Liver and Biliary Pathology Practice Questions

Q: Grossly pigmented liver is seen in which syndrome?

Dubin-Johnson syndrome. **Explanation:** The hallmark of **Dubin-Johnson Syndrome (DJS)** is a **grossly black or darkly pigmented liver** [1]. This occurs due to a mutation in the **MRP2 gene**, which encodes a canalicular multidrug resistance-associated protein responsible for the transport of conjugated bilirubin and other organic anions into the bile [1]. The failure of this transporter leads to the accumulation of epinephrine metabolites (melanin-like polymers) within lysosomes of hepatocytes, giving the liver its characteristic dark appearance. **Analysis of Options:** * **Dubin-Johnson Syndrome (Correct):** Characterized by conjugated hyperbilirubinemia, normal total bile acid levels, and a dark/black liver [1]. A key diagnostic feature is the altered urinary coproporphyrin excretion pattern (80% is Coproporphyrin I). * **Rotor Syndrome:** Similar to DJS (conjugated hyperbilirubinemia) but lacks liver pigmentation [1]. It is caused by defects in OATP1B1 and OATP1B3 transporters. The liver appears grossly normal. * **Gilbert Syndrome:** The most common hereditary hyperbilirubinemia, caused by reduced activity of **UGT1A1** [1]. It results in mild **unconjugated** hyperbilirubinemia; the liver is structurally and grossly normal. * **Crigler-Najjar Syndrome:** Caused by a total (Type I) or near-total (Type II) absence of UGT1A1 [1]. It leads to severe **unconjugated** hyperbilirubinemia. The liver is not pigmented. **High-Yield NEET-PG Pearls:** * **DJS vs. Rotor:** DJS has a black liver and gallbladder is usually not visualized on oral cholecystography. Rotor has a normal liver and the gallbladder is visualized. * **Biopsy:** In DJS, biopsy shows coarse, iron-negative, dark brown granules in hepatocytes [1]. * **Trigger:** Jaundice in Gilbert syndrome is typically triggered by stress, fasting, or infection. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Liver and Gallbladder, p. 860.

Question 1

Which of the following hereditary hyperbilirubinemia syndromes is most fatal?

Accepted Answer

Crigler-Najjar syndrome type I

Answer

Dubin Johnson syndrome

Answer

Rotor syndrome

Answer

Crigler-Najjar syndrome type II

Question 2

Which of the following is characteristic of alcoholic liver disease?

Accepted Answer

Mallory hyaline

Answer

Perivenular fibrosis

Answer

Spotty necrosis

Answer

Zonal necrosis

Question 3

Defects in the enterohepatic circulation of bile acids due to altered canalicular function are seen in which of the following conditions?

Accepted Answer

Primary biliary cirrhosis

Answer

Cirrhosis

Answer

Gilbert's syndrome

Answer

Dubin-Johnson syndrome

Question 4

A 22-year-old female has a congenital anemia that required multiple transfusions of red blood cells for many years. She now has no significant findings on physical examination, but her liver function test results are abnormal. Which of the following findings would most likely appear in a liver biopsy?

Accepted Answer

Hemosiderin in hepatocytes

Answer

Steatosis in hepatocytes

Answer

Bilirubin in canaliculi

Answer

Glycogen in hepatocytes

Question 5

All of the following increase the risk for cholangiocarcinoma except?

Accepted Answer

Gall stones in the common bile duct

Answer

Ulcerative colitis

Answer

Sclerosing cholangitis

Answer

Chlonorchis

Question 6

Grossly pigmented liver is seen in which syndrome?

Accepted Answer

Dubin-Johnson syndrome

Answer

Crigler-Najjar syndrome

Answer

Gilbert syndrome

Answer

Rotor syndrome

Question 7

A mononuclear portal inflammatory infiltrate that disrupts the limiting plate and surrounds individual hepatocytes (piecemeal necrosis) is characteristic of which of the following conditions?

Accepted Answer

Chronic active hepatitis

Answer

Ascending cholangitis

Answer

Acute alcoholic hepatitis

Answer

Cholestatic jaundice

Question 8

Which of the following organ systems is least commonly involved in hemochromatosis?

Accepted Answer

Eye

Answer

Liver

Answer

Skin

Answer

Joints

Question 9

All of the following are features of alpha-1 antitrypsin deficiency except?

Accepted Answer

Autosomal dominant inheritance

Answer

Mutation in the SERPINA1 gene

Answer

Emphysema with cirrhosis can be seen

Answer

Alpha-1 antitrypsin is a serine protease inhibitor

Question 10

Which of the following is associated with primary hepatic malignancy?

Accepted Answer

Vinyl chloride

Answer

Cadmium

Answer

Chromium compounds

Answer

Asbestos

Liver and Biliary Pathology — MCQs

Liver and Biliary Pathology — MCQs

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