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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. Biliary atresia is the most common reason for pediatric liver trasplantation in the United States. [2]
A hepatoportoenterostomy or Kasai portoenterostomy is a surgical treatment performed on infants with Type IVb choledochal cyst and biliary atresia to allow for bile drainage. In these infants, the bile is not able to drain normally from the small bile ducts within the liver into the larger bile ducts that connect to the gall bladder and small ...
Biliary atresia, or inflammation and destruction of the bile ducts, may lead to jaundice. Vomiting and swelling of the abdominal region are features that suggest improper positioning of the intestines. Poor positioning of the intestine also makes it more prone to blockage, which can result in numerous chronic health issues. [4]
The image produced by this type of medical imaging, called a cholescintigram, is also known by other names depending on which radiotracer is used, such as HIDA scan, PIPIDA scan, DISIDA scan, or BrIDA scan. [1] [2] Cholescintigraphic scanning is a nuclear medicine procedure to evaluate the health and function of the gallbladder and biliary system.
A surgically created passage between the common bile duct and the jejunum in a procedure called a choledochojejunostomy, can be carried out to relieve the symptoms of biliary obstruction as well as allows the bile duct to drain. [5] In infants with biliary atresia, hepatoportoenterostomy is an alternative method of providing bile drainage.
A liver biopsy may indicate too few bile ducts (bile duct paucity) or, in some cases, the complete absence of bile ducts (biliary atresia). Bile duct paucity results in the reduced absorption of fat and fat-soluble vitamins (A, D, E and K), which may lead to rickets or a failure to thrive.
Intrahepatic bile duct atresia (Alagille syndrome) ... T cells recognize biliary epithelial cell antigens causing injury and eventual atresia. Other causes
Percutaneous transhepatic cholangiography, percutaneous hepatic cholangiogram (PTHC) is a radiological technique used to visualize the anatomy of the biliary tract. [1] A contrast medium is injected into a bile duct in the liver, after which X-rays are taken.