- Chronic progressive liver disease characterized by replacement of liver tissue with fibrosis, scar tissue, and regenerative nodules, resulting in progressive liver deformation and decreased function
- Signs & symptoms - ascites (most common), spider hemangiomata, palmar erythema, gynecomastia, hypogonadism, splenomegaly, hepatic encephalopathy
Define nonalcoholic fatty liver disease (NAFLD).
- Group of disorders characterized by hepatic steatosis (fat buildup in liver), not associated with other diseases, disorders, or hepatotoxins
- Accumulation of fat can lead to inflammation and scarring called nonalcoholic steatohepatitis (NASH)
- Risk factors - obesity, diabetes, hypertriglyceridemia, severe weight loss; poor diet, tuberculosis, intestinal bypass, and corticosteroids can lead to NAFDL
- Labs - persistently elevated ALT levels with no other cause
Define primary biliary cirrhosis.
- Chronic inflammatory condition of liver
- T-cell mediated attack of small bile duct epithelial cells resulting in loss of bile ducts and cholestasis, leading to liver necrosis and cirrhosis
- Increased risk for hepatocellular cancer
- 95% of patients are female
- Associated with autoimmune disorders including rheumatoid arthritis, Sjogren's, and scleroderma
- Early symptoms - fatigue and pruritis; hypoprothrombinemia related bleeding may occur
- Late sign - jaundice
- Other signs - fat malabsorption and low levels of fat-soluble vitamins r/t decreased bile secretion; xanthomas, hyperlipidemia, anemia
- Treatment goals - suppress ongoing liver damage, prevent complications, symptom management; med = ursodiol (Actigall)
Define Wilson's disease.
- Autosomal recessive - progressive, familial, terminal neurologic disease accompanied by chronic liver disease leading to cirrhosis
- Copper storage - decreased hepatocellular excretion of copper into bile, increased hepatic copper accumulation and cell injury
- Hallmark sign - Kayser-Fleischer rings, brownish red colored rings seen in the cornea near limbus on eye exam
- Other neurologic dysfunctions include tremor, involuntary movements, drooling, dysarthria, rigid dystonia, seizures, migraine headaches, and insomnia
- Labs - elevated serum ALT & AST, low serum ceruloplasmin, low serum uric acid, elevated urinary copper
- Treatment - chelating agents such as D-penicillamine or trientine (Syprine), promote excretion of urinary copper
Define hemochromatosis (HH).
- Iron disorder - increased and inappropriate storage of dietary iron
- Systemic disease, affects liver, heart, pancreas, endocrine system
- Progressive, slow disease
- Major causes of death - decompensated cirrhosis, hepatocellular carcinoma, diabetes mellitus, cardiomyopathy
Four types of cirrhosis.
- Alcoholic (a.k.a. portal or nutritional) cirrhosis: Fatty deposits in liver due to excess alcohol intake; with continued alcohol abuse, widespread scar formation
- Postnecrotic cirrhosis: Complications of viral, toxic, or idiopathic (autoimmune) hepatitis; broad bands of scar tissue formation
- Biliary cirrhosis: Chronic biliary obstruction and infection leading to diffuse fibrosis; jaundice is main feature
- Cardiac cirrhosis: Longstanding, severe right-sided heart failure causing chronic hepatic congestion, resulting in fibrosis
Early manifestations of cirrhosis.
- Usually insidious onset
- Liver has altered ability to metabolize carbs, fats, and proteins
- Abdominal pain - dull, heavy feeling in RUQ or epigastrium
- GI disturbances are common - Anorexia, dyspepsia, flatulence, nausea and vomiting, and changes in bowel habits (diarrhea or constipation)
- Other symptoms - Fever, lassitude, slight weight loss, hepatosplenomegaly (enlarged liver & spleen)
Later manifestations of cirrhosis.
- Symptoms may be severe r/t liver failure and portal hypertension
- Jaundice, peripheral edema, and ascites
- Advanced stage, liver becomes small and nodular
- Jaundice - liver unable to conjugate and excrete bilirubin (hepatocellular jaundice)
- Pruritis - bile salts accumulate under skin
- Skin lesions - spider angiomas (nose, cheek, upper trunk, neck, shoulders) and palmar erythema; r/t liver's decreased ability to metabolize estrogen
- Thrombocytopenia, leukopenia, and anemia - splenomegaly (caused by backup of blood from portal vein into spleen) causes overactive spleen, increasing removal of blood cells from circulation
- Bleeding disorders - liver incapable of producing prothrombin and other clotting factors, causing decreased coagulation ability
- Endocrine - liver incapable of metabolizing adrenocortical hormones, estrogen, and testosterone; in men, gynecomastia, alopecia (axillary/pubic), testicular atrophy, loss of libido; in women, amenorrhea in menopausal women or vaginal bleeding in postmenopausal women; hyperaldosteronism causes sodium and water retention and potassium loss
- Lab values - high sodium, low potassium
- Peripheral neuropathy - dietary deficiency in thiamine, folic acid, and cobalamin
Complications of cirrhosis.
Portal hypertension resulting in esophageal varices, peripheral edema and ascites, hepatic encephalopathy (coma), and hepatorenal syndrome.
Define portal hypertension.
- Hypertension in the portal vein and its tributaries (veins from intestines to liver)
- Symptoms caused by blood being forced down alternate channels rather than into the portal system
- Symptoms include ascites, hepatic encephalopathy, bacterial peritonitis, hepatorenal syndrome, splenomegaly, caput medusae (distended paraumbilical veins), and esophageal, gastric, and anorectal varices
Define esophageal varices.
- Distention of esophageal veins; bleed easily
- Most life-threatening complication of cirrhosis
- Risk factors - ingesting poorly masticated or coarse foods or alcohol, acid reflux, increased pressure r/t nausea, vomiting, straining at stool, coughing, sneezing, lifting heavy object
Treatment of esophageal varices.
- No aspirin, alcohol, or irritating foods; chew food 28 times or eat mechanical soft diet
- Avoid coughing, treat URI
- Prophylactic propanolol (Inderal) - lowers portal hypertension, prevents recurrent GI bleed
- Endoscopic sclerotherapy - endoscopic injection of sclerosing agent (e.g., morrhuate/Scleromate) into veins; NPO, conscious sedation; post procedure, check gag reflex, bleeding
- Balloon tamponade - tube-ballon down esophagus, inflate to put pressure against esophageal wall; keep scissors in reach if ballon slips to grab, cut, and pull tube to remove airway blockage
- Banding/ligation - tie off vessels to stop bleeding
- Shunt - shunting blood away from liver reduces pressure in esophageal veins
- Fresh frozen plasma and packed RBCs - for blood loss
- Vitamin K - blood clotting
- H2 histamine blockers and proton pump inhibitors - reduce reflux, reduce acidity and irritation
- Vasopresin (Pitressin) - potent vasoconstrictor
- Octreotide (Sandostatin) - decreases bleeding and pressure
- Nitroglycerine - vasodilator, lowers blood pressure, acts on heart to dilate heart arteries
- Neomycin and Lactulose - decease bacterial flora in the colon, which decreases ammonia production
Transjugular intrahepatic portosystemic shunt (TIPS).
- A&P: Some of the esophageal veins drain into the portal vein (esophageal veins -> left gastric vein -> portal vein)
- Problem: Hepatitis causes blood to congest in liver, back up into portal vein and cause venous distention and esophageal varices
- Solution: Create a bypass from the portal vein to the hepatic vein so that some blood is routed past the liver, relieving pressure in portal vein
- How: Using the jugular vein as an entrance, a shunt is guided down the superior to inferior vena cava and into the hepatic vein, punctured out the hepatic vein, and directed towards and anchored into the portal vein
- Contraindications: Severe hepatic encephalopathy, hepatic carcinoma, and portal vein thrombosis
- Complications: Increased levels of serum ammonia and resultant encephalopathy (ammonia is filtered by the liver, bypassing liver decreases filtering)
Hepatic encephalopathy - define and treat.
- Define: Neuropsychiatric manifestation of liver damage, caused by excess ammonia in systemic circulation (liver filters ammonia)
- How: Disorder of protein metabolism and excretion with nitrogenous
- Problem: Considered terminal complication of liver disease
- "Stop a Bus" Test: Put hands straight out, palms out, like hand signal to stop a bus, if high ammonia, hands will flap
- Nursing considerations: Seizure precautions, suction in room, lactulose for life (3-4x daily), neomycin to bring down bacterial load in gut
- Resolution: When ammonia levels decrease, mental state clears
Ascites - define and treat.
- Define: Accumulation of serous fluid in the peritoneal or abdominal cavity causing increased weight, increased girth, striae, dehydration, decreased potassium, and decreased urine output
- How: Proteins move from blood vessels into capillaries and into lymph space; lymphatic system unable to carry off excess proteins and water; osmotic pressure of proteins pulls additional fluid into peritoneal cavity
- Treat: Paracentesis, shunting, salt restriction, diuretics, water restriction
Fulminant hepatic failure - define and treat.
- Define: Acute liver failure, severe impairment of liver function associated with hepatic encephalopathy
- Cause: Most commonly drug use, usually acetaminophen with alcohol; second most common is HBV; also mushroom poisoning ("death cap" mushroom)
- How: Drug disrupts essential intracellular processes or causes buildup of toxic metabolic products
- Treat: Liver transplant
- Nursing Considerations: Frequent mental status checks for decline in LOC; quiet environment to minimize agitation; seizure precautions; I&O for renal function; oral and skin care to avoid breakdown and infection; monitor renal function, glucose, F&E; watch for increased ICP; HOB at 30 degrees
- Metastatic carcinoma of the liver is more common than primary carcinoma
- 80% of liver cancer patients also have cirrhosis of liver
- Hep C responsible for 50-60% of liver cancers
- Hep B responsible for 20% of liver cancers
- Clinical manifestation similar to cirrhosis
- Liver biopsy is definitive test
- Management similar to cirrhosis; lobectomy or liver transplant
- If no signs of metastasis, treatment options may include radiofrequency ablation, cryoablation, percutaneous ethanol injection or percutaneous acetic acid injection
- Prognosis is poor; cancer grows rapidly, death within 4-7 months from hepatic encephalopathy or massive blood loss from GI bleed