Abstract
1.Regarding pathogenesis of genetically determined cholestatic disorders of the liver the following are TRUE:1.Inheritance of progressive familial intrahepatic cholestasis (PFIC) is autosomal recessive2.PFIC 1 is due to mutations in ABCB 11 gene encoding for bile salt excretory protein (BSEP)3.BSEP defect results in accumulation of bile salts in the hepatocyte with consequent hepatocellular damage4.Benign recurrent intrahepatic cholestasis (BRIC) can occur with mutations on the same genes that cause PFIC5.Intrahepatic cholestasis of pregnancy and drug induced cholestasis are not related to these genetic mutations2.Regarding the clinical features and diagnosis of PFIC the following are TRUE EXCEPT:1.PFIC 2 is the most severe form with highest propensity to develop liver failure, portal hypertension and hepatocellular carcinoma2.Majority of patients with PFIC 3 will present within first 3 months of life3.Extrahepatic manifestations are most common in PFIC 34.PFIC 1 and 2 have normal gamma glutamyl transpeptidase levels5.Serum alpha-fetoprotein levels are raised in PFIC 23.Regarding treatment of PFIC the following are TRUE:1.Ursodeoxycholic acid (UDCA) is useful in PFIC2.Cholestyramine and rifampicin are the drugs of choice in PFIC3.Biliary diversion procedures are useful to delay histological progression in patients who do not respond to medical therapy4.Ileal bypass is the biliary diversion surgical procedure of choice in PFIC5.PFIC can recur after liver transplantation4.Regarding the pathogenesis of Gaucher disease the following are TRUE:1.It is an autosomal dominant disease2.It results from deficiency of the lysosomal enzyme glucocerebrosidase3.Accumulation of glucosylsphingosine correlates with severity of neuronopathic disease4.N370S substitution is associated with neurological disease5.There is an absolute genotype–phenotype correlation in Gaucher disease5.Regarding the clinical features and diagnosis of Gaucher disease the following are TRUE EXCEPT:1.Type 1 Gaucher disease does not have neurological involvement2.Type 3 is the acute neuronopathic form of Gaucher disease3.Spleen is enlarged disproportionately more than the liver4.Cirrhosis is a common presenting feature5.Histopathological examination of bone marrow or spleen is the gold standard for diagnosis6.Regarding management of Gaucher disease the following are TRUE:1.Bone marrow transplantation is the treatment of choice2.Enzyme replacement therapy (ERT) is most useful in type 2 Gaucher disease3.ERT does not treat lung parenchymal disease and pulmonary hypertension4.Cirrhosis or advanced fibrosis is not reversible with ERT5.Drugs targeted at inhibition of glucosylceramide synthesis are useful for induction therapy7.Regarding pathogenesis and presentation of portal cavernoma cholangiopathy (PCC) the following are TRUE EXCEPT:1.Biliary system is supplied solely by the hepatic arterial system2.PCC may reverse only partially after shunt surgery3.Only minority of patients with PCC are symptomatic4.Majority of patients with PCC become symptomatic at a young age, soon after the diagnosis of portal cavernoma5.PCC term is applicable to biliary changes associated with portal hypertension of any etiology8.Regarding management of PCC the following are TRUE:1.Magnetic resonance cholangiography (MRC) is the diagnostic modality of choice2.Therapeutic endoscopic retrograde cholangiography (ERC) is required in all cases3.Majority of patients with symptomatic PCC cannot undergo shunt surgery due to absence of suitable veins4.Majority of patients of symptomatic PCC become asymptomatic after successful portosystemic shunt procedure5.Liver transplantation is not feasible in PCC9.Regarding hepatic encephalopathy (HE) the following are TRUE EXCEPT:1.Normal neurological examination and normal psychometric testing is characteristic of minimal HE2.The term covert HE includes minimal HE and grade I HE3.Covert HE does not need to be treated4.Magnetic resonance spectroscopy findings of HE are characterized by increased intracellular glutamate/glutamine signal and a decrease in myoinositol, taurine, and choline signals5.Lactulose acts solely by its laxative effect in HE10.Regarding non-alcoholic fatty liver disease (NAFLD) the following are TRUE:1.Asians origin individuals are at higher risk for developing NAFLD irrespective of their current place of residence2.The association of patatin-like phospholipase domain-containing protein 3 (PNPLA3) gene polymorphisms with NAFLD has been demonstrated for Caucasians only3.Only a minority of patients will be able to achieve desired weight loss despite intensive lifestyle modification programs.4.Weight loss medications are not superior to lifestyle modification programs in achieving desired weight loss5.Transient elastography is unreliable in predicting fibrosis in NAFLD
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