Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with Caroli's disease are reported who did not have renal abnormalities or hepatic fibrosis. Extra hepatic complications of Caroli's disease in these two patients were best evaluated by computed tomography (CT). Although ultrasound evaluates the ductal abnormalities of Caroli's disease well, it does not delineate subphrenic or subhepatic abscesses, pancreatitis, pancreatic phlegmon, or pseudocysts as well CT.
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PMID:The role of computed tomography in the evaluation of Caroli's disease. 274 96

Obstructive biliary disease in childhood is not common, but should be considered in the differential diagnosis of a child with jaundice, abdominal pain, or an abdominal mass. We have reviewed the experience at Vanderbilt Children's Hospital from 1970 to 1985, during which 87 children 18 years of age or younger with biliary tract disease were seen. Twelve patients (14%) had congenital disorders including choledochal stenosis, Caroli's disease, choledochal cyst, teratoma of the common hepatic duct and common bile duct, congenital septate biliary tree, and isolated atresia of the distal common bile duct. Thirty patients had neonatal cholestatic syndromes, and 37 had calculous disease of the gallbladder or extrahepatic bile ducts. Three patients had obstruction of the common bile duct caused by fibrosing pancreatitis. Two had sclerosing cholangitis. Obstruction of the common bile duct was caused by metastatic neoplasm in three patients. We discuss principles of diagnosis and management.
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PMID:Spectrum of biliary disease in childhood. 377 60

Using case reports, attention is drawn to rare causes of cholestasis the clarification of which by ERC/PTC and by endoscopic therapy, if necessary, will clearly shown the usefulness of this method. The picture of an unusual choledocholith, the extraction of a "suture stone", the case of a Mirizzi syndrome, a cholecystocolic fistula, a cystic dilatation of the choledochus with Caroli's syndrome and status after choledochojejunostomy, the extraction of an ascarid from the papilla at biliary pancreatitis as well as the detection of a biliobronchial fistula resulting from an unsound hepaticojejunostomy are examples to underline the diagnostic usefulness of the ERC/PCT including its therapeutic capabilities and consequences in contrast to other methods, such as x-ray, ultrasound or computertomography.
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PMID:[Rare causes of cholestasis in ERC/PTC]. 748 14

We report the case of a 31-year-old male patient with a diffuse form of Caroli's disease presenting as recurring bouts of biliary pancreatitis. Following sphincterotomy, the patient remained asymptomatic for 5 years. He then developed acute cholangitis and, at laparotomy, all superficial liver cysts were fenestrated in order to remove intrahepatic bile duct calculi. A right hepatectomy, removing the most severely affected liver parenchyma was not considered feasible because of the small size of the left lobe and the existence of an associated congenital hepatic fibrosis. In diffuse forms of Caroli's disease biliary drainage techniques have often proved ineffective in preventing recurring bouts of cholangitis. Moreover, liver resection is seldom feasible because of associated congenital hepatic fibrosis. In this setting, liver transplantation may represent the only effective and durable form of treatment.
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PMID:Caroli's disease: a surgical dilemma. 826 56

The goal of this study was to determine the prevalence, epidemiology and clinical-therapeutical evolution of hepatolithiasis (HL) in Argentina. With this purpose a survey was conducted sending a questionnaire to ten referencial and interventional radiology centers in the country. Seven centers answered on time. In the last five years a total of 8,736 consecutive patients were examined for cholangiography (endoscopic retrograde cholangiography, PTC). A total of 5,920 (68%) were biliary lithiasis and 53 (0.9%, range 0.5-2.6%) of these were HL. In case of HL the diagnostic procedure was the ERCP in 68% of the cases, and the PTC in the remainder 32%. The patients with HL (53% females, mean age 52, range 23-85) clinically presented cholangitis (79%); pancreatitis (6%) and five (9.4%) showed evolution to a biliary cirrhosis. Associated diseases or abnormalities of the biliary tree were: biliary postsurgical strictures (BPS), 28%; Caroli's Syndrome, 20%; and choledocholithiasis, 28%. While a 9.4% presented a "biliary history" (that was defined as two or more episodes of biliary surgery) and a 5.7% lacked associated or predisposing diseases. Follow-up was lost in 23% of the cases and in 77% a follow up of 38 months (range 8-60) was observed with 4.8% mortality rate. The treatment was hepatobiliary surgery in 58% of the cases; endoscopic papillotomy in 17% and combined treatments that included extracorporeal shock wave lithotripsy and ursodeoxycholic acid (UDCA) in 15%. Four out of 53 cases (7.5%) received UDCA as the only successful therapy. HL is an entity with high biliary morbidity in 85% of the cases and development in to cirrhosis in 9.4%. When the diagnosis is made in the western world both BPS and Caroli must be discarded first. Combined treatments or only UDCA are new therapeutical alternative in the western world.
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PMID:[Hepatolithiasis and Caroli's disease in Argentina: results of a multicenter study]. 1043 88

Cystic diseases of the bile ducts are rare, but relatively more prevalent in females, and more common in Japan and Asia. Most are diagnosed in children under 10 years of age, with varying patterns of symptoms including right upper quadrant pain, jaundice, and fever. Up to 20% of bile duct cysts are diagnosed in adults, including during pregnancy, in whom the diagnosis can be confounded by associated cholelithiasis, or by abnormalities of the pancreatic junction with pancreatitis. The risk of malignant transformation increases with age, and is more common in cysts of Alonso-Lej Types I, IV, and V (Caroli's Disease). Intracystic lithiasis is frequently associated with tumour, and can give similar radiological appearances. Tumours are often first diagnosed at laparotomy, and can already be unresectable. More than half the tumours are intracystic, but malignant change in cyst mucosa without a tumour mass is often not recognised at surgery. In addition, malignant change in bile duct epithelium can occur after cyst excision, sometimes after many years, and in areas of the biliary tree remote from the cyst, including the gall-bladder. Cyst excision should be attempted at all ages, and patients closely monitored thereafter. Malignancy should be suspected in all adults with bile duct cysts. Hepatectomy, partial or total with transplantation, is the treatment of choice in Caroli's disease.
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PMID:Choledochal cystic malignancies. 1043 95

Caroli's disease is a rare condition characterized by congenital polycystic dilatation of the intrahepatic bile ducts. The most frequent clinical presentation of a simple type (Caroli's disease) is recurrent cholangitis, gallstone with pain, obstructive jaundice and episodes of pancreatitis in childhood and early adulthood. A more frequent type combined with congenital hepatic fibrosis is usually manifested with bleeding from esophageal varices consequential to portal hypertension. Treatment options, both conservative and surgical, are relatively limited and depend on the clinical presentation, localization of cysts in the liver, and stage of the disease. A 20-year-old man with Caroli's disease manifested with cholelithiasis and choledocholithiasis with recurrent pancreatitis at the age of 16 is presented. The diagnosis was confirmed by endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography. Treatment with ursodeoxycholic acid was initiated and after two years of follow-up the disease is stable.
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PMID:[Caroli's disease]. 1458 72

''Pure'' Caroli's disease is rarely observed, usually presenting with symptoms of cholangitis: cases of pancreatitis seem most infrequent in the literature. A case of recurrent pancreatitis in Caroli's disease associated with renal policystic disease is presented: the rareness of this manifestation is stressed and the possible pathogenetic hypotheses are discussed, as well as indications for the selected treatment by endoscopic sphincterotomy.
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PMID:[Recurrent pancreatitis in Caroli's disease. Treatment by endoscopic sphincterotomy]. 1649 81

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

Splenic artery aneurysm is a rare disorder (0.7%) that arises mainly as a sequlae to portal hypertension. Other causes of splenic artery aneurysm are atherosclerosis, arterial wall injury due to trauma, pancreatitis, and medial dysplasias of the wall. However, though Caroli's disease is known to cause portal hypertension, the rise of vascular pressure leading to aneurysm is not yet reported (extensive Medlar search failed to reveal any publication). Every effort should be made to diagnose this condition as early as possible because 25% of ruptured splenic aneurysms are fatal. A unique case of Caroli's disease giving rise to splenic artery aneurysm and its possible pathogenesis is reported.
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PMID:Caroli's syndrome leading to splenic artery aneurysm: a rare presentation. 1849 92


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