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1. An RNA virus spread faecal-orally.

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