Jaundice in Children

Background

• Jaundice is a yellow or yellow-greenish discoloration of skin, sclera, and mucous membranes due to hyperbilirubinemia (total serum bilirubin > 17 mcmol/L [1 mg/dL]) from excess bilirubin production or reduced elimination.
• There are 2 types of hyperbilirubinemia:
  • unconjugated or indirect hyperbilirubinemia - increased total serum bilirubin with < 15% conjugated or direct bilirubin
  • conjugated or direct hyperbilirubinemia - increased total serum bilirubin with > 20% conjugated or direct bilirubin
• Jaundice due to unconjugated hyperbilirubinemia can be from increased bilirubin production, impaired conjugation/excretion, or impaired uptake.
• Jaundice due to conjugated hyperbilirubinemia can be from intrahepatic cellular damage/cholestasis, extrahepatic cholestasis, genetic disorders of bilirubin metabolism, vascular liver disease, or total parenteral nutrition.
• Complications of hyperbilirubinemia may include kernicterus and intractable pruritus.
• The prognosis depends on the underlying cause of the jaundice.

Evaluation

• Obtain a compete history including medication, past, family, and social histories and perform a thorough physical examination focusing on the skin, abdominal, and neurologic exams.
• Evaluate all children with jaundice for signs of acute liver failure such as altered mental status, tachycardia, hypotension, and abdominal distention.
• Initial laboratory assessment of a child presenting with jaundice must begin with the measurement of total serum bilirubin with fractionation to assess for hyperbilirubinemia.
• The initial evaluation of unconjugated hyperbilirubinemia should include
  • complete blood count
  • reticulocyte count
  • peripheral blood smear
  • serum markers of hemolysis
  • additional testing as needed to identify the underlying cause.
• The initial evaluation of conjugated hyperbilirubinemia should include
  • complete metabolic profile
  • complete blood count
  • prothrombin time/INR
  • additional testing as needed to identify the underlying cause

Management

• Perform emergency management on any jaundice patient with signs and symptoms of liver failure such as encephalopathy, coma, hypoglycemia, or coagulopathy.
• The initial management of liver disease may include treating hypoglycemia, addressing coagulopathy, and providing nutritional support.
• Cause-specific management should be undertaken in any patient with unconjugated or conjugated hyperbilirubinemia.
• Consult a pediatric hepatologist for any child with jaundice exhibiting severe liver disease or failure.