Question: 1 of 10 / Overall score: 60%

30715

In a child whose jaundice begins at less than 24 hours of age, the following diagnosis should
be considered:

True / False

CMV infection Correct

ABO incompatibility Correct

Hereditary spherocytosis Correct

Hypothyroidism Incorrect answer selected

High gastrointestinal obstruction Incorrect answer selected

Causes of neonatal jaundice include:

LESS THAN 24 HOURS: Haemolytic disease (Rhesus, ABO, enzyme or red cell
membrane defects), congenital infection.

24 HOURS - TWO WEEKS: Physiological, breast milk, infection (e.g. urinary tract
infection), haemolysis (as above), bruising, polycythaemia, Crigler-Najjar Syndrome.

GREATER THAN TWO WEEKS:

o Unconjugated - physiological or breast milk, infection (particularly urinary tract

infection), hypothyroidism, haemolysis, high gastrointestinal obstruction.

o Conjugated (>10% total bilirubin) - bile duct obstruction, e.g. biliary atresia,
neonatal hepatitis.

Question: 2 of 10 / Overall
score: 60%

3187

The following statements are true:

 True / False

Wilson's disease can cause cirrhosis in childhood. Correct

Hepatitis A infection can cause mixed conjugated and unconjugated

hyperbilirubinaemia. Correct

Biliary atresia is characterised by bile stained vomiting. Incorrect

answer selected

Conjugated hyperbilirubinaemia is water soluble. Correct

Hepatic veins drain directly into the portal vein. Incorrect answer
selected

Conjugated hyperbilirubinaemia is a
rare but important finding in the
neonate. It accounts for the minority of
cases of prolonged jaundice, but is
always pathological. Biliary atresia,
neonatal hepatitis syndrome, Wilson's
Disease, cystic fibrosis, and alpha0
antitripsin deficiency are all recognised
causes. Viral infections, such as CMV,
hepatitis A and B, and protozoal
infections such as toxoplasma can also
cause it. In the latter cases it may be a
mixed form, because of partial
obstruction to uptake of bilirubin
precursors. The hepatic veins leave the
posterior surface of the liver and
immediately enter the inferior vena
cava, while the portal veins drain from
the gut.

Question: 3 of 10 / Overall score: 73%

30688

The following conditions can present with a clinical picture resembling Reye's Syndrome:

True / False
 Medium change acell co-adyhydrogenase deficiency Correct

Systemic carnatine deficiency Correct

Meningitis Correct

Valproate ingestion Correct

Fructose 1,6, diphosphatase deficiency Correct

Classic Reye's Syndrome exhibited as stereotypic biphasic course. It occurs in previously

healthy children with a prodromal febrile illness (90% upper respiratory tract infection, or
chickenpox 6%) followed by apparent recovery. There is subsequent abrupt protracted
vomiting a week later resulting in delirium, stupor and combative behaviour. This progress to
seizures, coma and death with a notable absence of focal neurology. The liver may be mildly
enlarged but the child does not have jaundice. CSF is clear apart from elevated pressure. A 5
stage grading system is suggested:

I. Lethargy.

II. Confusion.

III. Light coma -/+ seizures with decorticate rigidity.

IV. Deep coma, seizures and a cerebrate rigidity.

V. Deep coma with absent tendon reflexes, respiratory arrest and fixed dilated pupils
with flacidity or intermittent decerebrations.

The differential diagnosis includes:

Metabolic disease.

Organic acidurias, urea cycle defects, defects in fatty acid metabolism (including
carnatine deficiency).

CNS infection (meningitis, encephalitis, toxic encephalopathy).

Haemorrhagic shock with encephalopathy.

Drug ingestion (salicylates, valproate).

 Question: 4 of 10 / Overall
score: 75%

3154

Alpha 1 antitripsin:
True / False

Is a naturally occurring protein. Correct

Is an acute phase protein. Correct

Is produced in the liver. Correct

Causes liver disease in its homozygote form. Correct

Is associated in children with increased susceptibility to alcoholic liver

disease in the heterozygote form. Incorrect answer selected

Acute phase reactants are plasma

constituents that appear or increase
during the inflammatory state. They
include the erythrocyte sedimentation
rate (ESR), C-reactive protein (CRP),
serum mucoproteins, various
a{alpha}-globulins, gamma globulins,
some complement components, and
certain proteins such as transferrin. A
small percentage of individuals
homozygous for deficiency of the major
serum protease inhibitor, alpha0-
antitrypsin, have neonatal cholestasis
and later childhood cirrhosis. A1AT, a
glycoprotein synthesised by the liver,
accounts for 80% of the serum
a{alpha}1-globulin fraction. A1AT is
present in more than 20 different
codominant alleles, only a few of which
are associated with defective protease
inhibitors. The most common allele of
the protease inhibitor (Pi) system is M,
and the normal phenotype is PiMM. The
2 allele predisposes to clinical
deficiency; patients with liver disease
are usually PiZZ and have serum
alpha0-antitrypsin levels less than 2
 mg/mL (approximately 10-20% of
normal).
The incidence of the PiZZ genotype in
the white population is estimated at
1:2,000-4,000. Intermediate
phenotypes PiMS, PiMZ, and PiSZ are
not definitively associated with liver
disease. The null genotype has no
periodic acid-Schiff (PAS)-positive
inclusions and is not associated with
liver disease. Of all PiZZ persons, less
than 20% will develop neonatal
cholestasis. These patients are
indistinguishable from other infants
with "idiopathic" neonatal hepatitis, of
whom they constitute approximately
500%. In affected patients the course
of liver disease is highly variable.
Jaundice, acholic stools, and
hepatomegaly are present during the
1st week of life, but the jaundice
usually clears during the 2nd-4th
months. There may follow complete
resolution, persistent liver disease, or
the development of cirrhosis. Older
children may present with
manifestations of chronic liver disease
or cirrhosis, with evidence of portal
hypertension.
Question: 5 of 10 / Overall
score: 64%

24616

Concerning a hepatitis E infection:

True / False

It can be transmitted with hepatitis B. Incorrect answer selected

It is a recognised cause of chronic liver disease. Incorrect answer

selected

CT scan of the liver with contrast shows diagnostic appearances.

Incorrect answer selected

The incidence of chronic liver disease is reduced by administration of alpha

interferon. Incorrect answer selected
 It does not result in a carrier state. Correct

E. Five hepatitis viruses form a

heterogeneous group causing similar
clinical illnesses. Hepatitis A, C, D, and
E are all RNA viruses coming from 4
different families; and hepatitis B is a
DNA virus. Hepatitis A & E cause acute
illness, with the former causing most
hepatitis in childhood and hepatitis E
being very rare. Hepatitis B, C, and D
cause chronic morbidity and mortality,
with B causing a third of cases,
hepatitis C a fifth of cases, and D being
very rare. Hepatitis D illness cannot
occur without B as a helper virus.
Hepatitis B can be treated with
interferon-alpha, which improves liver
disease.
Question: 6 of 10 / Overall
score: 70%

3136

In cirrhosis with portal hypertension,

the following may occur:
True / False

Acute upper gastrointestinal bleeding Correct

A positive puddle sign Correct

Bacterial peritonitis Correct

Irritability and sleepiness Correct

Renal tubular acidosis Correct

Cirrhosis is a final common pathway for

many forms of liver disease.
Pathologically, there is extensive
 fibrosis with regenerative nodules. This
results in decreased hepatic function
and portal hypertension, with
splenomegaly, varices and ascites.
There is an increased risk of
hepatocellular carcinoma. If
compensated, cirrhosis may be
asymptomatic. As the cirrhosis
increases, malnutrition, abdominal
distension from hepatosplenomegaly
and ascites, scrotal swelling, and
dilated abdominal veins occur (caput
medusae). The oesophageal varices
can bleed requiring transfusion,
vasopressin analogue, sclerotherapy or
endoscopic injection. Portocable shunts
may preclude liver transplantation.
Ascites can result in shifting dullness
and a positive puddle sign. Albumin
transfusion, paracentesis or peritoneo-
venous shunts may be required. There
is an increased incidence of
spontaneous bacterial peritonitis.
Encephalopathy may be precipitated by
gastrointestinal haemorrhage, sepsis,
sedatives, renal failure, or electrolyte
imbalance. Children become irritable
and sleepy with changes in mood and
intellectual performance. The plasma
ammonia may be elevated and the EEG
is abnormal. Renal failure may be
secondary to renal tubular acidosis,
acute tubular necrosis or functional
renal failure.
Question: 7 of 10 / Overall
score: 71%

27606

The following statements about

hepatitis are true:
True / False

Hepatitis C rarely leads to a chronic condition. Incorrect answer

selected

A raised IgM antibody titre confirms the diagnosis of hepatitis A. Correct

Hepatitis A vaccine is very effective. Correct

 Hbs AB persists for more than 10 years. Correct

Hbe Ag is associated with increased infectivity. Correct

Hepatitis C is a transfusion-related
virus that is causing immense
problems to the Blood Transfusion
Service. Hepatitis A and E cause acute
disease, but B, C and D can all cause
chronic hepatitis.
Question: 8 of 10 / Overall
score: 70%

702

Familial hepatic diseases associated

with cirrhosis include:
True / False

galactosaemia Correct

Osler-Weber-Rendu syndrome Correct

cystinosis Incorrect answer selected

Marfan's syndrome Incorrect answer selected

Wilson's disease Correct

Familial hepatic diseases associated

with cirrhosis include Wilson's,
haemochromatosis, galactosaemia,
indian familial childhood cirrhosis and
alpha1 antitypic deficiency. Osler-
weber-rendu is associated with
hereditary telangiectasia and may be
associated with large GI haemorrhage.
Marfan's is not really associated with
any hepatic disease. Cystinosis is a
 rare disease causing the accumulation
of the amino acid, cystine. Although
cystine accumulates in the liver it does
not cause cirrhosis. Cystinosis is
associated with renal failure, muscle
wasting, diabetes and hypothyroidism.

True

Question: 9 of 10 / Overall
score: 71%

10052

Features of biliary atresia include:

True / False

Chalk-coloured stools Correct

Unconjugated hyperbilirubinaemia Correct

Prolonged hyperbilirubinaemia Correct

Normal excretion of isotope into duodenum in HIDA scan Incorrect

answer selected

Osteoporosis Correct

Chalk-coloured stools , dark urine ,

weight loss, jaundice and abdominal
distension are characteristic of biliary
atresia. Breast milk jaundice is a
benign self limiting condition causing
unconjugated hyperbilirubinaemia;
biliary atesia causes prolonged
obstructive jaundice. If the isotope
passes from the liver into the
duodenum the bile ducts would be
patent, unlike in biliary atresia.
Osteoporosis and octeomalacia are
potential consequences of Vitamin D
malabsorbtion.

Question: 10 of 10 / Overall score: 70%

 30687

The following drugs are inducers of hepatic enzymes:

True / False

Cimetidine Incorrect answer selected

Phenytoin Correct

Rifampicin Correct

Allopurinol Incorrect answer selected

Carbamazepine Correct

Drug metabolism within the hepatocyte involves two primary enzymatic processes: phase I, or
nonsynthetic, and phase II, or synthetic reactions.

Phase I reactions include oxidation, reduction, hydrolysis, and hydroxylation reactions,

whereas phase II reactions primarily involve conjugation with glycine, glucuronide, or
sulphate. Most drug-metabolising enzymes are located in the smooth endoplasmic reticulum of
cells that are recovered as the microsomal fraction on homogenation. Of these mixed function
oxidase systems, the cytochrome P-450 system has been studied in greatest detail.

In addition, the extent of fetal hepatic drug metabolism may be influenced by hepatocyte
concentrations of ligandin. Ligandin, or Y protein, is a basic protein responsible for substrate
uptake by metabolising cells. Ligandin binds bilirubin and organic anions, including drugs.
Although concentrations of ligandin at birth are low, values comparable to those in adults have
been observed in the 1st 500 days of postnatal life. Inducers fall into three categories:

1. General, stimulating many pathways e.g. barbiturates, alcohol, nicotine, rifampicin,

phenothiazines

2. Polycyclic hydrocarbons e.g. 3-methylchloranthrene

3. Steroids stimulating microsomal enzymes, especially hydroxylation, dealkylation,

deamination, desulphuration and glucuronidation.

Important induction interactions include:

Oral anticoagulants
 Rickets with anti-epileptics

Decreased OCP effectiveness

Decreased efficacy of antiepileptics

Cimetidine retards oxidative hepatic drug metabolism by binding to microsomal cytochrome P450. It should be
avoided in patients stabilised on warfarin, phenytoin, and theophylline (or aminophylline),