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    (p. 1431 of WONG)

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    a liver disease characterized by permanentscarring of the liver that interferes with itsnormal functions

    Occurs as an end stage of many chronic liverdiseases, including biliary atresia and chronichepatitis.

    This condition is irreversibly damaged.

    It affects about three million Americans a year.

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    Infection Autoimmune (0.6% to 2%) Toxic factors (Prolonged exposure to certain types

    of chemicals and medications like arsenic,methotrexate, toxic doses of vitamin A)

    Chronic diseases such as hemophilia and cysticfibrosis

    Hepatitis B and C (African Americans) Bile duct disorders such as primary biliary cirrhosis

    and primary sclerosing cholangitis. Metabolic disorders such as hemachromatosis,

    Wilsons disease, and alpha-1 antitrypsin deficiency Others like Schistosomiasis,

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    ple type) is more dangerous than weight gained around the hips and flank area (pear type). Fat cells in the upper body have diffe

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    Ascites (fluid buildup in the abdomen)

    Variceal hemorrhage, severe bleeding from varices(enlarged veins in the esophagus and upper stomach)

    Spontaneous bacterial peritonitis, a severe infection of

    the abdominal fluid Hepatic encephalopathy, damage to the brain caused

    by buildup in the body of toxins such as ammonia

    Hepatocellular carcinoma, a type of liver cancer

    Hepatorenal syndrome, when kidney failure occursalong with severe cirrhosis

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    Kidney Failure

    Osteoporosis

    Insulin Resistance and Type 2 Diabetes.

    Heart Problems.

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    Cirrhosis is divided into two stages: Compensatedand Decompensated.

    Compensated cirrhosis means that the bodystill functions fairly well despite scarring of theliver. Many people with compensated cirrhosisexperience few or no symptoms.

    Fatigue and loss of energy Loss of appetite and weight loss Nausea or abdominal pain Spider angiomas may develop on the skin. These are

    pinhead-sized red spots from which tiny blood vesselsradiate. (upper torso)

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    Decompensated cirrhosis means thatthe severe scarring of the liver hasdamaged and disrupted essential bodyfunctions. Patients with decompensatedcirrhosis develop many serious and life-threatening symptoms and complications.

    Fluid buildup in the legs and feet (edema) and inthe abdomen (ascites). (Ascites is associated withportal hypertension, which is described in theComplications section of this report.)

    Jaundice. This yellowish cast to the skin and eyesoccurs because the liver cannot process bilirubinfor elimination from the body.

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    Poor growth

    Muscle weakness

    Lethargy

    Impaired pulmonary function ( dyspnea and

    cyanosis during exertion)

    Intrapulmonary shunts (hypoxemia)

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    Ascites

    Edema

    GI bleeding

    Anemia

    Abdominal pain

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    Past health history

    Physical examination (firm, often enlarged and rock-hard) Laboratory evaluation

    Liver function tests: Bilirubin Aminotransferase Ammonia Albumin Cholesterol Prothrombin time

    Imaging Tests Magnetic resonance imaging (MRI) computed tomography (CT) scan Liver biopsy (Transjugular Liver Biopsy, Percutaneous Liver

    Biopsy and laparoscopy) ***liver biopsy can cause internal bleeding thats why

    monitoring vital signs and laboratory values, especiallyhematocrit, is very important to check for any signs ofhemorrhage or shock.

    Doopler ultrasonography of the liver and spleen ( to checkfor ascites)

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    Monitor liver function and manage specificcomplications such as esophageal varices andmalnutrition

    Nutritional support IV fluids Blood products Vasopressin Gastric lavage Balloon tamponade with a Sengstaken-Blakemore tube

    ( to control bleeding )

    Endoscopic sclerotherapy Endoscopic banding ligation Diuretics ( potassium sparring) Albumin administration or paracentesis ( for ascites ) Limit the ammonia formation and absorption by

    administering neomycin and lactulose.

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    Treatment for cirrhosis depends on the cause of cirrhosis.

    Chronic Hepatitis. Many types of antiviral drugs are used to treat chronic hepatitis B,including pegylated interferon, nucleoside analogs, and nucleotide analogs. Patientswith chronic hepatitis C are treated with combination therapy with pegylated interferonand ribavarin. [For more information, see In-Depth

    Autoimmune Hepatitis. Autoimmune hepatitis is treated with the corticosteroidprednisone and also sometimes immunosuppressants, such as azathioprine (Imuran).

    Bile Duct Disorders. Ursodeoxycholic acid (Actigall), also known as ursodiol or UDCA,is used for treating primary biliary cirrhosis but does not slow the progression. Itching isusually controlled with cholesterol drugs such as cholestyramine (Questran) andcolestipol (Colestid). Antibiotics for infections in the bile ducts and drugs that quiet theimmune system (prednisone, azathioprine, cyclosporine, methotrexate) may also beused. Several surgical procedures may also be tried to open up the bile ducts.

    Nonalcoholic Fatty Liver Disease (NAFLD) and Nonalcoholic Steatohepatitis(NASH). Weight reduction through diet and exercise, and diabetes and cholesterolmanagement are the primary approaches to treating these diseases. Investigators arealso studying whether various drugs used to treat type 2 diabetes may help treatNAFLD and NASH.

    Hemochromatosis. Hemachromatosis is treated with phlebotomy, a procedure thatinvolves removing about a pint of blood once or twice a week until iron levels arenormal.

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    The goal of cirrhosis therapy is to remove or alleviate the

    underlying cause of cirrhosis, prevent further liverdamage, and prevent or treat complications:

    Vitamins and nutritional supplements promote healing of damagedhepatic cells and improve the patients nutritional status.

    Na consumption is usually restricted, and liquid intake is limited toor reduces to help manage ascites and edema.

    Drug therapy requires special caution detoxify harmful substancesefficiently.

    Antacids may be prescribed to reduce gastric distress and decreasethe potential for GI Bleeding.

    Alcohol is restricted.

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    Sedatives should b avoided. Acetaminophen isespecially hapatotoxic, particularly when combinedwith alcohol.

    To minimize the the risk of bleeding, warn the patientagainst taking non-steroidal anti-inflammatory drugs,straining to defecate, and blowing his nose orsneezing too vigorously. Suggest using an electricrazor and a soft toothbrush.

    Advise the patient tot ake adequate rest because itdecreases the metabolic demands of the liver.

    Teach the patient to have small frequent meals.Teach him to alternate periods of rest and activity toreduce the oxygen demand and prevent fatigue.

    Tell the patient to avoid stress and to avoid exposureto infection.

    Emotional support for the family of the child (toreduce anxiety in preparation for liver transplantationor unexpected death)

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