Younger males

Primary sclerosing cholangitis

Larger intra- and extrahepatic ducts

Ulcerative colitis



Older females

Primary biliary cirrhosis

Smaller intrahepatic bile ducts

Autoimmune diseases such as rheumatoid arthritis

Antimitochondrial antibody (AMA)


Comprehension Questions

Match the following diagnoses (A-F) with the most likely clinical situation described in Questions [41.1] to [41.4).

A. Hemolysis

B. Alcoholic hepatitis

C. Gilbert disease

D. Pancreatic cancer

E. Gallstones

F. Primary sclerosing cholangitis

[41.1] A 38-year-old man with a 12 pack of beer per day alcohol history presents with jaundice, ascites, and dark urine. His laboratory results are AST 350 U/mL. ALT 150 U/mL. alkaline phosphatase 120 U/mL, total bilirubin 25 mg/dL, direct bilirubin 12 mg/dL, and albumin 2.1 g/dL.

[41.2] A 40-year-old moderately obese woman presents with abdominal pain after eating and mild scleral icterus. Her laboratory results are AST 200 U/L. ALT 150 U/L, alkaline phosphatase 355 U/L, total bilirubin 3.5 mg/dL. direct bilirubin 1.8 mg/dL, and albumin 3.5 g/dL.

[41.3] A 25-year-old man presents with 3 days of scleral icterus but has been otherwise feeling well. His laboratory results are AST 45 U/L, ALT 48 U/L, alkaline phosphatase 100 U/L. total bilirubin 4.0 mg/dL, direct bilirubin 0.2 mg/dL. and albumin 3.5 g/dL. Complete blood count is normal.

[41.4] A 32-year-old man with a 5-year history of episodic bloody diarrhea and abdominal cramping pain presents with scleral icterus and fever. His laboratory results are AST 100 U/L, ALT 125 U/L, alkaline phosphatase 550 U/L, total bilirubin 5.5 mg/dL, direct bilirubin 3.0 mg/dL, and albumin 2.9 g/dL.


[41.1] B. The patient's laboratory results show a conjugated hyperbilirubinemia with evidence of hepatocellular disease (hypoalbuminemia, ascites). The AST and ALT levels show the 2:1 ratio consistent with alcohol-related liver disease.

[41.2] E. The patient's laboratory results show a conjugated hyperbilirubinemia consistent with an obstructive pattern. She has the risk factors for gallstones (middle age, female, obese) and has symptoms of postprandial abdominal pain.

[41.3] C. The patient's laboratory results show an unconjugated hyperbilirubinemia without other abnormality. He is otherwise healthy without symptoms of systemic disease or anemia. No treatment is necessary.

|41.4| F. The patient's laboratory results show a conjugated hyperbilirubinemia with an obstructive pattern. The history is consistent with inflammatory bowel disease, which is associated with primary sclerosing cholangitis. The initial evaluation should include ultrasonography to rule out gallstones; if negative, ERCP could confirm the diagnosis by demonstrating multiple strictures of the extrahepatic bile ducts. Treatment options include stenting of the larger bile duct strictures and immunosuppression to slow the progression of the disease.

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