Bile Ducts, Intrahepatic: Passages within the liver for the conveyance of bile. Includes right and left hepatic ducts even though these may join outside the liver to form the common hepatic duct.Caroli Disease: Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.Bile Ducts: The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.Bile Duct Diseases: Diseases in any part of the ductal system of the BILIARY TRACT from the smallest BILE CANALICULI to the largest COMMON BILE DUCT.Bile Ducts, Extrahepatic: Passages external to the liver for the conveyance of bile. These include the COMMON BILE DUCT and the common hepatic duct (HEPATIC DUCT, COMMON).Bile Duct Neoplasms: Tumors or cancer of the BILE DUCTS.Cholestasis, Intrahepatic: Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC).Common Bile Duct: The largest bile duct. It is formed by the junction of the CYSTIC DUCT and the COMMON HEPATIC DUCT.Bile: An emulsifying agent produced in the LIVER and secreted into the DUODENUM. Its composition includes BILE ACIDS AND SALTS; CHOLESTEROL; and ELECTROLYTES. It aids DIGESTION of fats in the duodenum.Adenoma, Bile Duct: A benign tumor of the intrahepatic bile ducts.Muridae: A family of the order Rodentia containing 250 genera including the two genera Mus (MICE) and Rattus (RATS), from which the laboratory inbred strains are developed. The fifteen subfamilies are SIGMODONTINAE (New World mice and rats), CRICETINAE, Spalacinae, Myospalacinae, Lophiomyinae, ARVICOLINAE, Platacanthomyinae, Nesomyinae, Otomyinae, Rhizomyinae, GERBILLINAE, Dendromurinae, Cricetomyinae, MURINAE (Old World mice and rats), and Hydromyinae.Biliary Tract: The BILE DUCTS and the GALLBLADDER.Cholangiography: An imaging test of the BILIARY TRACT in which a contrast dye (RADIOPAQUE MEDIA) is injected into the BILE DUCT and x-ray pictures are taken.Clonorchiasis: Infection of the biliary passages with CLONORCHIS SINENSIS, also called Opisthorchis sinensis. It may lead to inflammation of the biliary tract, proliferation of biliary epithelium, progressive portal fibrosis, and sometimes bile duct carcinoma. Extension to the liver may lead to fatty changes and cirrhosis. (From Dorland, 27th ed)Cholestasis: Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).Cholangiocarcinoma: A malignant tumor arising from the epithelium of the BILE DUCTS.Liver Cirrhosis, Biliary: FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.Hepatic Duct, Common: Predominantly extrahepatic bile duct which is formed by the junction of the right and left hepatic ducts, which are predominantly intrahepatic, and, in turn, joins the cystic duct to form the common bile duct.Bile Acids and Salts: Steroid acids and salts. The primary bile acids are derived from cholesterol in the liver and usually conjugated with glycine or taurine. The secondary bile acids are further modified by bacteria in the intestine. They play an important role in the digestion and absorption of fat. They have also been used pharmacologically, especially in the treatment of gallstones.Cholangitis: Inflammation of the biliary ductal system (BILE DUCTS); intrahepatic, extrahepatic, or both.Cholangiopancreatography, Endoscopic Retrograde: Fiberoptic endoscopy designed for duodenal observation and cannulation of VATER'S AMPULLA, in order to visualize the pancreatic and biliary duct system by retrograde injection of contrast media. Endoscopic (Vater) papillotomy (SPHINCTEROTOMY, ENDOSCOPIC) may be performed during this procedure.Biliary Tract Neoplasms: Tumors or cancer in the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Biliary Atresia: Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Cholagogues and Choleretics: Gastrointestinal agents that stimulate the flow of bile into the duodenum (cholagogues) or stimulate the production of bile by the liver (choleretic).Biliary Tract Diseases: Diseases in any part of the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Alagille Syndrome: A multisystem disorder that is characterized by aplasia of intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC), and malformations in the cardiovascular system, the eyes, the vertebral column, and the facies. Major clinical features include JAUNDICE, and congenital heart disease with peripheral PULMONARY STENOSIS. Alagille syndrome may result from heterogeneous gene mutations, including mutations in JAG1 on CHROMOSOME 20 (Type 1) and NOTCH2 on CHROMOSOME 1 (Type 2).Clonorchis sinensis: A species of trematode flukes of the family Opisthorchidae. Many authorities consider this genus belonging to Opisthorchis. It is common in China and other Asiatic countries. Snails and fish are the intermediate hosts.Cysts: Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Gallbladder: A storage reservoir for BILE secretion. Gallbladder allows the delivery of bile acids at a high concentration and in a controlled manner, via the CYSTIC DUCT to the DUODENUM, for degradation of dietary lipid.Dilatation, Pathologic: The condition of an anatomical structure's being dilated beyond normal dimensions.1-Naphthylisothiocyanate: A tool for the study of liver damage which causes bile stasis and hyperbilirubinemia acutely and bile duct hyperplasia and biliary cirrhosis chronically, with changes in hepatocyte function. It may cause skin and kidney damage.Choledochal Cyst: A congenital anatomic malformation of a bile duct, including cystic dilatation of the extrahepatic bile duct or the large intrahepatic bile duct. Classification is based on the site and type of dilatation. Type I is most common.Cholangitis, Sclerosing: Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.Cholelithiasis: Presence or formation of GALLSTONES in the BILIARY TRACT, usually in the gallbladder (CHOLECYSTOLITHIASIS) or the common bile duct (CHOLEDOCHOLITHIASIS).Liver Diseases: Pathological processes of the LIVER.Gallbladder Neoplasms: Tumors or cancer of the gallbladder.ChymotrypsinogenSecretin: A peptide hormone of about 27 amino acids from the duodenal mucosa that activates pancreatic secretion and lowers the blood sugar level. (USAN and the USP Dictionary of Drug Names, 1994, p597)Hepatectomy: Excision of all or part of the liver. (Dorland, 28th ed)Common Bile Duct Diseases: Diseases of the COMMON BILE DUCT including the AMPULLA OF VATER and the SPHINCTER OF ODDI.Portal Vein: A short thick vein formed by union of the superior mesenteric vein and the splenic vein.Liver Neoplasms: Tumors or cancer of the LIVER.Drainage: The removal of fluids or discharges from the body, such as from a wound, sore, or cavity.Portasystemic Shunt, Transjugular Intrahepatic: A type of surgical portasystemic shunt to reduce portal hypertension with associated complications of esophageal varices and ascites. It is performed percutaneously through the jugular vein and involves the creation of an intrahepatic shunt between the hepatic vein and portal vein. The channel is maintained by a metallic stent. The procedure can be performed in patients who have failed sclerotherapy and is an additional option to the surgical techniques of portocaval, mesocaval, and splenorenal shunts. It takes one to three hours to perform. (JAMA 1995;273(23):1824-30)Gallstones: Solid crystalline precipitates in the BILIARY TRACT, usually formed in the GALLBLADDER, resulting in the condition of CHOLELITHIASIS. Gallstones, derived from the BILE, consist mainly of calcium, cholesterol, or bilirubin.Pancreatic Ducts: Ducts that collect PANCREATIC JUICE from the PANCREAS and supply it to the DUODENUM.Liver Transplantation: The transference of a part of or an entire liver from one human or animal to another.Cholestasis, Extrahepatic: Impairment of bile flow in the large BILE DUCTS by mechanical obstruction or stricture due to benign or malignant processes.Common Bile Duct Neoplasms: Tumor or cancer of the COMMON BILE DUCT including the AMPULLA OF VATER and the SPHINCTER OF ODDI.Hepatocytes: The main structural component of the LIVER. They are specialized EPITHELIAL CELLS that are organized into interconnected plates called lobules.Centromere Protein B: A DNA-binding protein that interacts with a 17-base pair sequence known as the CENP-B box motif. The protein is localized constitutively to the CENTROMERE and plays an important role in its maintenance.Chimera: An individual that contains cell populations derived from different zygotes.Epithelium: One or more layers of EPITHELIAL CELLS, supported by the basal lamina, which covers the inner or outer surfaces of the body.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Cystic Duct: The duct that is connected to the GALLBLADDER and allows the emptying of bile into the COMMON BILE DUCT.Tomography, X-Ray Computed: Tomography using x-ray transmission and a computer algorithm to reconstruct the image.Epithelial Cells: Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.Rats, Inbred F344Biliary Tract Surgical Procedures: Any surgical procedure performed on the biliary tract.Jaundice, Obstructive: Jaundice, the condition with yellowish staining of the skin and mucous membranes, that is due to impaired BILE flow in the BILIARY TRACT, such as INTRAHEPATIC CHOLESTASIS, or EXTRAHEPATIC CHOLESTASIS.Thoracic Duct: The largest lymphatic vessel that passes through the chest and drains into the SUBCLAVIAN VEIN.Ligation: Application of a ligature to tie a vessel or strangulate a part.Cholecystectomy, Laparoscopic: Excision of the gallbladder through an abdominal incision using a laparoscope.Cholecystectomy: Surgical removal of the GALLBLADDER.Treatment Outcome: Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.DNA, Satellite: Highly repetitive DNA sequences found in HETEROCHROMATIN, mainly near centromeres. They are composed of simple sequences (very short) (see MINISATELLITE REPEATS) repeated in tandem many times to form large blocks of sequence. Additionally, following the accumulation of mutations, these blocks of repeats have been repeated in tandem themselves. The degree of repetition is on the order of 1000 to 10 million at each locus. Loci are few, usually one or two per chromosome. They were called satellites since in density gradients, they often sediment as distinct, satellite bands separate from the bulk of genomic DNA owing to a distinct BASE COMPOSITION.Choledocholithiasis: Presence or formation of GALLSTONES in the COMMON BILE DUCT.Sphincterotomy, Endoscopic: Incision of Oddi's sphincter or Vater's ampulla performed by inserting a sphincterotome through an endoscope (DUODENOSCOPE) often following retrograde cholangiography (CHOLANGIOPANCREATOGRAPHY, ENDOSCOPIC RETROGRADE). Endoscopic treatment by sphincterotomy is the preferred method of treatment for patients with retained or recurrent bile duct stones post-cholecystectomy, and for poor-surgical-risk patients that have the gallbladder still present.Bile Canaliculi: Minute intercellular channels that occur between liver cells and carry bile towards interlobar bile ducts. Also called bile capillaries.Cholangiopancreatography, Magnetic Resonance: Non-invasive diagnostic technique for visualizing the PANCREATIC DUCTS and BILE DUCTS without the use of injected CONTRAST MEDIA or x-ray. MRI scans provide excellent sensitivity for duct dilatation, biliary stricture, and intraductal abnormalities.Salivary Ducts: Any of the ducts which transport saliva. Salivary ducts include the parotid duct, the major and minor sublingual ducts, and the submandibular duct.Hepatic Artery: A branch of the celiac artery that distributes to the stomach, pancreas, duodenum, liver, gallbladder, and greater omentum.Species Specificity: The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.Hypertension, Portal: Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN.Biliary Fistula: Abnormal passage in any organ of the biliary tract or between biliary organs and other organs.Lithiasis: A condition characterized by the formation of CALCULI and concretions in the hollow organs or ducts of the body. They occur most often in the gallbladder, kidney, and lower urinary tract.Ursodeoxycholic Acid: An epimer of chenodeoxycholic acid. It is a mammalian bile acid found first in the bear and is apparently either a precursor or a product of chenodeoxycholate. Its administration changes the composition of bile and may dissolve gallstones. It is used as a cholagogue and choleretic.Bile Pigments: Linear TETRAPYRROLES that give a characteristic color to BILE including: BILIRUBIN; BILIVERDIN; and bilicyanin.Ampulla of Vater: A dilation of the duodenal papilla that is the opening of the juncture of the COMMON BILE DUCT and the MAIN PANCREATIC DUCT, also known as the hepatopancreatic ampulla.Hybridization, Genetic: The genetic process of crossbreeding between genetically dissimilar parents to produce a hybrid.Jaundice: A clinical manifestation of HYPERBILIRUBINEMIA, characterized by the yellowish staining of the SKIN; MUCOUS MEMBRANE; and SCLERA. Clinical jaundice usually is a sign of LIVER dysfunction.Liver Cirrhosis: Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.
  • Colonoscopic biopsies showed chronic inflammatory changes of the caecum and the ascending and transverse colon compatible with Crohn disease. (biomedcentral.com)
  • Accumulating bile acids from cholestasis lead to reduced pH, increased apoptosis and activation of ERK1/2, Akt and NF-κB pathways that encourage cell proliferation, migration and survival. (biomedcentral.com)
  • Disorders of the biliary system usually give rise to the symptoms and signs of biliary obstruction (cholestasis), including pain (ranging from 'dyspepsia' to severe right hypochondrial colic), jaundice, itching, nausea and vomiting (which may be prominent in sudden obstruction of the bile duct, usually by a gallstone), fevers, and rigors (indicating bacterial infection of the biliary tract, which frequently accompanies partial obstruction). (enetmd.com)
  • A number of factors contribute to the development of the disease, such as cholestasis, infection, and anatomic abnormity of bile duct and bile metabolic defect. (hindawi.com)
  • Although the pathogenesis of intrahepatic stones has not been disclosed clear so far, it is still reported to be related with the environment, nutritional status, bile duct infection, cholestasis, parasites, the variation of bile duct, bile metabolic defect, and so forth. (hindawi.com)
  • Among these factors, cholestasis, infection, and anatomic abnormity of bile duct and bile metabolic defect occupy the most important positions. (hindawi.com)
  • Any factor which affects bile production and excretion can cause cholestasis. (hindawi.com)
  • we describe a case of pure form Caroli' s disease involving both hepatic lobes, in which both US (detection of fibrovascular bundles inside the cystic lesions) and MRCP (demonstration of the communication of the multiple cyst with biliary tree) allowed a noninvasive diagnosis. (eurorad.org)
  • Typically, these disconnected biliary structures are present in a very early disease stage, but remain asymptomatic until cyst growth initiates in adulthood [ 4 ]. (biomedcentral.com)
  • Technetium 99m Tc hepatobiliary iminodiacetic acid (HIDA) scan is often used and is particularly useful for showing continuity with bile ducts and diagnosis of cyst rupture. (51digg.info)
  • Bile duct cyst type V (Caroli's disease): surgical strategy and results. (omjournal.org)
  • Contrast-enhanced CT or gadolinium-enhanced MR imaging shows the central dot sign corresponds to a portal vein branch protruding into the lumen of a dilated bile duct or a linear hyperattenuating or high-signal-intensity focus containing a portal vein and bridging the cyst wall in carolis disease. (barnard.in)
  • What is a bile duct cyst? (healthhype.com)
  • Eventually, usually by age two or three, but sometimes not until adolescence or adulthood, bile accumulates in the duct resulting in damage to the duct and formation of a fluid-filled sac or cyst which then prevents bile from reaching the intestine. (liver.ca)
  • Secretion of bile into the duodenum occurs between weeks 13 and 16. (mhmedical.com)
  • Although the length of this common channel varies from person to person, it is less than 3 mm in most people, and in some, the two ducts do not converge but drain into the duodenum through two adjacent but separate orifices. (clinicalgate.com)
  • A remnant of the dorsal bud duct persists as the accessory duct of Santorini in approximately 40% of people, with its opening in the minor papilla of the duodenum ( Fig. 36.1 ). (clinicalgate.com)