Skip to Main Content

Jaundice

For further information, see CMDT Part 16-01: Jaundice & Evaluation of Abnormal Liver Biochemical Tests

Key Features

Essentials of Diagnosis

  • Jaundice results from accumulation of bilirubin in body tissues; the cause may be hepatic or nonhepatic (Table 16–1)

  • Hyperbilirubinemia may be due to abnormalities in the formation, transport, metabolism, or excretion of bilirubin (Table 16–2)

  • Persistent mild elevations of the aminotransferase levels are common in clinical practice and caused most often by nonalcoholic fatty liver disease

  • Evaluation of obstructive jaundice begins with ultrasonography and is usually followed by cholangiography

General Considerations

  • May be caused by predominantly unconjugated or conjugated bilirubin in the serum (Table 16–1)

  • In the absence of liver disease, hemolysis rarely elevates the serum bilirubin level to more than 7 mg/dL (119.7 mcmol/L)

  • "Cholestasis" denotes retention of bile in the liver, and "cholestatic jaundice" implies conjugated hyperbilirubinemia from impaired bile flow

  • Mediators of pruritus due to cholestasis have been identified to be

    • Lysophosphatidic acid (LPA)

    • Autotaxin, the enzyme that forms LPA

    • Mas-related G-protein coupled receptor X4

Table 16–1.Classification of jaundice.
View Table||Download (.pdf)
Table 16–1. Classification of jaundice.
Type of Hyperbilirubinemia Location and Cause
Unconjugated hyperbilirubinemia (predominantly indirect bilirubin)

Increased bilirubin production (eg, hemolytic anemias, hemolytic reactions, hematoma, pulmonary infarction)

Impaired bilirubin uptake and storage (eg, posthepatitis hyperbilirubinemia, Gilbert syndrome, Crigler-Najjar syndrome, drug reactions)
Conjugated hyperbilirubinemia (predominantly direct bilirubin) Hereditary Cholestatic Syndromes (see also Table 16–2)
Faulty excretion of bilirubin conjugates (eg, Dubin-Johnson syndrome, Rotor syndrome) or pathogenic variant in genes coding for bile salt transport proteins (eg, progressive familial intrahepatic cholestasis syndromes, benign recurrent intrahepatic cholestasis, and some cases of intrahepatic cholestasis of pregnancy)
Hepatocellular Dysfunction

Biliary epithelial and hepatocyte damage (eg, hepatitis, hepatic cirrhosis)

Intrahepatic cholestasis (eg, certain drugs, biliary cirrhosis, sepsis, postoperative jaundice)

Hepatocellular damage or intrahepatic cholestasis resulting from miscellaneous causes (eg, spirochetal infections, infectious mononucleosis, cholangitis, sarcoidosis, lymphomas, hyperthyroidism, industrial toxins)
Biliary Obstruction
Choledocholithiasis, biliary atresia, carcinoma of biliary duct, sclerosing cholangitis, IgG4-related cholangitis, ischemic cholangiopathy, COVID cholangiopathy, choledochal cyst, external pressure on bile duct, pancreatitis, pancreatic neoplasms

Ig, immunoglobulin.

Table 16–2.Hyperbilirubinemic disorders.
View Table||Download (.pdf)
Table 16–2. Hyperbilirubinemic disorders.
  Nature of Defect Type of Hyperbilirubinemia Clinical and Pathologic Characteristics
Gilbert syndrome1 Reduced activity of uridine diphosphate glucuronyl transferase Unconjugated (indirect) bilirubin Benign, asymptomatic hereditary jaundice. Hyperbilirubinemia increased by 24- to 36-hour fast. No treatment required. Associated with reduced mortality from CVD.
Dubin-Johnson syndrome2 Reduced excretory function of hepatocytes Conjugated (direct) bilirubin Benign, asymptomatic hereditary jaundice. Gallbladder does not visualize on oral cholecystography. Liver darkly pigmented on gross examination. Biopsy shows centrilobular brown pigment. Prognosis excellent.
Rotor syndrome3 Reduced hepatic reuptake of bilirubin conjugates Conjugated (direct) bilirubin Similar to Dubin-Johnson syndrome, but the liver is not ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.

This site uses cookies to provide, maintain and improve your experience. MH Privacy Center Close