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 sclerosing
cholangitis.

• 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 above

the waist (apple type) is more
dangerous than weight gained around
the hips and flank area (pear type).
Fat cells in the upper body have
different qualities than those found in
hips 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 severe
cirrhosis

Others:

• Kidney Failure
• Osteoporosis
• Insulin Resistance and Type 2 Diabetes.
 • Heart Problems.

CLINICAL MANIFESTATION

Cirrhosis is divided into two stages: Compensated and Decompensated.

• Compensated cirrhosis means that the body still functions fairly well
despite scarring of the liver. Many people with compensated cirrhosis
experience 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 has
damaged and disrupted essential body functions. Patients with
decompensated cirrhosis develop many serious and life-threatening
symptoms and complications.

• Fluid buildup in the legs and feet (edema) and in the abdomen
(ascites). (Ascites is associated with portal hypertension, which is
described in the Complications section of this report.)
• Jaundice. This yellowish cast to the skin and eyes occurs because the
liver 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
 • 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 that’s why monitoring vital signs
and laboratory values, especially hematocrit, is very important to check for
any 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 )

 • 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 hepatitis
B, including pegylated interferon, nucleoside analogs, and nucleotide analogs.
Patients with chronic hepatitis C are treated with combination therapy with
pegylated interferon and ribavarin. [For more information, see In-Depth

Autoimmune Hepatitis. Autoimmune hepatitis is treated with the corticosteroid

prednisone 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 is usually controlled with cholesterol drugs such as
cholestyramine (Questran) and colestipol (Colestid). Antibiotics for infections in the
bile ducts and drugs that quiet the immune system (prednisone, azathioprine,
cyclosporine, methotrexate) may also be used. 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 cholesterol management are the primary approaches to treating these
diseases. Investigators are also studying whether various drugs used to treat type 2
diabetes may help treat NAFLD and NASH.

Hemochromatosis. Hemachromatosis is treated with phlebotomy, a procedure

that involves removing about a pint of blood once or twice a week until iron levels
are normal.

MEDICAL MANAGEMENT:

Cirrhosis is an irreversible condition. Treatment focuses on slowing the

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

 LIVER TRANSPLANT

• Assess the child’s 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 patient’s nutritional status.
• 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)