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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.
Cholic acid, sold under the brand name Cholbam, is approved for use in the United States and is indicated as a treatment for children and adults with bile acid synthesis disorders due to single enzyme defects, and for peroxisomal disorders such as Zellweger syndrome.
Cholestasis is a condition where the flow of bile from the liver to the duodenum is impaired. [1] The two basic distinctions are: [1] obstructive type of cholestasis, where there is a mechanical blockage in the duct system that can occur from a gallstone or malignancy, and
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.
Chronic diarrhea may be caused by excess bile salts entering the colon rather than being absorbed at the end of the small intestine (the ileum). This condition of bile acid malabsorption occurs after surgery to the ileum , in Crohn's disease , with a number of other gastrointestinal causes, or is commonly a primary, idiopathic condition.
The bile components that form gallstones include cholesterol, bile salts, and bilirubin. [2] Gallstones formed mainly from cholesterol are termed cholesterol stones, and those formed mainly from bilirubin are termed pigment stones. [2] [3] Gallstones may be suspected based on symptoms. [4] Diagnosis is then typically confirmed by ultrasound. [2]
Genetic mutations affecting hepatic bile salt transport molecules have also been found in patients with progressive familial intrahepatic cholestasis. It has been found that mothers of patients with this disease have a higher incidence of ICP, suggesting that heterozygote carriers of these mutations are also predisposed to ICP.
In the lower small intestine and colon, bacteria dehydroxylate some of the primary bile salts to form secondary conjugated bile salts (which are still water-soluble). Along the proximal and distal ileum, these conjugated primary bile salts are reabsorbed actively into hepatic portal circulation. Bacteria deconjugate some of the primary and ...