Hepatobiliary Quiz—9 (2014)

Abstract

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 B2.Flare of hepatitis B is more common in HBeAg negative individuals3.Flares of hepatitis B can be due to activation of immune system or due to reactivation of hepatitis B virus4.Reactivation of hepatitis B virus invariably occurs in the setting of immunosupression5.Reactivation of hepatitis B virus does not occur in HBsAg negative patients2.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 infection2.In acute hepatitis B infection the 19s subtype of IgM anti-HBc is more common3.IgG anti-HBc can be seen in both acute hepatitis B as well as reactivation of chronic hepatitis B infection4.Hepatitis B DNA levels are higher in acute hepatitis B infection5.HBsAg and HBeAg titres are lower in acute hepatitis B infection3.Regarding pathogenesis and clinical features of Wilson disease the following are TRUE:1.Wilson disease occurs due to mutations in ATP7A gene2.Wilson disease is inherited in an autosomal recessive pattern3.Wilson disease has a definite phenotype–genotype correlation4.Liver disease manifests at earlier age than neurological disease5.Most patients with neurological presentation of Wilson disease will have underlying liver disease4.Regarding the diagnosis of Wilson disease the following are TRUE:1.Kayser–Fleischer ring is almost universally present in patients with neurological Wilson disease2.Total serum copper is reduced in Wilson disease3.Low ceruloplasmin levels are specific for Wilson disease4.d-Penicillamine challenge test is useful to diagnose Wilson disease in adults and asymptomatic siblings of affected patients5.Testing for H1069Q mutation is useful to establish the diagnosis in Indian patients5.Regarding treatment of Wilson disease the following are TRUE EXCEPT:1.Zinc acts by increasing urinary excretion of copper2.d-Penicillamine may cause initial worsening of neurological symptoms3.Trientine is contraindicated in patients intolerant to d-Penicillamine4.Ammonium tetrathiomolybdate may be useful for patients whose neurological disease worsens with other chelators5.Development of anemia or leukopenia during chelation therapy may be due to copper deficiency6.Regarding the pathophysiology of neonatal hemochromatosis the following are TRUE:1.Neonatal hemochromatosis is a hereditary disorder2.Gestational alloimmune liver disease (GALD) is the probable cause of neonatal hemochromatosis3.GALD is an IgM mediated disease4.Complement mediated hepatocyte injury via the classical pathway is the hallmark of GALD5.Histopathological changes are panlobular, and typically spare the portal tracts7.Regarding presentation and diagnosis of neonatal hemochromatosis the following are TRUE:1.Most cases will present after few weeks of birth with liver failure2.Hepatic transaminase levels are very high3.α-fetoprotein levels are very high4.Serum ferritin is very high and transferrin levels are low5.Presence of extrahepatic siderosis is helpful in making the diagnosis8.Regarding treatment and prevention of neonatal hemochromatosis the following are TRUE:1.Iron chelators are useful2.Plasma exchange and intravenous immunoglobulin (IVIg) are therapy of choice3.Liver injury in neonatal hemochromatosis is irreversible with therapy4.Liver transplantation has excellent success rates5.Women with prior affected babies should receive IVIg prophylaxis in subsequent pregnancies9.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 death2.Ideal donor includes donation after cardiac death3.Ideally the serum sodium of the donor should be below 150 mEq/L4.Exogenous hormone therapy like thyroid hormones and corticosteroids given prior to organ procurement in brain dead donors improves transplantation outcomes5.The Model for End-stage Liver Disease (MELD) score is the best predictor of post-transplant outcomes10.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 donors2.High levels of hepatic transaminases in the donor are a contraindication for liver donation3.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 transplantation5.Outcomes from liver grafts from non-heart beating donors are invariably poor