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Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, chronic liver failure or chronic hepatic failure and end-stage liver disease, is an acute condition of the liver in which the normal functioning tissue, or parenchyma, is replaced with scar tissue and regenerative nodules as a result of chronic liver disease.
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.
Treatment generally includes supportive care including pain management and possibly diuretics. [5] In those with severe disease due to a bone marrow transplant, defibrotide is a proposed treatment. [6] It has been approved for use in severe cases in Europe and the United States. [7] [8] A placebo controlled trial, however, has not been done as ...
No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a liver transplant, [1] but disease recurrence can occur in 25–30% of cases. [6] PSC is a rare disease and most commonly affects people with IBD. [2]
In some patients this syndrome has also been associated with Sjögren's syndrome and hemolytic anemia. Typical clinical features include jaundice, elevated blood levels of alkaline phosphatase, calcinosis cutis, telangiectasias, and pruritus. This disease may cause white or yellow-ish spots on the arms or legs.
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.
Liver cancer and cholangiocarcinoma may occur, but at a lower incidence than other chronic liver diseases, and the risk is greatly reduced with treatment. [13] With early detection and treatment, most of those affected can live relatively normal lives and have a life expectancy close to that of the general population. [ 13 ]
Bile duct obliteration usually worsens over time, causing cirrhosis of the liver and eventual failure. [48] Diagnosis usually occurs using the classic criteria by looking at changes associated with the five systems discussed earlier. [53] Like FIH, the definitive treatment is a liver transplant. [54]
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