Jaundice

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Basics

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

Pediatric Considerations
Intrahepatic cholestasis:
  • 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)

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Basics

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

Pediatric Considerations
Intrahepatic cholestasis:
  • 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)

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