Hepatic disease primary biliary cirrhosis

Immunoglobulins. Immunoglobulins are commonly increased in cirrhosis, autoimmune hepatitis, and primary biliary cirrhosis but are normal in most other types of liver disease. IgG is increased in autoimmune hepatitis and cirrhosis IgM is increased in primary biliary cirrhosis. IgA tends to be increased in aU types of cirrhosis. None of these findings are specific, and they are seldom used in the diagnosis of liver disease. 

Liver disease (primary biliary cirrhosis, autoimmune hepatitis, primary sclerosing cholangitis) Hepatitis C virus, hepatitis D virus, herpes simplex virus, enteric bacteria... 

Matsumoto T, Kobayashi S, Shimizu H, et al (2000) The liver in collagen diseases pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver. Liver 20 366-373 Meyers RL, Scaife ER (2000) Benign liver and biliary tract masses in infants and toddlers. Semin Pediatr Surg 9 146-155... 

Autoimmune diseases such as primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis,... 

Hepatic or severe renal dysfunction, including primary biliary cirrhosis preexisting gallbladder disease hypersensitivity to gemfibrozil. 

A 70-year-old woman with a 2-year history of primary biliary cirrhosis confirmed by histological and immunological criteria took colestyramine sachets twice daily for 2 months and developed lethargy, confusion, and drowsiness (3). She had signs of chronic liver disease, portal hypertension, and hepatic encephalopathy. Laboratory investigations confirmed a metabolic acidosis (pH 7.15) and hyperchloremia. Multiple cultures failed to reveal sepsis, and a urinary pH of 4.85 together with tests of renal acidification excluded renal tubular acidosis. No other cause was found and she responded to 600 mmol of sodium bicarbonate intravenously over 36 hours. 

Caeruloplasmin Copper-incorporating a glycoprotein true function remains unclear but acts as a copper donor and oxidative enzyme Low caeruloplasmin levels may be seen in cirrhosis (especially primary biliary cirrhosis) as caeruloplasmin is excreted hepatically Levels increased in infection, injury or inflammation. Low levels are found in Wilson s disease, which is an autosomal recessive disorder of copper metabolism it results in copper deposition in the liver, basal ganglia and eyes, and culminates in cirrhosis and neurological impairment... 

Dietrich, C.F., Lenschner, M.S., Zenzem, S., Herrmann, G., Sarrazin, C., Caspary, W.F., Lenschner, U.F.H. Peri hepatic lymphadenopathy in primary biliary cirrhosis reflects progression of the disease. Eur. J. Gastroenterol. Hepatol. 1999 11 747-753... 

Simko, V., Michael, S., Prego, V. Ursodeoxycholic therapy in chronic liver disease A meta-analysis in primary biliary cirrhosis and in chronic hepatitis. Amer. X. Gastroenterol. 1994 89 392—398... 

Polyclonal increases in serum immunoglobulins are the normal response to infections. IgG response predominates in autoimmune responses IgA in skin, gut, respiratory, and renal infections and IgM in primary viral infections and bloodstream parasites, such as malaria. Chronic bacterial infections may cause an increase in serum levels of all immunoglobulins. In such cases, estimations of the individual immunoglobulins seldom provide more information than protein electrophoresis. They are of value, however, in the differential diagnosis of liver disease and of intrauterine infections. In primary biliary cirrhosis, the IgM level is greatly increased in chronic active hepatitis, IgG and sometimes IgM are increased and in portal cirrhosis, IgA and sometimes IgG are increased. In intrauterine infections, production of IgM by the fetus increases, and the IgM level in umbilical cord blood is increased. Estimations of IgE are used in the management of asthma and other allergic conditions, especially in children. 

In liver disease, decrease in hepatic 25-hydroxylase activity has little effect on serum l,25-(OH)2D concentration. Even when serum 25-(OH)D concentration is reduced in primary biliary cirrhosis, the 1,25-(OH)2D level... 

Liver disease, autoimmune. Diseases caused by autoimmune-mediated inflammation and/or fibrosis autoimmune hepatitis, -primary biliary cirrhosis, and primary sclerosing cholangitis. 

Sjogren syndrome. Chronic inflammatory autoimmune disease of the exocrine glands of unknown etiology. Its primary symptoms are keratoconjunctivitis sicca and xerostomia. Two types of Sjogren syndrome are distinguished a primary (isolated) type and a secondary type associated with another underlying autoimmune disease (e.g. rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, primary biliary cirrhosis, autoimmune hepatitis, multiple sclerosis, thyroiditis, autoimmune, etc.). Ro/SS-A and La/SS-B autoantibodies are used as classification criteria. 

Anomalies in the acute phase protein response have been reported in various diseases. Studies in systemic lupus erythematosus, dermatomyositis, systemic sclerosis, mixed connective tissue disease, ulcerative colitis, primary biliary cirrhosis, and chronic active hepatitis have shown low acute phase protein responses for the amount of inflammatory activity present (P6). This may be due either to disease-related unresponsiveness or to down-regulation of the acute phase response in chronic inflammation (Ml, W16). Patients with systemic sclerosis do not mount an appreciable acute phase response to therapeutic infusion of PGE, compared with patients with atherosclerosis (W24), although they do to infections (C20). 

Kayser-Fleischer rings by slit lamp Present - In up to 40% of patients with Hepatic Wilson disease - In most asymptomatic siblings Primary biliary cirrhosis... 

Other Disorders. Copper retention is expected in any form of liver disease which interferes with biliary excretion. Primary biliary cirrhosis shows a consistent and gross hepatic copper retention. 

Diseases in which the body produces antibodies against its own components. Among the diseases which are thought to have an autoimmune basis are primary myxoedema, thyrotoxicosis, pernicious anaemia, Addison s disease, Goodpasture s syndrome, myasthenia gravis, some haemolytic anaemias, primary biliary cirrhosis, active chronic hepatitis, ulcerative colitis, Sjogren s syndrome, rheumatoid arthritis and systemic lupus erythematosus. See separate entries for some of these diseases. 

The conditions for which hepatic transplantation has been applied differ in pediatric and adult cases and are shown in Tables 1 and 2. 50% of the pediatric cases have been transplanted for biliary atresia.The majority of the other pediatric recipients have been transplanted for alpha 1 antitrypsin deficiency or chronic liver disease of unknown ethiology. In contrast, the leading conditions for which hepatic transplantation has been applied in adult recipients are postnecrotic cirrhosis, primary biliary cirrhosis and primary hepatic malignancy. 

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