I. Approve or contradict:

1. women are affected more frequently than men;

2. Over a period of week or months the patient notes gradually ceas­ing weakness, anorexia, great or slight weight loss, etc.;

3. Clinical signs and symptoms can reflect accurately the extent and precise nature of cirrhosis process;

:ess, evidence

hypertension^, „notes ^radi^lly eight loss, jaun- A f iypfand^ШШША liver may be the огЦу typical'^!|^«gs^clude muscle j^aitfJngT^ angiomas, testicular atrophy (in men), men-

4. enorrhea (in wpmetijy palmar erythem^ spleno-

   Гої^а gland^^4rge>ment, pur- mentatipii of the skin №

   Ее fever without

   low-grade fever without shaking chills is often in patients with active disease;

5. although some patients die during the acute exacerbations, тоні recover after several weeks.

II. Look for the answers to the following questions.

1. Why do the morphologic elements of cirrhosis often have dramatir clinical counterparts?

2. How many types of cirrhosis can be classified?

3. What may not reflect accurately the extent and precise nature of cirrhosis process?

4. What occur with greater frequency in cirrhotic paients than in normal subject?

5. What lead to further episodes of hepatic decompensation?

BILIARY CIRRHOSIS

Biliary cirrhosis refers to a disorder in patients with clinical and chemical signs of chronic impairment of bile excretion and morphologic evidence of progressive liver destruction centered about the intrahepatic bile ducts. Major clinical concomitants of impaired bile, excretion include protracted itching; progressive and prolonged jaundice; steatorrhea; hepatomegaly; laboratory findings of marked elevations of serum alkaline phosphatase, cholesterol, and other lipid fractions; and slowly progressive decline of health. Morphologically most forms of biliary cirrhosis evolve from chronic inflammatory lesions of the periportal liver cells, ductules, and interlobular ducts, and true cirrhosis represents a late and often non­specific phase.

Primary biliary cirrhosis is associated with s number of specific and nonspecific immunologic features:

1. Elevated serum levels of IgM are seen in about 80 percent of patients with primary biliary cirrhosis, but this abnormality is nonspe­cific and occurs occasionally in patients with mechanical bile duct ob­struction.

2. Immunofluorescent studies have demonstrated a circul j anti­body that reacts with the cytoplasm of bile ductular cells in 75 percent of patients with primary biliary cirrhosis. However, similar antibody chan­ges occur in many patients with viral hepatitis.

3. Periductal lymphocytes in liver biopsy specimens from patients with primary biliary cirrhosis have been shown to form IgM immunoglob­ulins, again raising the possibility of a self-perpetuating immune process.

4. Impaired lymphocytic transformation has been documented in some patients, suggesting derangement of delayed immune responses.

5. A circulating antibody (IgG) is present in the serum of patients with primary biliary cirrhosis that reacts with mitochondria-rich cells (mitochondrial antibody). These antibodies have been detected in over 95 percent of patients with primary biliary cirrhosis but only rarely in other form of liver disease. These observations suggest strongly that disordered immune responses play a major role in the initiation or progression of the chronic hepatic lesion of biliary cirrhosis, but the exact mechanisms involved are unclear.

Most instances of secondary biliary cirrhosis result from longstand­ing partial or total obstruction of the common bile duct or its major branches. In adults, chronic bile duct obstruction by postoperative stric­tures or by gallstones, usually with superimposed infectious cholangitis, is the most common cause of this type of biliary cirrhosis. Tumors of the pancreas, biliary free, or gallbladder that produce obstruction of the com­mon bile duct occasionally induce cirrhosis but only rarely permit sur­vival at this stage.

Although not diagnostic, the early clinical course of primary biliary cirrhosis is quite characteristic. Typically, the patient is a middle-aged woman who develops persistent generalized itching (the earliest symptom in about 50 percent of cases), dark urine, pale stools, and jaundice, with some darkening (melanosis) of the exposed areas of the skin. In contrast to many other forms of cirrhosis, there are few early signs of liver cell failure and hepatic fibrosis, and most of the features reflect impaired bile excretion. Steatorrhea with associated malabsorption of lipidsoluble vita­mins often produces purpura, diarrhea, and osteomalacia; the last may be manifested by backache and bone pain. Protracted elevation of serum lipids, especially cholesterol, leads to the deposit of yellowish plaques or nodules in the suhputaneous tissues in the form of periorbital xanthelas­mas and xanthomas over joints, in skin folds, and at sites of trauma. Over a period of months to years, the itching, jaundice, and hyperpigmentation slowly increase. At that time the pruritus and skin lipid deposits may decrease, ascites and edema usually appear, and signs of liver cell failure and portal hypertension supervene. Most patients die within 5 to 10 years from the first signs of the illness. Death usually is due to hepatic insuf­ficiency that is often precipitated by variceal bleeding, intercurrent infec­tion, or surgical procedures.

Physical examination may be entirely normal in the early phase of the disease when pruritus is the sole complaint. In addition, however, there may be jaundice of varying intensity, hyperpigmentation of the exposed skin areas, xanthelasmas and xanthomas, moderate to striking hepato­megaly, splenomegaly, and clubbing of the fingers. Fever and chills are rare and usually indicate mechanical biliary obstruction or other associ­ated diseases. Muscle wasting, spider angiomas, palmar erythema, ascites and edema, and the bony tenderness of osteomalacia appear in advanced stages of the diseases.

Patients who develop secondary biliary cirrhosis usually have a long­standing history and evidence of previous biliary tract disease. Pain varies in type. There may be right upper quadrant pain due to stretching of the liver capsule or due to disease in the gallbladder or bile duct. Signs and symptoms of true cirrhosis appear slowly; ascites and massive upper gastrointestinal hemorrhage usually occur terminally.