biliary cirrhosis


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Related to biliary cirrhosis: secondary biliary cirrhosis

cirrhosis

 [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.
alcoholic cirrhosis Laënnec's cirrhosis.
atrophic cirrhosis cirrhosis in which the liver is decreased in size; it may be seen in the alcoholic, but is more common in posthepatic or postnecrotic cirrhosis.
biliary cirrhosis cirrhosis caused by obstruction or infection of the major extra- or intrahepatic bile ducts (except in primary biliary cirrhosis). It is marked by jaundice, abdominal pain, steatorrhea, and enlargement of the liver and spleen.
cardiac cirrhosis cirrhosis complicating heart disease, with recurrent intractable congestive heart failure. Called also cardiocirrhosis.
congestive cirrhosis cirrhosis resulting from increased hepatic venous pressure or thrombosis; commonly due to congestive heart failure (see cardiac cirrhosis) or to obstruction of the hepatic vein.
fatty cirrhosis cirrhosis in which liver cells are infiltrated with fat (triglyceride), the infiltration usually being due to alcohol ingestion; see laënnec's cirrhosis.
Laënnec's cirrhosis cirrhosis associated with chronic alcoholism. In the early stages, liver enlargement may reflect fatty infiltration of liver cells (fatty cirrhosis) with necrosis and inflammation due to acute alcohol injury; progressive fibrosis extending from portal areas separates uniform small regeneration nodules. Some attribute the condition to a nutritional deficiency associated with alcoholism and others to chronic exposure to alcohol as a hepatotoxin. Called also alcoholic cirrhosis.
macronodular cirrhosis morphological changes that cause the liver to become small and shrunken.
metabolic cirrhosis cirrhosis associated with metabolic diseases such as hemochromatosis, wilson's disease, glycogen storage disease, galactosemia, and disorders of amino acid metabolism.
micronodular cirrhosis morphological changes in the liver resulting in an enlarged liver.
mixed cirrhosis morphological changes in the diseased liver that represent both the micronodular and macronodular patterns.
portal cirrhosis Laënnec's cirrhosis.
posthepatic cirrhosis cirrhosis (usually macronodular) resulting as a sequel to acute hepatitis.
postnecrotic cirrhosis cirrhosis following submassive necrosis of the liver (subacute yellow atrophy) due to toxic or viral hepatitis.
primary biliary cirrhosis a rare form of biliary cirrhosis of unknown etiology, occurring without obstruction or infection of the major bile ducts, sometimes developing after the administration of such drugs as chlorpromazine and arsenicals. Affecting chiefly middle-aged women, it is characterized by chronic cholestasis with pruritus, jaundice, and hypercholesterolemia with xanthomas, and malabsorption.
secondary biliary cirrhosis cirrhosis resulting from chronic bile obstruction due to congenital atresia or stricture.

bil·i·ar·y cir·rho·sis

cirrhosis due to biliary obstruction, which may be a primary intrahepatic disease or secondary to obstruction of extrahepatic bile ducts; the latter may lead to cholestasis and proliferation in small bile ducts with fibrosis, but marked disturbance of the lobular pattern is infrequent.
See also: primary biliary cirrhosis.

biliary cirrhosis

n.
1. Cirrhosis of the liver caused by obstruction of the bile ducts.

biliary cirrhosis

Etymology: L, bilis + kirrhos, yellow-orange, osis, condition
an inflammatory condition in which the flow of bile through the ductules of the liver is obstructed. Primary biliary cirrhosis most commonly affects women in their middle years and is often associated with antimitochondrial antibodies. Its cause is unknown. It is characterized by itching, jaundice, steatorrhea, and enlargement of the liver and spleen. The disease is slowly progressive. Treatment includes ursodeoxycholic acid. Care must be taken to rule out secondary biliary cirrhosis caused by obstruction of the biliary structures outside the liver, because the latter condition can be treated more successfully. Compare biliary calculus, biliary obstruction.
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Biliary cirrhosis

bil·i·ar·y cir·rho·sis

(bil'ē-ār-ē sir-ō'sis)
Hepatic disorder due to biliary obstruction,which may be a primary intrahepatic disease or secondary to obstruction of extrahepatic bile ducts; the latter may lead to cholestasis and proliferation in small bile ducts with fibrosis, but marked disturbance of the lobular pattern is infrequent.

biliary cirrhosis

A slowly developing liver disease in which a widespread inflammation of the small internal bile ducts causes a gradual replacement of functioning liver with scar tissue. It may be an AUTOIMMUNE disorder, secondary to obstruction to the outflow of bile from the liver, or of unknown cause (primary biliary cirrhosis).
References in periodicals archive ?
found that patients with primary biliary cirrhosis presented low spinal and femoral neck BMD and reduced fractional calcium absorption.
The histology was reported as highly suggestive of primary biliary cirrhosis despite non-supportive serology.
These findings are consistent with primary biliary cirrhosis, stage II (see Table I, November 2012).
Association of estrogen receptor single-nucleotide polymorphisms with abnormal alkaline phosphatase, gamma-glutamyl transpeptidase, and TBIL in primary biliary cirrhosis patients
The report provides a snapshot of the global therapeutic landscape of Primary Biliary Cirrhosis
Hyperlipidaemicstate and cardiovascular risk in primary biliary cirrhosis.
Primary biliary cirrhosis most commonly affects middle-aged women, with a female to male ratio ranging from 9 to 14 women to every 1 man.
Coverage of the Primary Biliary Cirrhosis pipeline on the basis of route of administration and molecule type.
Clonal expansion of EBV-infected B cells has been found in the thyroid glands of Hashimoto's thyroiditis patients, blood in SLE, cerebrospinal fluid in MS, salivary glands in Sjogren's syndrome, synovium in rheumatoid arthritis, liver in primary biliary cirrhosis and muscle in dermatomyositis and polymyositis.
Women with primary biliary cirrhosis outnumber men by approximately 10:1, and most are older than 50 years at disease onset.
Cholangitis/cholangiohepatitis is a potentially fatal liver disease and should be considered as a disease complex consisting of cholangitis, cholangiohepatitis and biliary cirrhosis where the tissues involved are either the biliary tree alone or more commonly the biliary tree and surrounding hepatocellular parenchyma.
Other causes of cirrhosis liver are autoimmune hepatitis, primary biliary cirrhosis, Wilson's disease, haemochromatosis, a-1 antitrypsin deficiency, primary sclerosing cholangitis (PSC), granulomatous disease like sarcoidosis, type IV glycogen storage disease, various drugs like methotrexate, alpha methyldopa, amiodarone, venous outflow obstruction like Budd-chiari syndrome, veno-occlusive disease, chronic right-sided heart failure, and tricuspid regurgitation.