United Network for Organ Sharing. 1100 Boulders Parkway, Suite 500, P.O. Box 13770, Richmond, VA 23225-8770. (804) 330-8500. http://www.unos.org .
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Cirrhosis of the Liver. April 200 [cited October 2002]. http://www.niddk.nih.gov/health/digest/pubs/cirrhosi/cirrhosi.htm .
[ sĭ-ro´sis ]
a liver disease (actually a group of chronic diseases) characterized by loss of the normal microscopic lobular architecture and regenerative replacement of necrotic parenchymal tissue with fibrous bands of connective tissue that eventually constrict and partition the organ into irregular nodules. It has a lengthy latent period, usually followed by the sudden appearance of abdominal pain and swelling, hematemesis, dependent edema, or jaundice. In advanced stages, ascites, pronounced jaundice, portal hypertension, and central nervous system disorders, which may end in hepatic coma. become prominent. adj. adj cirrhot´ic.
Clinical Manifestations. The signs and symptoms are manifestations of interference with the major functions of the liver: (1) the storage and release of blood to maintain adequate circulating volume, (2) the metabolism of nutrients and the detoxification of poisons absorbed from the intestines, (3) the regulation of fluid and electrolyte balance, and (4) production of clotting factors.
The patient with alcoholic cirrhosis (Laënnec's cirrhosis ) may be admitted to the hospital with acute alcoholic hepatitis, marked by fever and dehydration. Prominent spider angiomas and redness of the palms of the hands (palmar erythema) are usually present. delirium tremens may be a difficult problem during the early phase of hospitalization. Data from liver function tests usually show elevated transaminase levels, elevated bilirubin levels, and decreased values for albumin and clotting factors. (See also alcoholism .)
Continued fluid and electrolyte imbalances and inefficient metabolism of nutrients produce ascites, hypoglycemia, and hypoproteinemia. Obstruction to the return of blood from the portal system causes increased pressure within the veins of the esophagus and stomach. These engorged vessels are subject to rupture with subsequent hemorrhage that is abetted by clotting disorders. jaundice develops as a result of biliary obstruction.
Neurological symptoms begin with subtle changes in mental acuity, mild memory loss, poor reasoning ability, and irritability. Tremor of the outstretched hands (asterixis) is common. These symptoms become more severe and may eventually progress to delirium, suicidal tendencies, and coma.
Treatment. The major goals of treatment for the patient with cirrhosis are: (1) to maintain liver function at its current level and prevent further deterioration of the organ, (2) to maintain electrolytes within normal limits, (3) to maintain sufficient respiratory function, (4) to prevent or resolve gastrointestinal bleeding, and (5) to provide adequate nutritional intake and a positive nitrogen balance.
Since there is no cure for cirrhosis, supportive measures are instituted to help the liver rebuild and repair its damaged cells. Prevention of further deterioration of the cells is accomplished by removing the primary cause; for example, restriction of the intake of alcohol or other toxic agent, treatment of infection, and providing an adequate nutritional intake; supplemental vitamins are often prescribed.
Severe blood loss is compensated for by transfusions of whole blood. Excessive bleeding from esophageal varices may necessitate insertion of a Sengstaken-Blakemore tube. This device has three channels: one for inflation of the esophageal balloon, one for inflation of the gastric balloon, and a third for aspiration of stomach contents.
Relief of portal hypertension sometimes is accomplished by a surgical procedure called a portacaval shunt. The portal vein is surgically connected to the inferior vena cava to allow drainage of excessive amounts of blood from the portal system to the general circulation. A similar procedure called the splenorenal shunt involves connecting the splenic vein to the renal vein.
Removal of fluid from the abdominal cavity (abdominal paracentesis ) may be necessary to relieve respiratory embarrassment or pressure on the abdominal organs caused by ascites. A more permanent resolution of the problem of chronic ascites is surgical creation of a peritoneovenous shunt .
Hepatic encephalopathy and coma can be precipitated by any of a number of factors, including gastrointestinal bleeding, fluid and electrolyte and acid-base imbalances, intercurrent infection and fever, administration of analgesics and sedatives that are central nervous system depressants, and increased dietary protein intake.
Patients with chronic hepatic encephalopathy are placed on a protein-restricted diet. An antibiotic may be prescribed to reduce bacterial flora in the intestine. Surgical removal of all or part of the bacteria-laden colon is an alternative treatment, but the surgical risk is high in these chronically ill patients.
Patient Care. In view of the many functions of the liver, it is essential that a thorough assessment of the patient be done to identify specific problems before a plan of care is developed. Among the problems likely to be associated with cirrhosis are self-care deficit related to low energy level due to inadequate metabolism of carbohydrates; fluid volume excess, especially ascites; potential for impairment of skin integrity related to edema, jaundice, and itching; electrolyte imbalance related to poor storage of minerals; alteration in comfort; tendency to bleed excessively related to deficits of vitamin K and prothrombin and the presence of esophageal varices; potential for infection related to decreased levels of gamma globulins and dysfunction of phagocytic Kupffer cells; impaired gas exchange related to pressure against the lungs by ascitic fluid; potential for injury related to altered levels of consciousness; alteration in nutrition related to indigestion, nausea, inability of liver cells to metabolize food elements, or confusion and depression; and diarrhea related to diminished bile production and decreased tolerance to fatty acids.
Patients in the advanced stages of cirrhosis require periodic and thorough monitoring to detect blood loss in the form of hematemesis, tarry stools, bleeding gums, frequent and heavy nosebleeds, and bruising. In order to evaluate fluid status the fluid intake and output and daily weight are measured and recorded.
Dietary restrictions and prohibition of alcohol may result in noncompliance in some patients. Education must include the purpose of these restrictions, the expected effect and dosage of medications that have been prescribed, and the importance of adequate nutrition, rest, and preservation of independence within the patient's capabilities. Compliance may be improved by enhancing the patient's self-esteem, emphasizing personal strengths, encouraging the use of available community resources such as alcoholics anonymous if alcoholism is a problem, and providing for active participation of the patient and family in planning and implementing some aspects of care.
acholangic cirrhosis a liver disorder affecting children up to 12 years of age, due to complete or partial agenesis of the intrahepatic, intralobular bile ducts, with manifestations similar to those seen in obstructive biliary cirrhosis.