Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear. Bile is a liquid produced in the liver that travels through the bile ducts to the gallbladder and then the small intestine, where it helps digest fats and fat-soluble vitamins A, D, E, and K. When the bile ducts become damaged from chronic inflammation, bile builds up in the liver, injuring liver tissue.
The genome of complex organisms is stashed away inside each cell's nucleus, a little like a sovereign shielded from the threatening world outside.
Researchers have discovered a novel molecular path that predisposes patients to develop primary biliary cirrhosis, a disease that mainly affects women and slowly destroys their livers. Primary biliary cirrhosis has no known cause.
Needle liver biopsy has been used as the "gold standard" for the assessment of liver fibrosis and disease stage of PBC.
Scientists of the Center for Applied Medical Research (CIMA) of the University of Navarra have discovered the molecular mechanism responsible for the effectiveness of an existing treatment for primary biliary cirrhosis, which combines two substances in order to produce an effect that does not result from either substance separately.
Liver biopsy is a widely used tool in the investigation of liver diseases. It is invasive and has a mortality risk ranging between 0.01% and 0.17%. A liver biopsy should therefore only be performed in patients who would potentially benefit from it.
Patients who undergo liver transplantation at age 60 or above have 1-year and 5-year survival rates similar to those of younger patients and they experience fewer episodes of rejection.
Armed with this new information, physicians could screen and assess first-degree relatives of PBC patients with a simple blood test, enabling them to diagnose and treat more patients before the disease causes irreversible liver damage. These findings were published in this month's issue of Hepatology.
Published in the March 2006 issue of Hepatology, researchers found significant clusters of the disease near Superfund toxic waste sites (SFS) and that the majority of patients in New York City who need liver transplants because of PBC, reside near SFS.
Liver disease is often associated with "sickness behaviors," such as malaise, listlessness, anorexia, difficulty concentrating, and fatigue. In cholestatic liver diseases (where bile production is impaired) such as primary biliary cirrhosis, fatigue occurs in up to 86 percent of patients.
A case-control study of more than 2000 people has identified a number of factors that may induce primary biliary cirrhosis (PBC) in genetically susceptible individuals. These include a history of urinary tract infections, hormone replacement therapy, tobacco use, and nail polish use.
In five randomized controlled trials involving 457 people, the authors report that for patients treated with methotrexate, as a single therapy or in combination with other drug treatment, pooled data showed a tendency toward an increased risk of death or liver transplantation. The brand names for methotrexate are Rheumatrex and Trexall.