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PRIMARY BILIARY CIRRHOSIS (PBC) inflammatory autoimmune disease mainly affecting the intrahepatic bile ducts. The primary feature of this disease is a nonsuppurative, inflammatory destruction of medium-sized intrahepatic bile ducts. It is accompanied by portal inflammation, scarring, and eventual development of cirrhosis and liver failure. middle-aged women, with a female predominance Family members of PBC patients have an increased risk of developing the disease. presents with fatigue and pruritus. Hepatomegaly is a typical finding eyelid xanthelasmas (arise as a result of infiltration of the nasal area of the eyelid by cholesterol-rich macrophages) Hyperpigmentation due to melanin deposition and an inflammatory arthropathy are seen in 25% to 40% of cases. Serum alkaline phosphatase and cholesterol are almost always elevated, even at onset; hyperbilirubinemia is a late development and usually signifies incipient hepatic decompensation. Antimitochondrial antibodies are present in 90% to 95% of patients. They are highly characteristic of PBC and an essential element for diagnosis, together with the elevation of alkaline phosphatase and -glutamyltransferase, which are markers of cholestasis.

Pathogenesis PBC is thought to be an autoimmune disorder aberrant expression of MHC class II molecules on bile duct epithelial cells, accumulation of autoreactive T cells around bile ducts, reaction of antimitochondrial antibodies to hepatocytes, or of other antibodies against cellular components (nuclear pore proteins, and centromeric proteins, among others) The antimitochondrial antibodies, the characteristic autoantibodies in PBC, target the E2 component of the pyrurate dehydrogenase complex (PDC-E2). PDC-E2specific T cells are also present in these patients, supporting the notion of immune-mediated pathogenesis. Morphology PBC is the prototype of conditions leading to small-duct biliary fibrosis and cirrhosis. During the pre-cirrhotic stage portal tracts are infiltrated by a dense accumulation of lymphocytes, macrophages, plasma cells, and occasional eosinophils . Interlobular bile ducts are infiltrated by lymphocytes and may show noncaseating granulomatous inflammation and undergo progressive destruction.

Clinical Features. Eventually, pruritus, fatigue, and abdominal discomfort develop followed in time by secondary features: skin pigmentation, xanthelasmas, steatorrhea, and vitamin D malabsorptionrelated osteomalacia and/or osteoporosis. PBC patients have an increased risk to develop hepatocellular carcinomas.

Treatment ursodeoxycholic acid, if started early, can provide complete remission and prolong survival Liver transplantation is the best form of treatment for persons with end-stage liver disease.

127 LIVER FLUKE ( FROM THE INTERNET) General Liver fluke infection is initiated by ingestion of fluke-infested, raw, freshwater fish and it causes chronic inflammatory bile duct disease.

Complications duct stone formation recurrent pyogenic cholangitis bile duct cancer

129 HEPATIC ABSCESS ( FROM THE INTERNET) Bacterial abscess of the liver is relatively rare. The 3 major forms of liver abscess, classified by etiology, are as follows:

Pyogenic abscess, which is most often polymicrobial, accounts for 80% of hepatic abscess cases in the United States. Amebic abscess due to Entamoeba histolytica accounts for 10% of cases.[2] Fungal abscess, most often due to Candida species, accounts for less than 10% of cases.

Sex

no sexual predilection currently exists.

Age liver abscess was most common in the fourth and fifth decades of life, primarily due to complications of appendicitis.

Clinical Presentation Fever (either continuous or spiking) Chills Right upper quadrant pain Anorexia Malaise Treatment & Management Antibiotic therapy as a sole treatment modality is not routinely advocated, though it has been successful in a few reported cases. Antimicrobial treatment is a common adjunct to percutaneous or surgical drainage.