Hepatobiliary Quiz—9 (2014)

• 1.
  Regarding acute hepatitic illnesses in patients with chronic hepatitis B (CHB) infection the following are TRUE:

  • 1.
    Most common cause is flare of hepatitis B
  • 2.
    Flare of hepatitis B is more common in HBeAg negative individuals
  • 3.
    Flares of hepatitis B can be due to activation of immune system or due to reactivation of hepatitis B virus
  • 4.
    Reactivation of hepatitis B virus invariably occurs in the setting of immunosupression
  • 5.
    Reactivation of hepatitis B virus does not occur in HBsAg negative patients
• 2.
  Regarding differentiation of acute hepatitis B infection from flare of chronic hepatitis B the following are TRUE EXCEPT:

  • 1.
    Presence of serum IgM anti-HBc is pathognomonic for acute hepatitis B infection
  • 2.
    In acute hepatitis B infection the 19s subtype of IgM anti-HBc is more common
  • 3.
    IgG anti-HBc can be seen in both acute hepatitis B as well as reactivation of chronic hepatitis B infection
  • 4.
    Hepatitis B DNA levels are higher in acute hepatitis B infection
  • 5.
    HBsAg and HBeAg titres are lower in acute hepatitis B infection
• 3.
  Regarding pathogenesis and clinical features of Wilson disease the following are TRUE:

  • 1.
    Wilson disease occurs due to mutations in ATP7A gene
  • 2.
    Wilson disease is inherited in an autosomal recessive pattern
  • 3.
    Wilson disease has a definite phenotype–genotype correlation
  • 4.
    Liver disease manifests at earlier age than neurological disease
  • 5.
    Most patients with neurological presentation of Wilson disease will have underlying liver disease
• 4.
  Regarding the diagnosis of Wilson disease the following are TRUE:

  • 1.
    Kayser–Fleischer ring is almost universally present in patients with neurological Wilson disease
  • 2.
    Total serum copper is reduced in Wilson disease
  • 3.
    Low ceruloplasmin levels are specific for Wilson disease
  • 4.
    d-Penicillamine challenge test is useful to diagnose Wilson disease in adults and asymptomatic siblings of affected patients
  • 5.
    Testing for H1069Q mutation is useful to establish the diagnosis in Indian patients
• 5.
  Regarding treatment of Wilson disease the following are TRUE EXCEPT:

  • 1.
    Zinc acts by increasing urinary excretion of copper
  • 2.
    d-Penicillamine may cause initial worsening of neurological symptoms
  • 3.
    Trientine is contraindicated in patients intolerant to d-Penicillamine
  • 4.
    Ammonium tetrathiomolybdate may be useful for patients whose neurological disease worsens with other chelators
  • 5.
    Development of anemia or leukopenia during chelation therapy may be due to copper deficiency
• 6.
  Regarding the pathophysiology of neonatal hemochromatosis the following are TRUE:

  • 1.
    Neonatal hemochromatosis is a hereditary disorder
  • 2.
    Gestational alloimmune liver disease (GALD) is the probable cause of neonatal hemochromatosis
  • 3.
    GALD is an IgM mediated disease
  • 4.
    Complement mediated hepatocyte injury via the classical pathway is the hallmark of GALD
  • 5.
    Histopathological changes are panlobular, and typically spare the portal tracts
• 7.
  Regarding presentation and diagnosis of neonatal hemochromatosis the following are TRUE:

  • 1.
    Most cases will present after few weeks of birth with liver failure
  • 2.
    Hepatic transaminase levels are very high
  • 3.
    α-fetoprotein levels are very high
  • 4.
    Serum ferritin is very high and transferrin levels are low
  • 5.
    Presence of extrahepatic siderosis is helpful in making the diagnosis
• 8.
  Regarding treatment and prevention of neonatal hemochromatosis the following are TRUE:

  • 1.
    Iron chelators are useful
  • 2.
    Plasma exchange and intravenous immunoglobulin (IVIg) are therapy of choice
  • 3.
    Liver injury in neonatal hemochromatosis is irreversible with therapy
  • 4.
    Liver transplantation has excellent success rates
  • 5.
    Women with prior affected babies should receive IVIg prophylaxis in subsequent pregnancies
• 9.
  Regarding donor selection for deceased donor liver transplantation the following are TRUE EXCEPT:

  • 1.
    The ideal donor is less than 40 years of age and has trauma as cause of death
  • 2.
    Ideal donor includes donation after cardiac death
  • 3.
    Ideally the serum sodium of the donor should be below 150 mEq/L
  • 4.
    Exogenous hormone therapy like thyroid hormones and corticosteroids given prior to organ procurement in brain dead donors improves transplantation outcomes
  • 5.
    The Model for End-stage Liver Disease (MELD) score is the best predictor of post-transplant outcomes
• 10.
  Regarding extended criteria donors for deceased liver transplantation the following are TRUE:

  • 1.
    Cold ischemia time is an important determinant of post-transplant outcomes in grafts from extended criteria donors
  • 2.
    High levels of hepatic transaminases in the donor are a contraindication for liver donation
  • 3.
    The risk of transmission of malignancy via hepatic graft is about 1%
  • 4.
    Organs from donors with previously treated low grade malignancies may be considered for transplantation
  • 5.
    Outcomes from liver grafts from non-heart beating donors are invariably poor

(Answers on page 81)