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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.
In the human liver, bile is composed of 97–98% water, 0.7% bile salts, 0.2% bilirubin, 0.51% fats (cholesterol, fatty acids, and lecithin), and 200 meq/L inorganic salts. [ 2 ] [ 3 ] The two main pigments of bile are bilirubin , which is orange-yellow, and its oxidised form biliverdin , which is green.
This is why chenodeoxycholic acid, and not cholic acid, can be used to treat gallstones (because decreasing bile acid synthesis would supersaturate the stones even more). [6] [7] Cholic acid and chenodeoxycholic acid are the most important human bile acids. Other species may synthesize different bile acids as their predominant primary bile ...
These conjugated bile acids are often referred to as bile salts. The pKa of the unconjugated bile acids are between 5 and 6.5, [ 4 ] and the pH of the duodenum ranges between 3 and 5, so when unconjugated bile acids are in the duodenum, they are almost always protonated (HA form), which makes them relatively insoluble in water.
Bile is secreted by the liver into small ducts that join to form the common hepatic duct. [4] Between meals, secreted bile is stored in the gallbladder. [5] During a meal, the bile is secreted into the duodenum (part of the small intestine) to rid the body of waste stored in the bile as well as aid in the absorption of dietary fats and oils. [5]
These lipid-soluble bile acids are conjugated (reversibly attached) mainly to glycine or taurine molecules to form water soluble primary conjugated bile acids, sometimes called "bile salts". These bile acids travel to the gall bladder during the interdigestive phase for storage and to the descending part of the duodenum via the common bile duct ...
Retention of bile salts within hepatocytes, which are the only cell type to express BSEP, causes hepatocellular damage and cholestasis. [citation needed] PFIC-3 is caused by a variety of mutations in ABCB4, the gene encoding multidrug resistance protein 3 (MDR3), [5] which codes for a floppase responsible for phosphatidylcholine translocation ...
Biliverdin (from the Latin for green bile) is a green tetrapyrrolic bile pigment, and is a product of heme catabolism. [ 1 ] [ 2 ] It is the pigment responsible for a greenish color sometimes seen in bruises .