominal pain, and fever that often lead to fatal sepsis. Development of portal hypertension and esophageal varices is usually a final feature of the periportal fibrosis type. Malignancies are also possible complications with Caroli's disease. During the recent 13 years, the author had experiences with eight patients with Caroli's disease of the simple type; six of these eight underwent hepatic resection: right lobectomy in two, left lobectomy in three, and left lateral segmentectomy in one. Other two patients died of sepsis and cholangiocellular carcinoma, respectively. All six patients with hepatic resections were relieved from the disabling symptoms after surgery and have had no recurrent hepatobiliary problems for 3 months to 13 years. Hepatic resection may be indicated for more patients than previously assumed in the treatment of Caroli's disease of the simple type.
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PMID:Successful treatment of Caroli's disease by hepatic resection. Report of six patients. 650 1

Two of three patients with infantile polycystic disease and all three patients with congenital hepatic fibrosis revealed multiple gross cystic dilatation of the intrahepatic biliary tree, referred to as Caroli's disease. All three patients with congenital hepatic fibrosis showed recurrent cholangitis related to coexisting Caroli's disease, and two of them died of sepsis following cholangitis. There were several common morphologic findings in the intrahepatic biliary tree of macroscopic and microscopic levels in infantile polycystic disease and congenital hepatic fibrosis with Caroli's disease: 1. irregular, non-obstructive dilatation of the duct lumen; 2. bulbar protrusion of the duct wall into the lumen; and 3. bridge formation of the duct wall across the lumen. These ductal changes might be caused by a combination of uneven and disproportionate overgrowth of biliary epithelia and their supporting connective tissue. This pathogenetic mechanism might be operative along the entire intrahepatic biliary system in this disease group, and involvement of the smaller levels in early life might result in infantile polycystic disease and congenital hepatic fibrosis and simultaneous or possibly later involvement of the larger levels in Caroli's disease.
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PMID:Caroli's disease in congenital hepatic fibrosis and infantile polycystic disease. 716 37

Six out of 1,612 patients operated on for biliary diseases between January 1974 and January 1985, had Caroli's disease. A summary of the clinical records is included. The treatment performed was: external drainage in a patient with sepsis, who did not improve and died; left hepatic lobectomy and biliary-jejunal diversion in two patients; right extended lobectomy in one patient; distal spleno-renal shunt in a patient with severe biliary cirrhosis and recurrent hemorrhage from esophageal varices. The last patient suffered from a combination of a choledochal cyst and dilatation of the left intrahepatic branches, and underwent complete resection of the choledochal cyst, and biliary jejunal anastomosis on a porta-hepatis using a Roux-en-Y jejunal loop. Currently, five patients are recovering and are free of symptoms. The authors discuss the indications for liver resection and resection of a choledochal cyst.
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PMID:Surgical treatment of congenital dilatation of the biliary system. 850 44

The extent of complement and contact activation is related to outcome in sepsis. A low functional index of their main blocker C1-esterase inhibitor (C1-INH) is considered as a relative deficiency of C1-INH and might contribute to the development of fatal complications in the intensive care unit. The first results of therapeutic intervention with C1-INH concentrate in septic shock are promising. We report on our experience of C1-INH concentrate administration in a young woman with Caroli's disease as ultimate rescue therapy for septic shock with capillary leakage syndrome after combined liver and kidney transplantation. No focus of infection was detectable and thus surgical intervention was not indicated. Antibiotic therapy at that time included vancomycin, tobramycin, meropenem and fluconazol. Hemodynamic stabilization occurred within hours after administration of C1-INH concentrate. Simultaneously a reduction in vasopressor medication was possible and negative fluid balance was achieved.
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PMID:Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor. 1050 63

Caroli's disease is a rare congenital hepatic disease, characterized by segmental dilatation of the biliary tree. Patients who have recurrent bouts of biliary infection, particularly those with complications related to portal hypertension, may require orthotopic liver transplantation (OLT). Few case reports have described the outcome of OLT in patients with Caroli's disease and to date there is no large series reported in the literature. We retrospectively analyzed the outcome of OLT in patients with Caroli's disease who underwent OLT between 1982 and 2002 at Starzl Transplantation Institute, University of Pittsburgh. Patients were identified and data was collected by computerized search of the electronic database system. All patients had confirmation of diagnosis by histopathology of explanted liver. A total of 33 patients with Caroli's disease were listed for liver transplantation, 3 of whom were excluded, as they were not transplanted. A total of 90% had signs of hepatic decompensation at the time of OLT. Median posttransplantation follow-up was 7.7 yr. Short-term graft and patient survival at 1 month was 83% and 86%, whereas overall long-term graft survival rates at 1, 5, and 10 yr were 73%, 62%, and 53%, respectively, and patient survival rates were 76%, 65%, and 56%, respectively. Long-term outcome in patients who survived the first year after transplantation was significantly better. Their survival rate at 5 and 10 yr was 90% and 78%. On univariable analysis, recipient age, donor male gender, coexistent congenital hepatic fibrosis, and re-OLT were associated with poor patient survival. Eight patients were retransplanted, 3 of whom had primary nonfunction. A total of 13 patients died; the most common cause of death being sepsis and cardiovascular complications. Patients who died of sepsis had cholangitis pre-OLT. In conclusion, OLT is a form of curative and life-saving therapy in patients with Caroli's disease, especially in those with decompensated liver disease. Overall survival is better with liver transplantation and is comparable with the survival of recipients who undergo OLT for other etiologies of chronic liver disease. Survival was poor in patients with congenital hepatic fibrosis (Caroli's syndrome) and in those who had cholangitis at the time OLT.
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PMID:Caroli's disease and orthotopic liver transplantation. 1649 55

In 1958 Caroli described a rare disease with multifocal, segmental and saccular dilation of the large intrahepatic bile ducts which causes stagnation of bile and formation of bile sludge and stones. This results in recurrent abdominal pain, cholangitis and hepatic abscesses. The diagnosis is confirmed with endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) and the purpose of treatment is to restore normal bile flow. Partial resection of the liver has given good results in patient with localized disease. The prognosis is poor despite drainage of bile and 46% of patients die from sepsis, hepatic abscesses, hepatic failure or portal hypertension. There is more than a hundred fold risk of cholangiocarcinoma. We report a case where a male who had a history of recurrent bouts of abdominal pain and pancreatitis was diagnosed with Caroli's disease. He later developed cholangiocarcinoma. Caroli's disease has not, to our knowledge, been reported in Iceland before.
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PMID:[Caroli's disease, case report and review of the literature]. 1782

Caroli's disease is a rare congenital disorder characterized by cystic dilatation of the large in-trahepatic bile ducts. The most frequent complications due to biliary stasis are cholelithiasis, cholangitis and sepsis as well as an increased risk of cholangiocarcinoma. Patients may have a history of intermittent abdominal pain, pruritus and/or symptoms of cholangitis. It is rarely diagnosed in childhood. A 12-year-old boy with isolated Caroli's disease is described. This child presented at the age of 2 years, with 4 episodes of recurrent bacterial infections. Interestingly he remained asymptomatic for over 10 years, between the second and third episode. During the 4th episode, when he presented with fever and slight abdominal pain, the diagnosis was made on the basis of radiological findings: U/S, CT, MRI and especially with MRCP, in relation with a more typical picture, resembling cholangitis. Since then he has been followed-up systematically for ten years and remains in good clinical condition without further relapses and with unchanged radiological findings. This atypically benign course of Caroli's disease, with intermittent asymptomatic periods, without any treatment, is very rare.
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PMID:Caroli's disease: Description of a case with a benign clinical course. 2471 23


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