Neonatal Cholestasis

Posted by e-Medical PPT Wednesday, July 28, 2010
Neonatal cholestasis is defined as conjugated hyperbilirubinemia developing within the first 90 days of extrauterine life.
Conjugated bilirubin exceeds 1.5 to 2.0 mg/dl or
Conjugated bilirubin generally exceeds 20% of the total bilirubin.
Neonatal cholestasis can be broadly classified into hepatocellular, where an impairment of bile formation occurs, and obstructive, where impedance to bile flow occurs after it is formed.

Obstructive cholestasis
  • Biliary atresia
  • Congenital bile duct anomalies (choledochal cysts)
  • Cholelithiasis
  • Primary sclerosing cholangitis
  • Infectious cholangitis (cholangitis)
  • Alagille syndrome
Hepatocellular cholestasis
  • Hepatitis (hepatitis A, hepatitis B, hepatitis C)
  • Alpha1-antitrypsin deficiency
  • Inborn errors of bile acid synthesis
  • Drug-induced cholestasis
  • Total parenteral nutrition (TPN)–associated cholestasis
  • Progressive familial intrahepatic cholestasis
  • Caroli’s disease
Patients with cholestasis may present clinically in many different ways depending on the disease process.
Scleral icterus may be apparent at conjugated bilirubin levels as low as 2 mg/dL
At higher levels of conjugated bilirubin, dark urine may be noted secondary to the filtering of bilirubin into the urine.
Cutaneous jaundice may not be noted until bilirubin levels reach 5 mg/dL or higher.
severe pruritus secondary to elevated bile acids.

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