Cirrhosis

Cirrhosis is a topic covered in the 5-Minute Pediatric Consult.

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Basics

Description

  • Cirrhosis is the end stage of progressive hepatic necrosis, fibrosis, and regenerative nodule formation that may occur as a result of many different liver diseases.
  • Results in distortion of liver architecture and compression of hepatic vascular and biliary structures
  • Cirrhosis is clinically defined by outcomes as either compensated or decompensated:
    • Compensated cirrhosis: typically asymptomatic with normal synthetic function
    • Decompensated cirrhosis: defined as loss of normal synthetic function, development of jaundice, and/or the clinical complications of portal hypertension such as ascites, variceal hemorrhage, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and portopulmonary hypertension
  • In its decompensated form/advanced form, cirrhosis is irreversible and often requires liver transplantation for survival of the patient.

Epidemiology

  • There are varying causes of cirrhosis; accordingly, no specific epidemiologic pattern can be identified.
  • Cirrhosis due to chronic hepatitis C virus (HCV) infection, alcoholic liver disease, and nonalcoholic fatty liver disease (NAFLD) are the most common indications for liver transplantation in adults.
  • Biliary cirrhosis due to biliary atresia is the most common indication for liver transplantation in children.

Risk Factors

Genetics

  • Many distinct genetic disorders can cause cirrhosis, such as Wilson disease and hereditary hemochromatosis.
  • Human leukocyte antigen (HLA) associations have been identified in several autoimmune disorders, including sclerosing cholangitis and autoimmune hepatitis.

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