Disease may be asymptomatic, but majority have mild
illness and recover both clinically and biochemically within 2-4 weeks. Some may develop prolonged cholestatic hepatitis or fulminant hepatitis. Chronic liver disease does not occur. a. Hepatitis D b. Hepatitis A c. Hepatitis E d. Hepatitis B e. Hepatitis C 2. Features include nausea, vomiting, abdominal pain, lethargy and jaundice (in 50-70%). A large tender liver is common and 30% will have splenomegaly. The liver transferases are usually elevated 10-fold. Coagulation is usually normal. a. Biliary Atresia b. Alagille's syndrome c. Choledocal cysts d. Alpha 1 antitrypsin deficiency e. Viral Hepatitis 3. Uncommon, but high mortality. Most childhood cases due to a viral hepatitis and metabolic conditions. May present within hours or weeks with jaundice, encephalopathy, coagulopathy, hypoglycaemia and electrolyte disturbance. Complications include cerebral oedema, haemorrhage, coagulopathy, sepsis, pancreatitis. A, Wilson's disease b. Congenital hepatic fibrosis c. Acute liver failure d. Autoimmune hepatitis e. Reye's syndrome 4. The end stage of many forms of liver disease. It is defined pathologically as extensive fibrosis with regenerative nodules. a. Cirrhosis b. Hepatocellular carcinoma c. Portal hypertension d. Hepatocellular disease e. Chronic bile duct obstruction 5. An autosomal recessive disorder with an incidence of 1 in 2000-4000 in the UK. Severe forms are associated with liver disease (and lung disease in adults). a. Glactosaemia b. Biliary Atresia c. Alpha 1 antitrypsin deficiency d. TPN cholestasis e. Choledocal cysts 6. Causes rickets and pathological fractures. a. Vitamin K deficiency b. Vitamin E deficiency c. Vitamin D deficiency d. Vitamin A deficiency e. Vitamin C deficiency 7. A defective RNA virus which depends on another hepatitis virus for replication. It occurs as a co-infection or as a superinfection. Cirrhosis develops in 50-70% of those who develop chronic infection. a. Hepatitis E b. Hepatitis D c. Hepatitis C d. Hepatitis A e. Hepatitis B 8. Precipitated by gastrointestinal haemorrhage, sepsis, sedatives, renal failure or electrolyte imbalance. Infants present with irritability and sleepiness, while older children present with abnormalities in mood, sleep rhythm, intellectual performance and behaviour. EEG is always abnormal. a. Oesophageal varices b. Spontaneous bacterial peritonitis c. Encephalopathy d. Ascites e. Renal failure 9. Presents in children over 2 with hepatosplenomegaly, abdominal distension and portal hypertension. Renal disease may coexist. Liver function tests are normal in the early stage. a. Autoimmune hepatitis b. Congenital hepatic fibrosis c. Reye's syndrome d. Acute liver failure e. Wilson's disease 10. Babies with this condition have a normal birthweight but fail to thrive. They are jaundiced from the 2nd day, stools pale, urine dark, with hepatomegaly. Splenomegaly may develop a. Breast milk jaundice b. Haemolytic anaemia c. G6PD deficiency d. Crigler-Najjar syndrome e. Biliary atresia 11. Which of the following is NOT an example of a cause of jaundice due to unconjugated hyperbilirubinaemia? a. Crigler-Najjar syndrome b. Biliary atresia c. urinary tract infection d. breast milk jaundice e. G6PD deficiency 12. Alleviated by phenobarbital to stimulate bile flow, cholestyramine, which is a bile salt resin, ursodeoxycholic acid, an oral bile acid or evening primrose oil a. Pruritus b. Oesophageal varices c. Ascites d. Encephalopathy e. Spontaneous bacterial peritonitis 13. An autosomal dominant condition. Characteristic triangular facies, skeletal abnormalities, peripheral pulmonary stenosis, renal tubular disorders, defects in the eye and intrahepatic biliary hypoplasia with severe pruritus and failure to thrive. a. Choledocal cysts b. Alagille's syndrome c. Alpha 1 antitrypsin deficiency d. Biliary Atresia e. Progressive familial intrahepatic cholestasis (PFIC) 14. About 25% present in infancy with cholestasis. Diagnosis is by ultrasound or radionuclide scanning. Treatment is by surgical excision with the formation of a roux-en-Y anastomosis to the biliary duct. a. Choledocal cysts b. Glactosaemia c. Biliary Atresia d. Alpha 1 antitrypsin deficiency e. TPN cholestasis 15. A rare disorder (incidence 1 in 40000). Infants develop poor feeding, vomiting, jaundice and hepatomegaly when fed milk. Chronic liver failure, cataracts and developmental delay are inevitable if untreated a, TPN cholestasis b. Glactosaemia c. Biliary Atresia d. Choledocal cysts e. Alpha 1 antitrypsin deficiency 16. Which of the following is an example of intrahepatic biliary hypoplasia? a. Alpha 1 antitrypsin deficiency b. TPN cholestatis c. Galactosaemia d. Alagille's syndrome e. Cystic fibrosis 17. Causes night blindness in adults and retinal changes in infants. a. Vitamin E deficiency b. Vitamin K deficiency c. Vitamin D deficiency d. Vitamin A deficiency e. Vitamin C deficiency 18. A heterogeneous group of cholestatic disorders of bile acid transporter defects. Children present with jaundice, itching, failure to thrive, diarrhoea and a variable progression of liver disease. a. Alpha 1 antitrypsin deficiency b. Alagille's syndrome c. Progressive familial intrahepatic cholestasis (PFIC) d. Biliary Atresia e. Choledocal cysts 19. Which of the following is NOT am indication for liver transplantation in chronic liver failure? a. severe unresponsive malnutrition b. failure of growth and development c. sepsis d. recurrent complications e. poor quality of life 20. Which of the following is NOT an example of a cause of jaundice due to conjugated hyperbilirubinaemia? a. Cystic fibrosis b. Galactosaemia c. Alpha 1 antitrypsin deficiency d. Choledochal cyst e. Hypothyroidism 21. Mean age of presentation is 7-10 years. More common in girls. May present as acute hepatitis, fulminant hepatic failure or chronic liver disease with autoimmune features such as skin rash, lupus erythematosus, arthritis, haemolytic anaemia or nephritis. Diagnosis based on hypergammaglobulinaemia; positive autoantibodies, a low serum complement (C4); and typical histology. a. Congenital hepatic fibrosis b. Autoimmune hepatitis c. Acute liver failure d. Reye's syndrome e. Wilson's disease 22. Neurological features are common in the second decade including deterioration in school performance, mood and behaviour change, and incoordination, tremor and dysarthria. Renal tubular dysfunction, with vitamin D-resistant rickets, and haemolytic anaemia also occur. a. Acute liver failure b. Reye's syndrome c. Wilson's disease d. Congenital hepatic fibrosis e. Autoimmune hepatitis 23. An acute non-inflammatory encephalopathy with microvesicular fatty infiltration of the liver. Close association with aspirin therapy. a. Autoimmune hepatitis b. Wilson's disease c. Reye's syndrome d. Congenital hepatic fibrosis e. Acute liver failure 24. DNA virus which causes acute and chronic liver disease. High prevalence in the Far East, sub-Saharan Africa and parts of North and South America. Transmitted by: perinatal transmission, blood transfusions, needlestick injuries or biting insects, renal dialysis, horizontal spread. There is no treatment for acute infection. A, Hepatitis D b. Hepatitis C c. Hepatitis A d. Hepatitis E e. Hepatitis B 25. Prolonged neonatal jaundice may be diagnosed when jaundice has persisted until a. 3 days of age b. 6 days of age c. 2 days of age d. 3 weeks of age e. 2 weeks of age 26. Results in impaired coagulation and consequent bleeding a. Vitamin K deficiency b. Vitamin D deficiency c. Vitamin C deficiency d. Vitamin E deficiency e. Vitamin A deficiency 27. Also known as fulminant hepatitis a. Autoimmune hepatitis b. Acute liver failure c. Wilson's disease d. Reye's syndrome e. Congenital hepatic fibrosis 28. Autosomal recessive disorder with an incidence of 1 in 200000. Due to mutations on chromosome 13. Leads to an accumulation of copper in the liver, brain, kidney and cornea. Rarely presents in children under 3. Kayser-Fleischer rings not seen before 7 years. a. Autoimmune hepatitis b. Congenital hepatic fibrosis c. Acute liver failure d. Reye's syndrome e. Wilson's disease 29. What is the most common presentation of liver disease in the neonatal period? a. encephalopathy b. spider naevi c. prolonged jaundice d. hepatomegaly e. epistaxis 30. Causes peripheral neuropathy, haemolysis and ataxia. It is very poorly absorbed in cholestatic conditions and high oral doses are required. a. Vitamin A deficiency b. Vitamin D deficiency c. Vitamin C deficiency d. Vitamin K deficiency e. Vitamin E deficiency 31. Surgical bypass of fibrotic biliary ducts, the jejunum is anastomosed to patent ducts in the cut surface of the porta hepatis. a. Whipple's procedure b. Hepatosplenodectomy c. Wasai procedure d. Hepatoportoendectomy e. Hepatoportoenterostomy 32. Treatment includes penicillamine in combination with zinc. Pyridoxine is given to prevent peripheral neuropathy. 30% of children will die from hepatic complications if untreated. a. Wilson's disease b. Autoimmune hepatitis c. Acute liver failure d. Congenital hepatic fibrosis e. Reye's syndrome 33. Managed by treating the precipitating factor (sepsis, gastrointestinal haemorrhage), by protein restriction or by using oral lactulose to reduce ammonia reabsorption by lowering colonic pH and increasing colonic transit. a. Ascites b. Renal failure c. Encephalopathy d. Spontaneous bacterial peritonitis e. Oesophageal varices 34. Physical signs include palmar and plantar erythema and spider naevi, malnutrition and hypotonia. a. Autoimmune hepatitis b. Hepatocellular carcinoma c. Cirrhosis d. Wilson's disease e. Reye's syndrome 35. Consider if there is undiagnosed fever, abdominal pain, tenderness or an unexplained deterioration in hepatic or renal function. Perform diagnostic paracentesis. Treatment is with broad-spectrum antibiotics. a. Spontaneous bacterial peritonitis b. Oesophageal varices c. Encephalopathy d. Ascites e. Renal failure 36. An RNA virus. Prevalence is high among intravenous drug users. Seldom causes an acute infection, but at least 50% develop chronic liver disease, with cirrhosis and hepatocellular carcinoma. a. Hepatitis B b. Hepatitis D c. Hepatitis C d. Hepatitis A e. Hepatitis E 37. Diagnosed in up to 60% of overweight children but can also be found in lean individuals. The pathogenesis is not understood but may be linked to insulin resistance. In obese children liver function tests improve with weight loss. A, Autoimmune hepatitis b. Congenital hepatic fibrosis c. Wilson's disease d. Non-alcoholic fatty liver disease (NAFLD) e. Reye's syndrome 38. Ninety per cent of children with this condition will respond to prednisolone and a. azathioprine a. Congenital hepatic fibrosis b. Acute liver failure c. Wilson's disease d. Autoimmune hepatitis e. Reye's syndrome Key 1. B 2. E 3. C 4. A 5. C 6. C 7. B 8. C 9. B 10. E 11. B 12. A 13. B 14. A 15. B 16. D 17. D 18. C 19. C 20. E 21. B 22. C 23. C 24. E 25. D 26. A 27. B 28. E 29. C 30. E 31. E 32. A 33. C 34. C 35. A 36. C 37. D 38. D