Cholestasis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

In medicine, cholestasis is a condition where bile cannot flow from the liver to the duodenum. Bile formation is a secretory function of the liver. It begins in bile canaliculi that form between two adjacent surfaces of liver cells (hepatocytes) similar to the terminal branches of a tree. The canaliculi join each other to form larger and larger structures, sometimes referred to as Canals of Hering, which themselves join to form small bile ductules that have an epithelial surface. The ductules join to form bile ducts that eventually form either the right main hepatic duct that drains the right lobe of the liver and the left main hepatic duct draining the left lobe of the liver. The two ducts join to form the common hepatic duct, which in turn joins the cystic duct from the gall bladder, to give the common bile duct. This duct then enters the duodenum at the ampulla of Vater. The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system such as 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.

Under a microscope, the individual hepatocytes will have a brownish-green stippled appearance within the cytoplasm, representing bile that cannot get out of the cell. Canalicular bile plugs between individual hepatocytes or within bile ducts may also be seen, representing bile that has been excreted from the hepatocytes but cannot go any further due to the obstruction. When these plugs occur within the bile duct, sufficient pressure (caused by bile accumulation) can cause them to rupture, spilling bile into the surrounding tissue, causing hepatic necrosis. These areas are known as bile lakes, and are typically seen only with extra-hepatic obstruction.

Blood tests may show higher than normal levels of bilirubin and alkaline phosphatase.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Under a microscope, the individual hepatocytes will have a brownish-green stippled appearance within the cytoplasm, representing bile that cannot get out of the cell. Canalicular bile plugs between individual hepatocytes or within bile ducts may also be seen, representing bile that has been excreted from the hepatocytes but cannot go any further due to the obstruction. When these plugs occur within the bile duct, sufficient pressure (caused by bile accumulation) can cause them to rupture, spilling bile into the surrounding tissue, causing hepatic necrosis. These areas are known as bile lakes, and are typically seen only with extra-hepatic obstruction.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

There are many causes of cholestasis.

• Extrahepatic cholestasis occurs outside the liver. It can be caused by:

• Bile duct tumors
• Cysts
• Narrowing of the bile duct (strictures)
• Stones in the common bile duct
• Pancreatitis
• Pancreatic tumor or pseudocyst
• Pressure on an organ due to a nearby mass or tumor
• Primary sclerosing cholangitis

• Intrahepatic cholestasis occurs inside the liver. It can be caused by:

• Alcoholic liver disease
• Amyloidosis
• Bacterial abscess in the liver
• Being fed through a vein (IV)
• Lymphoma
• Intrahepatic cholestasis of pregnancy
• Primary biliary cirrhosis
• Primary sclerosing cholangitis
• Sarcoidosis
• Serious infections that have spread through the bloodstream (sepsis)
• Tuberculosis
• Viral hepatitis
• Sjogren syndrome

• Certain medications can also cause cholestasis.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

• After liver transplantation
• After ulcer surgery
• Alagille Syndrome
• Alcoholic Hepatitis
• Alpha-1 Antitrypsin Deficiency
• Amebiasis
• Amyloidosis
• Bile duct carcinoma
• Bile duct calculi
• Bile duct cysts
• Biliary atresia
• Budd-Chiari Syndrome
• Bylers Syndrome
• Carbonic anhydrase inhibitors
• Caroli’s Syndrome
• Cholangiocellular carcinoma
• Cholecystectomy
• Choledochotomy with T-tube drainage
• Chronic active hepatitis
• Cryptosporidiosis
• Cystic Fibrosis
• Drugs
• Dubin-Johnson Syndrome
• Duodenal abscess
• Echinococcosis
• Familiar progressive cholestasis
• Hemochromatosis
• Hydatid cyst
• Idiopathic postoperative jaundice
• Idiopathic relapsing cholestasis
• Impaired bile acid synthesis
• Intermittent cholestasis
• Ischemia of other origin
• Leptospirosis
• Liver abscess
• Liver Cancer
• Liver cysts
• Liver metastases
• Lymphoma
• Mirizzi’s Syndrome
• Neoplasm
• Pancreatic Cancer
• Pancreatic cyst
• Pancreatitis
• Pancreatoduodenectomy
• Papillary carcinoma
• Parasites
• Parasitosis
• Pericholedochal infection
• Postoperative
• Pregnancy cholestasis
• Primary and secondary sclerosing cholangitis
• Primary biliary cirrhosis
• Primary sclerosing cholangitis
• Rare congenital causes
• Relapsing cholangitis
• Reye’s Syndrome
• Sarcoidosis
• Sepsis
• Sickle Cell Anemia
• Stenosis of the bile papilla
• Stricture
• Total parenteral nutrition
• Toxic, allergic
• Toxic Shock Syndrome
• Viral Hepatitis
• Wilson’s Disease
• Zieve’s Syndrome

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