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Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. [ 1 ] [ 2 ] [ 3 ] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis .
Cholestatic pruritus is the sensation of itch due to nearly any liver disease, but the most commonly associated entities are primary biliary cholangitis, primary sclerosing cholangitis, obstructive choledocholithiasis, carcinoma of the bile duct, cholestasis (also see drug-induced pruritus), and chronic hepatitis C viral infection and other forms of viral hepatitis.
biliary dyskinesia; ICD-10 code K83: other diseases of the biliary tract: cholangitis (including ascending cholangitis and primary sclerosing cholangitis) obstruction, perforation, fistula of biliary tract (bile duct) spasm of sphincter of Oddi; biliary cyst; biliary atresia
These diseases include primary biliary cirrhosis, primary sclerosing cholangitis, AIDS cholangiopathy, disappearing bile duct syndromes, Alagille's syndrome, cystic fibrosis, and biliary atresia. Often either the best or the only curative treatment option for cholangiopathies is liver transplantation , and as a group, cholangiopathies account ...
Jaundice of the skin or eyes is an important physical finding in biliary obstruction. Jaundice and/or clay-colored stool may raise suspicion of choledocholithiasis or even gallstone pancreatitis. [1] If the above symptoms coincide with fever and chills, the diagnosis of ascending cholangitis may also be considered.
The canals of Hering are destroyed early in primary biliary cholangitis and may be primary sites of scarring in methotrexate toxicity. Research has indicated the presence of intraorgan stem cells of the liver that can proliferate in disease states, so-called oval cells. [5] Liver stem cells are theorized to originate in the niches of the canals.
Primary sclerosing cholangitis is one of the major known risk factors for cholangiocarcinoma, [40] a cancer of the biliary tree, for which the lifetime risk among patients with PSC is 10-15%. [3] This represents a 400-fold greater risk of developing cholangiocarcinoma compared to the general population. [ 1 ]
Complications from delayed surgery include pancreatitis, empyema, and perforation of the gallbladder, cholecystitis, cholangitis, and obstructive jaundice. [13] Biliary pain in the absence of gallstones, known as postcholecystectomy syndrome, may severely affect the patient's quality of life, even in the absence of disease progression. [14]