Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Caroli's disease (congenital dilatation of the intrahepatic bile ducts) was diagnosed in an 11-year-old girl with the aid of intravenous cholangiography. Recurrent abdominal pain, fever, and intermittent obstructive jaundice are the most common symptoms and usually develop during childhood or young adulthood. Operative cholangiography has been the most valuable method of diagnosing the condition and determining the location and extent of the disease process.
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PMID:Congenital dilatation of the intrahepatic bile ducts (Caroli's disease). 66 69

Three sisters with cystic dilatation of the intrahepatic bile ducts (Caroli's disease) are reported. The index case, a 41-year-old woman with remittent high fever and right upper quadrant abdominal pain, was diagnosed as Caroli's disease with hepatic lithiasis and cholangitis based on findings of ultrasonography, computed tomography and endoscopic retrograde cholangiography. Her two older sisters were also examined and found to have the same disease without clinical symptoms. Their symptoms, locations of the dilated ducts and complications all varied. The hereditary mode of Caroli's disease in 13 families (32 cases) reported in the world literature including our study was examined. While Caroli's disease is thought to be an autosomal recessive disease, a conclusion on the hereditary mode of transmission could not be made in this study because of an insufficient investigation of family members, especially the parents.
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PMID:Caroli's disease in three siblings. 146 9

Eight patients with Caroli's Disease are presented, studied by Endoscopic Retrograde Cholangiopancreatography (ERCP) from January 1976 through January 1990. In this period of time 1,525 procedures were carried out, this entity thus representing 0.52% of patients submitted to ERCP in our population. Six patients were females, being female: male ratio 3:1. Mean age was 52 years (range: 40-75). All patients presented a clinical history of recurring episodes of abdominal pain and/or crisis of cholangitis. In the ERCP carried out in these eight patients, cystic dilatation of intrahepatic left lobe bile ducts were confirmed in five patients, dilatation generalized to both lobes in two, and affecting exclusively the right lobe in one patient.
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PMID:[Caroli's disease. Presentation of 8 cases studied with ERCP]. 193 Dec 43

A 27-year-old woman was admitted to Nagasaki University Hospital complaining of abdominal pain and fever. Percutaneous transhepatic cholangiography showed multiple cystic dilatations of the intrahepatic bile ducts without stone formation. The illness was diagnosed as a pure type of Caroli's disease. No surgical intervention was made. Subsequently, the patient suffered repeated attacks of cholangitis, and six years later, computed tomography demonstrated cystic lesions of both lobes of the liver and stones within the cystic lesions of the right lobe. We collected 62 cases of Caroli's disease reported in the Japanese literature, including our own case. Among them, cholangitis was found in 43.5%, hepatolithiasis in 30.6%, esophageal varices in 17.7% and cholangiocarcinoma in 8.1%. It is difficult to successfully treat Caroli's disease involving both lobes of the liver. Long-term repeated cholangitis could lead to fibrosis of the liver, hepatolithiasis, portal hypertension and cholangiocarcinoma. Considering the above-mentioned aspects of the disease, careful follow-up studies are required in each case.
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PMID:Caroli's disease associated with hepatolithiasis: a case report and review of the Japanese literature. 200 60

A 27 year old woman who presented with upper abdominal pain was found on ultrasonography to have multiple liver cysts consistent with hydatid disease. Three years previously she had undergone evacuation of multiple infected liver cysts thought to be due to hydatid disease. Computed tomographic scanning supported the diagnosis of hydatid disease affecting the right lobe of the liver. At laparotomy the right lobe contained multiple cysts which were removed by right lobectomy. Histology revealed congenital dilatation of the intrahepatic bile ducts with fibrosis (Caroli's disease) but no evidence of hydatid disease.
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PMID:Caroli's disease misdiagnosed as hydatid liver cysts. 205 31

We report our experience with nine adult patients (six females, three males) with Caroli's syndrome. Most of these patients were young (mean age 27.0 yr), and had presented at a mean interval of 12.1 yr after the onset of symptoms. Recurrent abdominal pain was the most common symptom, present in eight of nine patients. Mild jaundice (serum bilirubin 1.8-4.2 mg/dl) and raised serum alkaline phosphatase (18-36 King-Armstrong units) were the only other significant findings. Ultrasound proved to be a good screening test. However, the diagnosis was confirmed on cholangiography. Six of nine patients had associated extrahepatic biliary cysts (type IVa). The operative management was directed mainly at excising the extrahepatic cysts and draining the biliary tree, and was met with satisfactory results.
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PMID:Caroli's syndrome: a heterogeneous entity. 230 39

Interposition of the gallbladder is a rare anomaly, but its diagnosis is important since it represents a surgically correctable cause of jaundice. The patients present with jaundice, abdominal pain and sometimes an enlarged gallbladder. Radiological diagnosis may be difficult since the condition may be mistaken for a choledochal cyst, hydrops of the gallbladder or Caroli's disease. The ultrasound, cholangiogram and surgical findings of dilated intrahepatic ducts adjacent to a normal or enlarged gallbladder with no dilatation of the common bile duct are presented in two children with this condition.
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PMID:Interposition of the gallbladder--or the absent common hepatic duct and cystic duct. 355 Jun 67

Congenital cystic dilatation of the intrahepatic biliary ducts, known as Caroli's disease, is rarely recognized in general surgical practice and often overlooked. Until now little more than a hundred cases have been described although modern diagnostic procedures disclose more frequently new ones. Three patients with this anomaly have been treated by the authors during the past years and the different aspects concerning diagnosis and treatment are discussed and compared with existing information. In all three patients colic pain in the upper abdomen was the first symptom reported in the first years of life. In one patient jaundice and fever were associated with the abdominal pain and cholangitis developed a secondary biliary cirrhosis. Somatic underdevelopment, consequence of the cirrhosis in this child was observed. In two patients an intrahepatic hepaticojejunostomy was performed and a left hepatectomy in the third. In the two older children intrahepatic gallstones were found; in the younger one no stones were found inside the dilated ducts.
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PMID:[Cystic dilatation of the intrahepatic bile ducts (Caroli's disease)]. 356 75

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

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


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