Chapter 19 Candice Quillin, BSN, CGRN

2 March 2011

1. Describe Anatomy and Physiology of normal liver

2. Name three (3) diseases or diagnosis of the liver and treatment for them.

Hepatocytes – secrete bile Sinusoid cavities are between rows of

hepatocytes Sinusoids are lined with Kupffer cells Kupffer cells remove amino acids,

nutrients, sugars, broken down RBC’s, bacteria, and debris from blood. Detoxifies the blood.

Secrete bile

Bile formation and secretion Metabolism of carbohydrates, proteins,

fats, and steroids Manufacturer of substances necessary

for coagulation and anticoagulation Detoxification of foreign and toxic

substances Vitamin and mineral storage

Bile is secreted into the bile canaliculi, which branch and combine to form the right and left hepatic ducts. The right and left hepatic ducts merge into the common hepatic duct. The cystic duct joins the common hepatic duct to form the common bile duct. The common bile duct joins the duct of Wirsung at the ampulla of vater. Bile then empties out into the duodenum.

Carbohydrate metabolism converts glucose, fructose, and galactose to glycogen for storage in the liver

Breaks down glycogen to glucose to maintain blood glucose levels when carbohydrate intake decreases

Synthesizes glucose from noncarbohydrate nutrients to maintain blood glucose levels.

Breaks down amino acids to produce ketoacids and ammonia.

Synthesizes proteins Hydrolyzes fats to glycerol and fatty acids. Metabolizes steroids, glucocorticosteroids,

estrogen, testosterone, progesterone, and aldosterone.

The liver produces and synthesizes most of the bodies clotting factors like prothrombin, fibrinogen, Factor 5,7,9, and 10.

Anti-coagulant heparin and vasopressor substances after hemorrhage.

Mechanisms of detoxification include: Reduction Hydrolysis Conjugation Oxidation Excretion Degradation

Phagocytosis of viruses, bacteria, dyes, and foreign proteins also occurs in detoxification.

Vitamins A, B, C, D, E, and K Minerals copper and iron.

A liver disease that is characterized pathologically by loss of the normal microscopic lobular architecture, with fibrosis and nodular regeneration.

1. Alcoholic cirrhosis Up to 50% of adult cases of cirrhosis are

alcohol related Liver is enlarged and there is altered lipid

metabolism causing fatty infiltration. Treatment includes abstinence, counseling,

and high-caloric nutrient dense diet.

2. Immune-related bile duct injury Primary Biliary Cirrhosis (PBC) - Inflammation of

the bile ducts of the liver, which eventually blocks the flow of bile. This obstruction damages liver cells and leads to cirrhosis.

Primary Sclerosing Cholangitis (PSC) – A rare and serious condition in which inflammation involves the entire biliary tract; often related to GI or biliary tract infection.

3. Postnecrotic cirrhosis May be related to hepatitis, infection,

metabolic liver disease, or hepatotoxins or industrial chemicals.

History symptoms of Cirrhosis1. Weight loss2. Anorexia3. Abdominal pain4. Red spider veins under skin5. Ascites6. Itching especially of hands and feet at first7. Bruising8. Jaundice9. Mental confusion10. Difficulty concentrating

Abnormally increased blood pressure in the portal venous system.

Signs and Symptoms include:1. Melena2. Hematoemesis3. Jaundice4. Peripheral edema5. Palmar erythemia6. Bleeding tendencies7. Fetor hepaticus – sweet and fetid breath odor8. Spleenomegaly9. Varicies10. Dilated abdominal veins 11. Spider nevi12. Venous patterns on flanks

The goal of treatment for portal hypertension is to divert blood flow around the liver and away from collateral vessels.

Treatment options include1. Portacaval shunt2. Splenorenal shunt3. Mesocaval shunt4. Transjugular intrahepatic portosystemic

shunt (TIPS)5. Vasoconstriction Medications:

Somatostatin, Octreotide, Nitroglycerin, Vasopressin, and Terlipressin.

The effusion and accumulation of serous fluid in the abdominal cavity

Causes: Portal hypertension, cancer, cardiac

failure, pancreatic causes, trauma

Treatment: Sodium restriction, diuretics, bed rest. Peritoneal jugular shunt Paracentesis.

A syndrome characterized by functional renal failure, oliguria, and low urinary sodium concentration, without pathological renal changes.

Associated with cirrhosis and ascites or with obstructive jaundice.

Management is to diagnosis, remove cause, treat factors known to cause the renal failure.

High calorie, low protein, low sodium diet. Fluid challenge may help. Dialysis Liver transplant

A condition usually occurring secondary to advanced liver disease.

Stage 1 – mild confusion, mood changes, sleep disturbance, mild acterixis(rapid wrist flap)

Stage 2 – confusion, apathy, obvious asterixis, personality changes, disorientation, apraxia.

Stage 3 – sever confusion, incoherence, hyperactive deep tendon reflexes, muscular rigidity

Stage 4 – No reaction to stimuli, no corneal reflex, dilated pupils, flexion or extension posture, coma.

Treatment may include correcting pH and electrolyte imbalances, restricting dietary protein, removing excess protein from gut, preventing constipation, and preventing GI bleed so that protein does not get in bowel to be absorbed.

Treatment can include medications like lactulose which causes osmotic diarrhea which will promote excretion of ammonia and proteins through GI tract.

Antibiotics such as Metronidazole, Xifaxan or Rifaximin may decrease the number of bacteria that breakdown amino acids.

Inflammation of liver See chart for description of each type,

Hepatitis A,B,C,D,E,F, and G.

Inflammation of liver due to alcohol consumption.

Symptoms include: RUQ abdominal pain, fever, vomiting, anorexia, and dark urine. Patients do not present with jaundice.

Signs include tender liver, spleenomegaly, signs of chronic alcohol abuse, cirrhosis, and mental status changes.

Treatment includes supportive care, counseling, abstinence from alcohol.

Drugs can cause hepatic injury including Dose-related hepatotoxic reaction

(Tylenol, methotrexate, carbon tetrachloride)

Non-dose related viral-like hepatitis(Isoniazid, Flurazepam, Methyldopa, I.V. tetracycline)

Cholestasis due to drugs like birth control pills.

Massive liver cell death Cause include poisoning, infectious

disease, ischemia, cyanotic heart, severe asphyxia, cardiomyopathy, shock, metabolic disease.

Sx’s – jaundice, flu like symptoms, fever, anorexia, vomiting, abdominal pain, confusion, coagulopathy, ascites, coma

Rare condition characterized by an obstruction of the hepatic venous outflow.

Causes include: malignancy, thrombogenic disorders, inflammation disorders, and hormone disorders.

Diagnostic imaging is used to confirm. Treatment is based on cause and

include: anticoagulation, angioplasty, TIPS procedure and liver transplant.

NAFLD is any liver condition arising without association of alcohol exposure.

Non-Alcoholic Steatohepatitis or NASH is stage 3 and 4 of NAFLD and is detected by ultrasound and diagnosed by liver biopsy. It is fat accumulation in the liver.

Risk factors – obesity, type 2 diabetes and hyperlipidemia.

Treatment for NASH can include weight loss and exercise.

Benign vs. malignant Most benign liver tumors are

hemangiomas which rarely require any form of treatment.

Hepatocellular carcinoma is the most common primary malignant tumor of the liver.

Risk factors for HCC include: HBV carriers, HBV cirrhosis, HCV cirrhosis, cirrhosis from hemochromatosis, PBC, cirrhosis from auto-immune hepatitis, PSC, or repeated dietary exposure to aflatoxin B1.

Autosomal-recessive disorder Inability to metabolize copper (Kayser-Fleischer ring) a pigmented ring

at the outer margin of the cornea is the most unique sign

Cirrhosis of the liver, liver necrosis, dementia, brain damage and kidney failure are among the advanced symptoms of the disease.

Any of a group of disturbances of porphyrin metabolism, characterized by marked increase in formation and excretion of porphyrins or their precursors. Acute intermittent porphyria (AIP)-autosomal dominant,

most do not develop symptoms, high intake of glucose or carbohydrates causes disease suppression. Monitor H2O and sodium intake during an attack

Hereditary coproporphyria (HCP)- characterized by large amounts of coproporphyrin III in the feces and lesser amounts in urine. Photosensitivity. Monitor H2O and sodium intake during an attack.

Variegate porphyria (VP)-autosomal dominant, characterized by large amounts of coproporphyrin in the feces and urine. Photosensitivity.

Porphyria cutanea tarda (PCT)-hereditary or acquired, characterized by chronic skin lesions especially on light exposed skin, increased hair growth, and mild liver disease with fatty infiltration, focal necrosis.

A disorder of iron metabolism characterized by excess deposition of iron in the tissues

Sx’s -bronze pigmentation of the skin, weakness, weight loss, malaise, joint pain, symptoms related to diabetes, loss of hair, congestive heart failure.

Primary – Inherited – recessive gene Secondary – Acquired during life

Diagnosis includes elevated serum ferritin, elevated serum iron, hereditary hemochromatosis DNA mutation analysis, MRI and liver biopsy.

Treatment includes: Therapeutic phlebotomy and Deferoxamin which binds with iron in the bloodstream and enhances its elimination via urine and feces.

Intrahepatic biliary dysplasia (IHBD) or Alagille syndrome is a rare autosomal-dominant liver disease that incorporates a combination of anomalies in conjunction with chronic cholestasis.

Biliary atresia - Rare condition in newborn infants in which extrahepatic duct is blocked or absent.

Kasai (hepatoportoenterostomy) procedure allows for excretion of bile from the liver into the intestine via a surgically created duct, not a cure.

Biliary atresia is the most common indication for liver transplant in infants.

Inflammation of the liver which occurs in early infancy. 20% due to viruses including CMV, rubella, and hepatitis A, B, or C.

Common and benign congenital disorder Characterized by elevation in

unconjugated bilirubin All other liver tests are normal Rarely develop jaundice

The severity of chronic liver disease is determined by the Model for End Stage Liver Disease (MELD) score. Based on serum bilirubin, INR, and creatinine.

UNOS has made modifications to the score

A quick reference site to calculate MELD scores and 90 day survival rates is http://www.mayoclinic.org/meld/mayomodel5.html

Liver Puzzle Cells remove amino acids, nutrients,

sugars, broken down RBC’s, bacteria, and debris from the blood.

Liver Puzzle Most vascular organ of the body.

Liver puzzle Secreted from the liver

Liver Puzzle Transmitted by the ingestion of

contaminated food.

Liver Puzzle Most common cause of Cirrhosis

Liver Puzzle Vascular lesion that is a presenting sign

of chronic liver disease

Liver Puzzle Rare condition characterized by an

obstruction of the hepatic venous outflow

Liver Puzzle Autosomal recessive trait in which pt

can not metabolize copper

Liver Puzzle Disorder of iron metabolism

Liver Puzzle Developmental bile duct disorder in

infants that results in obstruction of the bile flow through the extrahepatic duct system

Liver Puzzle Protein produced by the liver converted

by thrombin into fibrin during blood coagulation in presence of ionized calcium.

Liver Puzzle Study of the liver.