Focal Biliary Fibrosis Cirrhosis

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This is a description of the pathology in the liver of CF patients. The scarring around the biliary tree is not generalized, but patchy, and likely occurs to some degree in most patients, although clearly some patients are more affected and go on to an extensive lesion with cirrhosis and consequent portal hypertension. The fibrosis alone does not produce symptoms. Biochemical tests of liver enzymes (aspartate aminotransferase, alanine aminotransferase, GGT, alkaline phosphatase) may, however, be mildly elevated (1.5 to 4 times normal), and if this is sustained for > 3 months while the patient is otherwise well, then treatment with ursodeoxycholic acid (20 mg/kg/d) is started. Once initiated, this would continue as lifelong therapy at most centers.

Liver disease, including focal biliary fibrosis, may be entirely silent until a patient presents with cirrhosis. About 15% of patients have or will develop cirrhosis or important liver disease, and liver disease is the third most common cause of death, accounting for about 2.5% of CF deaths each year. The basic genotype likely comes under the influence of modifier genes and other environmental influences in these patients.

The presentation of cirrhosis may be that of hepatosplenomegaly and portal hypertension with GI bleeding. Usually this bleeding is from esophageal varices, although of course not all bleeding in patients with cirrhosis is from varices; therefore, endoscopy will be needed not just for therapy, but to diagnose the bleeding site and lesion. Endoscopy confirms the site of bleeding and rules out gastric varices, hypertensive gastropathy, or peptic ulcer disease. Usual treatment for varices is then applied, including banding or sclerotherapy. For those where bleeding is not controlled, or those who have gastric varices that cannot be banded, surgical decompression by transjugular intrahepatic portosystemic shunt or surgical shunt such as splenorenal shunt may be required. Some patients who continue to bleed despite these aggressive measures, may need to be listed for transplant as the ultimate means of decompressing the portal circulation.

Many gastroenterologists will also think about going on to secondary prevention of bleeding varices with nonselective p-blockers, such as propranolol, to decrease splanchnic pressure and cardiac output (Shashidhar et al, 1999). The respirologist caring for the patient needs to be consulted before starting this therapy to ensure it will not interfere with other therapy, such as the use of bronchodilators for lung disease. Some centers would avoid the use of p-blockers except in those cases where bleeding cannot be controlled by banding, such as patients with gastric varices or portal hypertensive gastropathy. Should the drug need to be stopped at some point, the risk for rebleeding appears to return to baseline risk. Primary prevention of variceal bleeding in CF has not been well studied. Gastorenterologists who consider either p-blockers or banding must take into account the severity of the pulmonary disease, other medications the patient may be on, and the risk for general anesthesia. All these may argue against treatment before the first bleed.

Patients with complications of portal hypertension, such as bleeding, ascites, and malnutrition, are candidates for liver transplant, and referral should be made at an early stage, taking into account the severity of the lung disease in the patient. We have shown that survival is good and quality of life is improved posttransplant (Mack et al, 1995). Some patients do well with transplant of liver and lungs.

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