History
Patients with cholestasis may present clinically in many different ways depending on the disease process.
In most cases, scleral icterus is noted before any other sign; it 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.
In patients with cholestasis, another common presentation is severe pruritus secondary to elevated bile acids. At high concentrations (5 times the reference range), retained bile acids can cause severe pruritus in which patients are unable to sleep or concentrate and may resort to cutaneous mutilation for relief.
Physical
As noted above, the physical signs of cholestasis are usually scleral icterus or cutaneous jaundice, or both. These patients may have physical evidence of scratching or excoriation if they also have severe bile acid retention.
Xanthomas look like small white papules or plaques and are usually found on the trunk and diaper area and in areas of friction (eg, diaper line, creases of hands, elbows, neck).
Another important physical finding in patients with cholestasis may be evidence of failure to thrive with altered anthropometrics, such as reduced height and reduced weight for height due to fat malabsorption.
Causes
Causes include the following:
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Obstructive cholestasis
Biliary atresia
Congenital bile duct anomalies (choledochal cysts)
Infectious cholangitis (cholangitis)
Cholangitis associated with Langerhans cell histiocytosis
Alagille syndrome
Nonsyndromic ductal paucity
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Hepatocellular cholestasis
Hepatitis (hepatitis A, hepatitis B, hepatitis C)
Inborn errors of bile acid synthesis
Drug-induced cholestasis
Total parenteral nutrition (TPN)–associated cholestasis
Progressive familial intrahepatic cholestasis [10, 11, 12, 13, 14]
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Eruptive xanthomas. Courtesy of Duke University Medical Center.
