Jaundice

Description

• Yellow staining of tissues and body fluids due to hyperbilirubinemia, usually present at levels of >2.5 mg/dL
• Unconjugated (indirect) hyperbilirubinemia:
  • Direct breakdown product of hemoglobin
  • Water insoluble, circulates bound to albumin
  • Hemolytic:
    • Excessive production of unconjugated bilirubin
  • Hepatic:
    • Decreased hepatobiliary excretion of bilirubin
      • Defective uptake (drugs, Crigler–Najjar syndrome)
      • Defective conjugation (Gilbert syndrome, drugs)
      • Defective excretion of bilirubin by the liver cell (drugs, Dubin–Johnson syndrome)
• Conjugated (direct) hyperbilirubinemia:
  • Conjugated bilirubin is water soluble and measured as direct bilirubin
  • In conjugated hyperbilirubinemia, bilirubin is returned to the bloodstream after conjugation in the liver instead of draining into the bile ducts
  • Hepatocellular dysfunction:
    • Hepatitis
    • Cirrhosis
    • Tumor invasion
    • Toxic injury
  • Intrahepatic (nonobstructive) cholestasis
  • Extrahepatic (obstructive) cholestasis

Etiology

• Prehepatic:
  • Hemolysis (sickle cell, other hemoglobinopathies, G6PD deficiency)
  • Ineffective erythropoiesis
  • Drugs
  • Gilbert syndrome: Usually benign inherited form of unconjugated hyperbilirubinemia
  • Crigler–Najjar syndrome
  • Prolonged fasting
  • Cardiopulmonary bypass (ECMO)
  • Hematoma reabsorption
• Hepatocellular:
  • Hepatitis (infectious, alcoholic, autoimmune, toxin, drug induced)
  • Cirrhosis
  • Postischemic
  • Hemochromatosis
• Intrahepatic cholestasis:
  • Idiopathic cholestasis of pregnancy
  • Drugs (TPN, statins, etc.)
  • Dubin–Johnson syndrome
  • Rotor syndrome
  • Benign recurrent cholestasia
  • Familial syndromes
  • Sepsis
  • Postoperative jaundice
  • Lymphoma
• Extrahepatic obstruction:
  • Common duct stone
  • Biliary stricture
  • Bacterial cholangitis
  • Sclerosing cholangitis
  • Carcinoma (ampulla, gallbladder, pancreas), cholangiosarcoma
  • Pancreatitis, pancreatic pseudocyst
  • Hemobilia
  • Duodenal diverticula
  • Ascariasis
  • Postlaparoscopic cholecystectomy complications
  • Congenital biliary atresia
  • Congenital choledochal cyst

• Cardiovascular (congenital heart disease, congestive heart failure, shock, asphyxia)
• Metabolic or genetic (α₁-antitrypsin deficiency, trisomy 18 and 21, cystic fibrosis, Gaucher disease, Niemann–Pick disease, glycogen storage disease type IV)
• Infectious (bacterial sepsis, cytomegalovirus, enterovirus, herpes simplex virus, rubella, syphilis, TB, varicella, viral hepatitis)
• Hematologic (severe isoimmune hemolytic disease)