Liver: Hyperbilirubinemia

Hyperbilirubinemia

Icterus (Jaundice)

  • Yellow Visible Manifestation of Hyperbilirubinemia
  • Visible Once Bilirubin > 2.0-2.5 mg/dL
  • Sites
    • Mucous Membranes/Under Tongue (First Site)
    • Scleral Icterus
    • Skin

General Causes

  • Unconjugated Hyperbilirubinemia
    • Hemolysis
    • Extravasation
    • Bilirubin Overproduction
    • Hepatic Bilirubin Uptake Impairment
    • Hepatic Bilirubin Conjugation Impairment
  • Conjugated Hyperbilirubinemia
    • Biliary Obstruction
    • Cholangitis
    • Sepsis
    • Hepatitis/Cirrhosis
    • TPN/Drugs/Toxins
    • Malignancy

Scleral Icterus 1

Infant Hyperbilirubinemia

Increased Bilirubin Production – High Indirect Component

  • Breast Mild Jaundice
    • Benign Hyperbilirubinemia Persisting Beyond 2-3 Weeks
    • When Infant Only Given Human Breast Milk
    • Mechanism Uncertain
  • Lactation-Failure Jaundice
    • Cause: Lactation Failure Causing Hypovolemia & Weight Loss

Conjugation Deficiency – High Indirect Component

  • Benign Neonatal Hyperbilirubinemia (Physiologic Jaundice of Newborn)
    • Cause: Immature UDP-Glucuronosyltransferase
    • Normal Transition Seen in Newborn
    • Takes 14 Weeks to Reach Adult Levels
    • Tx: Phototherapy vs None
  • Gilbert’s Syndrome
    • Cause: Mild UDP-Glucuronosyltransferase Defect
      • Abnormal Conjugation
    • Most Common Inherited Bilirubin Metabolism Disorder
    • Tx: None
  • Crigler-Najjar Syndrome
    • Cause: Severe UDP-Glucuronosyltransferase Defect
      • Type I – No Conjugation
      • Type II – Very Low Activity
    • Tx: Lifelong Phototherapy; Liver Tx Curative

Excretion Deficiency – High Direct Component (Other LFT’s Normal)

  • Rotor’s Syndrome
    • Cause: Hepatic Storage Defect – Conjugated Bilirubin Diffuses into Plasma
    • Dx: High Urinary Coproporphyrin, < 70% Isomer I
    • Tx: None
  • Dubin-Johnson Syndrome
    • Cause: Hepatocyte Secretion Defect
    • Liver is Grossly Pigmented/Black
    • Dx: Normal Urinary Coproporphyrin, > 80% Isomer I
    • Tx: None

References

  1. Climans SA, Murphy JM, Evans GA, Shamseddin MK. Renal failure, hepatitis and myocarditis in a previously healthy man. Can J Infect Dis Med Microbiol. 2014 May;25(3):133-4. (License: CC BY-NC-4.0)