Primitive sclerosing cholangitis

Primitive sclerosing cholangitis

    * Introduction
    * Pathogenesis
    * Signs and symptoms
    * Diagnosis
    * Treatment
Primitive sclerosing cholangitis is a chronic liver disease carecterizata cholestasis with inflammation and fibrosis of intra and extrahepatic bile ducts. The disease can lead to liver cirrhosis with portal hypertension.
The most commonly affects young men between 30-60 years. Occurs mostly in people with inflammatory bowel disease, ulcerative colitis frequently. Tends to present to family. In some people have an infection or bile duct injury can trigger the condition. And bile ducts may be damaged during endoscopic procedures such as stents implanted to keep open the bile ducts.
On primitive sclerosing cholangitis liver scars are formed and cause severe liver cirrhosis. Scar tissue collapses and blocks the bile ducts in evolution. As a result of bile salts that help the body absorb fat are not normally secreted. The disease resembles primary biliary cirrhosis, except that affects the liver bile ducts outside the liver as well. The cause is unknown but is thought to be autoimmune.
Symptoms usually begins gradually with fatigue and severe itching. Jaundice tends to be installed later. Infetia recurrent inflammation and bile duct may occur in the disease when the ducts are damaged by an investigative procedure. Bacterial cholangitis attacks cause upper abdominal pain, jaundice and fever.
Since bile salts are secreted normally not sick enough not absorb fat and fat-soluble vitamin deficient. Inadequate secretion of bile and leads to osteoporosis, prolonged bleeding, the occurrence of petechiae and foul-smelling stools, steatorrhea and fat. You can develop gallstones and ductal.
As the disease progresses to install cirrhosis, ascites, peripheral edema, and ultimately liver failure.
Treatment relieves itching ursodeoxicolic acid. Recurrent bacterial cholangitis is treated with antibiotics. Blocked ducts can be dilated by ERCP. Implantation of stents may be necessary to keep open the bile ducts.
Liver transplantation is the only prolongs life. People with cirrhosis who have severe complications or those with recurrent bacterial cholangitis may require liver transplantation. The average survival from diagnosis until death is 12 years.