Ductopenia

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Ductopenia refers to a reduction in the number of ducts in an organ, in particular the absence of bile ducts of the expected size in the portal tract of the liver. [1] It is the histological hallmark of vanishing bile duct syndrome (typically <0.5 bile ducts per portal triad). The most common cause of ductopenia is primary biliary cholangitis.

Other causes of ductopenia include failing liver transplant, Hodgkin's lymphoma, graft-versus-host disease (GVHD), sarcoid, cytomegalovirus infection, HIV, and medication toxicity, such as phenothiazines.

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Duodenum First section of the small intestine

The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear, and the terms anterior intestine or proximal intestine may be used instead of duodenum. In mammals the duodenum may be the principal site for iron absorption. The duodenum precedes the jejunum and ileum and is the shortest part of the small intestine.

Bile Dark greenish-brown fluid aiding in the digestion of fats

Bile, or gall, is a dark-green-to-yellowish-brown fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is produced continuously by the liver and stored and concentrated in the gallbladder. After eating, this stored bile is discharged into the duodenum.

Gallbladder Organ in humans and other vertebrates

In vertebrates, the gallbladder, also known as the cholecyst, is a small hollow organ where bile is stored and concentrated before it is released into the small intestine. In humans, the pear-shaped gallbladder lies beneath the liver, although the structure and position of the gallbladder can vary significantly among animal species. It receives and stores bile, produced by the liver, via the common hepatic duct, and releases it via the common bile duct into the duodenum, where the bile helps in the digestion of fats.

Bile duct Type of organ

A bile duct is any of a number of long tube-like structures that carry bile, and is present in most vertebrates.

Caroli disease Medical condition

Caroli disease is a rare inherited disorder characterized by cystic dilatation of the bile ducts within the liver. There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally widened bile ducts affecting an isolated portion of liver. The second form is more diffuse, and when associated with portal hypertension and congenital hepatic fibrosis, is often referred to as "Caroli syndrome". The underlying differences between the two types are not well understood. Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about one in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease.

Cholecystectomy Surgical removal of the gallbladder

Cholecystectomy is the surgical removal of the gallbladder. Cholecystectomy is a common treatment of symptomatic gallstones and other gallbladder conditions. In 2011, cholecystectomy was the eighth most common operating room procedure performed in hospitals in the United States. Cholecystectomy can be performed either laparoscopically, or via an open surgical technique.

Biliary atresia Medical condition

Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. It has an incidence of one in 10,000–15,000 live births in the United States, and a prevalence of one in 16,700 in the British Isles. Biliary atresia is most common in East Asia, with a frequency of one in 5,000.

Common bile duct Gastrointestinal duct

The common bile duct, sometimes abbreviated as CBD, is a duct in the gastrointestinal tract of organisms that have a gallbladder. It is formed by the confluence of the common hepatic duct and cystic duct and terminates by uniting with pancreatic duct, forming the ampulla of Vater. The flow of bile from the ampulla of Vater into the duodenum is under the control of the sphincter of Oddi.

Cystic duct Organ duct

The cystic duct is the short duct that joins the gallbladder to the common hepatic duct. It usually lies next to the cystic artery. It is of variable length. It contains 'spiral valves of Heister', which do not provide much resistance to the flow of bile.

Primary sclerosing cholangitis Medical condition

Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as yellow discoloration of the skin and eyes, itching, and abdominal pain.

Cholangiocarcinoma Bile duct adenocarcinoma

Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts. Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever. Light colored stool or dark urine may also occur. Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater.

Common hepatic duct Exocrine duct

The common hepatic duct is the first part of the biliary tract. It joins the cystic duct coming from the gallbladder to form the common bile duct.

Intrahepatic bile ducts Exocrine organ ducts

Intrahepatic bile ducts compose the outflow system of exocrine bile product from the liver.

Cholestasis Medical condition

Cholestasis is a condition where bile cannot flow from the liver to the duodenum. The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system that can occur from a gallstone or malignancy, and metabolic types of cholestasis which are disturbances in bile formation that can occur because of genetic defects or acquired as a side effect of many medications. Classification is further divided into acute or chronic and extrahepatic or intrahepatic.

Ascending cholangitis Medical condition

Ascending cholangitis, also known as acute cholangitis or simply cholangitis, is inflammation of the bile duct (cholangitis), usually caused by bacteria ascending from its junction with the duodenum. It tends to occur if the bile duct is already partially obstructed by gallstones.

Lobules of liver

The lobules of liver, or hepatic lobules, are small divisions of the liver defined at the microscopic (histological) scale. The hepatic lobule is a building block of the liver tissue, consisting of a portal triad, hepatocytes arranged in linear cords between a capillary network, and a central vein.

Biliary tract Organ system

The biliary tract, refers to the liver, gall bladder and bile ducts, and how they work together to make, store and secrete bile. Bile consists of water, electrolytes, bile acids, cholesterol, phospholipids and conjugated bilirubin. Some components are synthesised by hepatocytes, the rest are extracted from the blood by the liver.

Liver Vertebrate organ involved in metabolism

The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it is located in the right upper quadrant of the abdomen, below the diaphragm. Its other roles in metabolism include the regulation of glycogen storage, decomposition of red blood cells, and the production of hormones.

Vanishing bile duct syndrome is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.

References

  1. Alastair D. Burt; Linda D. Ferrell; Stefan G Hubscher; Bernard C. Portmann (30 August 2011). MacSween's Pathology of the Liver E-Book. Elsevier Health Sciences. p. 499. ISBN   978-0-7020-4754-1.