Atlas of pathophysiology, 2 Edition

Part II - Disorders

Gastrointestinal Disorders

Cirrhosis

Cirrhosis is a chronic disease characterized by diffuse destruction and fibrotic regeneration of hepatic cells. Mortality is high; many patients die within 5 years of onset. As cirrhosis progresses, complications may occur; these include ascites, portal hypertension, jaundice, coagulopathy, hepatic encephalopathy, bleeding, esophageal varices, acute GI bleeding, liver failure, and renal failure.

Age Alert

Cirrhosis is especially prevalent among malnourished persons over age 50 with chronic alcoholism; it's also twice as common in men as in women.

Causes

·   Hepatitis

·   Alcoholism

·   Malnutrition

·   Autoimmune disease, such as sarcoidosis or chronic inflammatory bowel disease

·   Diseases of the biliary tree

·   Sclerosing cholangitis

·   Wilson's disease

·   Alpha1 antitrypsin deficiency

·   Hemochromatosis

·   Hepatic vein obstruction

·   Right-sided heart failure

Pathophysiology

The initial event in cirrhosis is hepatic scarring or fibrosis. The scar begins as an increase in extracellular matrix components—fibrin-forming collagens, proteoglycans, fibronectin, and hyaluronic acid. Hepatocyte function is eventually impaired as the matrix changes. Fat-storing cells are believed to be the source of the extracellular changes. Contraction of these cells may also contribute to disruption of the lobular architecture and obstruction of the flow of blood or bile. Cellular changes producing bands of scar tissue also disrupt the lobular structure.

As the disease progresses, portal hypertension develops and leads to esophageal varices. Ascites, hepatic encephalopathy, jaundice, and coagulation disorders develop as the body progresses to liver failure, renal failure, and death.

Signs and symptoms

Early stage

·   Anorexia; nausea and vomiting; diarrhea

·   Dull abdominal ache

Late stage

·   Respiratory—pleural effusion, limited thoracic expansion, impaired gas exchange

·   Central nervous system—progressive signs or symptoms of hepatic encephalopathy, including lethargy, extreme obtundation, coma

·   Hematologic—bleeding tendency, anemia, splenomegaly, portal hypertension

·   Endocrine—testicular atrophy, menstrual irregularities, gynecomastia, loss of chest and axillary hair

·   Skin—severe pruritus, extreme dryness and poor tissue turgor, spider angiomas, palmar erythema

·   Hepatic—jaundice, hepatomegaly, ascites and edema of the legs, hepatorenal syndrome

·   Miscellaneous—musty breath, enlarged superficial abdominal veins, pain in the right upper abdominal quadrant that worsens when patient sits up or leans forward, temperature of 101 to 103 F (38 to 39 C)

·   Hemorrhage from esophageal varices

Diagnostic test results

·   Liver biopsy reveals tissue destruction and fibrosis.

·   Abdominal X-ray shows enlarged liver, cysts, or gas within the biliary tract or liver, liver calcification, and massive fluid accumulation (ascites).

·   Computed tomography and liver scans show liver size, abnormal masses, and hepatic blood flow and obstruction.

·   Esophagogastroduodenoscopy reveals bleeding esophageal varices, stomach irritation or ulceration, or duodenal bleeding and irritation.

·   Blood studies reveal elevated levels of liver enzymes, total serum bilirubin, and indirect bilirubin; decreased levels of total serum albumin and protein; prolonged prothrombin time; decreased hemoglobin level, hematocrit, and serum electrolytes; and deficiency of vitamins A, C, and K.

·   Urine studies show increased bilirubin and urobilirubinogen level.

·   Fecal studies show decreased fecal urobilirubinogen level.

Treatment

·   Vitamins and nutritional supplements

·   Lactulose

·   Antacids

·   Potassium-sparing diuretics, loop diuretics

·   Vasopressin

·   Vitamin K supplementation

·   Blood products

·   Beta-adrenergic blockers

·   Esophagogastric intubation with multilumen tubes to control bleeding from esophageal varices or other hemorrhage sites

·   Gastric lavage

·   Esophageal balloon tamponade

·   Paracentesis

·   Surgical shunt placement

·   Sclerosing agents

·   Insertion of portosystemic shunts

·   Liver transplantation

P.165

CIRRHOTIC CHANGES

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