Ncm 30 Study Guide on the Child

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    NCM 30 STUDY GUIDE ON

    THE CHILD WITH GASTROINTESTINAL DYSFUNCTION

    CIRRHOSIS (p.1431 of Wong)

    a liver disease characterized by permanent scarring of the liver that

    interferes with its normal functions

    Occurs as an end stage of many chronic liver diseases, including biliary

    atresia and chronic hepatitis.

    This condition is irreversibly damaged.

    It affects about three million Americans a year.

    CAUSES

    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 cystic fibrosis

    Hepatitis B and C (African Americans)

    Bile duct disorders such as primary biliary cirrhosis and primary sclerosingcholangitis.

    Metabolic disorders such as hemachromatosis, Wilsons disease, and

    alpha-1 antitrypsin deficiency

    Others like Schistosomiasis,

    RISK FACTORS

    Obesity

    Genetic factors

    Moderate to heavy alcohol users.

    Co-infection with hepatitis B.

    Co-infection with HIV.

    Having large iron stores in the liver.

    nonalcoholic steatohepatitis (NASH)

    nonalcoholic fatty liver disease (NAFLD)

    Weight gain in the area of and abovethe waist (apple type) is more

    dangerous than weight gained aroundthe hips and flank area (pear type).

    Fat cells in the upper body have

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    different qualities than those found inhips and thighs.

    PATHOPHYSIOLOGY:

    COMPLICATIONS:

    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 occurs along with severecirrhosis

    Others:

    Kidney Failure

    Osteoporosis

    Insulin Resistance and Type 2 Diabetes.

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    Heart Problems.

    CLINICAL MANIFESTATION

    Cirrhosis is divided into two stages: Compensated and Decompensated.

    Compensated cirrhosis means that the body still functions fairly welldespite scarring of the liver. 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 vessels radiate. (upper torso)

    Decompensated cirrhosis means that the severe scarring of the liver hasdamaged and disrupted essential body functions. Patients withdecompensated cirrhosis develop many serious and life-threateningsymptoms and complications.

    Fluid buildup in the legs and feet (edema) and in the abdomen(ascites). (Ascites is associated with portal hypertension, which isdescribed in the Complications section of this report.)

    Jaundice. This yellowish cast to the skin and eyes occurs because theliver cannot process bilirubin for elimination from the body.

    Other Manifestations:

    Poor growth

    Muscle weakness

    Lethargy

    Impaired pulmonary function ( dyspnea and cyanosis during exertion)

    Intrapulmonary shunts (hypoxemia)

    Children (impaired intrahepatic blood flow)

    Ascites

    Edema

    GI bleeding

    Anemia

    Abdominal pain

    DIAGNOSTIC EXAMINATION

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    Past health history

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

    Laboratory evaluation

    Liver function tests:

    o Bilirubin

    o Aminotransferase

    o Ammonia

    o Albumin

    o Cholesterol

    o Prothrombin time

    Imaging Tests

    o Magnetic resonance imaging (MRI)

    o 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, especially hematocrit, is very important to check forany signs of hemorrhage or shock.

    Doopler ultrasonography of the liver and spleen ( to check for ascites)

    Therapeutic Management

    Monitor liver function and manage specific complications such as esophageal

    varices and malnutrition

    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 )

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    Limit the ammonia formation and absorption by administering neomycin and

    lactulose.

    Treatment of Underlying Conditions

    Treatment for cirrhosis depends on the cause of cirrhosis.

    Chronic Hepatitis. Many types of antiviral drugs are used to treat chronic hepatitisB, including pegylated interferon, nucleoside analogs, and nucleotide analogs.Patients with chronic hepatitis C are treated with combination therapy withpegylated interferon and 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 orUDCA, is used for treating primary biliary cirrhosis but does not slow theprogression. Itching is usually controlled with cholesterol drugs such ascholestyramine (Questran) and colestipol (Colestid). Antibiotics for infections in thebile ducts and drugs that quiet the immune system (prednisone, azathioprine,cyclosporine, methotrexate) may also be used. Several surgical procedures mayalso be tried to open up the bile ducts.

    Nonalcoholic Fatty Liver Disease (NAFLD) and NonalcoholicSteatohepatitis (NASH). Weight reduction through diet and exercise, anddiabetes and cholesterol management are the primary approaches to treating thesediseases. Investigators are also studying whether various drugs used to treat type 2diabetes may help treat NAFLD and NASH.

    Hemochromatosis. Hemachromatosis is treated with phlebotomy, a procedurethat involves removing about a pint of blood once or twice a week until iron levelsare normal.

    MEDICAL MANAGEMENT:

    Cirrhosis is an irreversible condition. Treatment focuses on slowing the

    progression of liver damage and reducing the risk of further complications. Yourdoctor will treat any underlying medical conditions that are the cause of yourcirrhosis. If liver damage progresses to liver failure, patients may be candidates forliver transplantation. Liver donations can come from either a cadaver or from aliving donor. Patients with cirrhosis who have a liver transplant have very goodchances for survival.

    LIVER TRANSPLANT

    Assess the childs degree of liver dysfunction to be evaluated for

    transplantation at the appropriate time.

    NURSING CARE MANAGEMENT:

    The goal of cirrhosis therapy is to remove or alleviate the underlying cause of

    cirrhosis, prevent further liver damage, and prevent or treat complications:

    Vitamins and nutritional supplements promote healing of damaged hepatic

    cells and improve the patients nutritional status.

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    Na consumption is usually restricted, and liquid intake is limited to or

    reduces to help manage ascites and edema.

    Drug therapy requires special caution detoxify harmful substances efficiently.

    Antacids may be prescribed to reduce gastric distress and decrease the

    potential for GI Bleeding.

    Alcohol is restricted.

    Sedatives should b avoided. Acetaminophen is especially hapatotoxic,

    particularly when combined with alcohol.

    To minimize the risk of bleeding, warn the patient against taking non-

    steroidal anti-inflammatory drugs, straining to defecate, and blowing his nose

    or sneezing too vigorously. Suggest using an electric razor and a soft

    toothbrush.

    Advise the patient to take adequate rest because it decreases the metabolic

    demands of the liver.

    Teach the patient to have small frequent meals. Teach him to alternate

    periods of rest and activity to reduce the oxygen demand and prevent

    fatigue.

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

    Emotional support for the family of the child (to reduce anxiety in

    preparation for liver transplantation or unexpected death)