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1-On physical examination, the right testis of an 18 month old boy is not in the scrotum:

True / False

There is likely to be a direct inguinal hernia on the same side

Incorrect

answer selected
Orchidopexy should be left until he is 10 years old
He is likely to suffer a late onset of puberty

Correct

Correct

A normally situated left testis rules out the possibility of testicular


feminization syndrome

Correct

A 3 month course of intra-muscular testosterone should be given to


encourage descent

Correct

In the above scenario, there is likely to be an indirect inguinal hernia if the cryptorchidism
is due to a true undescended testicle. Orchidopexy needs to ideally be carried out in his
second year of life to minimize the risks of malignancy and infertility. In testicular
feminisation syndrome, genetic males are born with female genitalia.Testes are usually
intraabdominal but may descend into the inguinal canal. In incomplete testicular
feminisation testes may be present in the labioscrotal folds.as males may exhibit a small
amount of masculinisation.

2-Escherichia coli 0157 / H7:


True / False

is a bowel commensal

Correct

causes haemorrhagic colitis

Correct

is an important cause of cholera-like illness

Correct

is a recognised cause of the haemolytic uraemic syndrome


can be prevented from causing clinical illness by vaccination

Correct
Correct

Escherichia coli 0157 / H7 characteristically causes a haemorrhagic colitis with abdominal


pain but little or no fever. An outbreak of 500 cases in the USA was described in 1993. This
outbreak was associated with the consumption of hamburgers. There were over 50 cases
of haemolytic uraemic syndrome and 4 fatalities. The source of an outbreak in Wishaw,
Scotland in 1996 was a butcher's. There were over 500 cases and 18 fatalities.

3-Penicillins:
True / False

Are bactericidal

Correct

Exert their actions by combining with a transpeptidase


selected

Incorrect answer

May be inactivated by enzymes which are plasmid coded


Have significant toxic effects on humans

Correct

Correct

Have a spectrum of action which is independent of the beta lactam side


chain

Correct

Penicillins are generally bactericidal and exert their effect by combining with and
inhibiting the transpeptidase enzyme which cross-links the peptidoglycans in the
cell wall. This weakens the cell wall and allows the cell to lyse under the influence
of an osmotic gradient.
The widespread resistance to penicillins is due to beta lactamase enzymes which
break the beta lactam ring and inactivate the drug. The beta lactamase enzyme is
often encoded in the bacterial plasmid. The plasmid is bacterial DNA (separate
from the main chromosome) that can be translocated from bacterium to
bacterium and it is for this reason that resistance may be also transferred.
Although the penicillins have similar spectra of action, their specific spectra may
be changed by altering the beta lactam side chain. They do not have significant
toxic effects on humans, but allergy is common.

4-Progressive spinal muscular atrophy of infancy presents with


True / False

severe generalised weakness

Incorrect answer selected

fasiculations seen in the tongue

Correct

loss of spinothalamic tract function

Correct

spontaneous fibrillation on electromyography


normal tendon reflexes

Correct

Correct

Spinal muscular atrophy , classified into three forms : Infantile, late infantile and juvenile.
The specific gene defect (AR inheritance) is localised to the Chr 5, which when defective
fails to suppress the normal preprogrammed neuronal cell death at birth. The characteristic
features are fasciculations seen best in the resting tongue, fibrillation potentials in EMG,
denervation pattern on a muscle biopsy.

5-Regarding standard error of the mean (SEM) and standard deviation (SD) the following are
true
True / False

SEM is calculate by taking the square root of the SD of the sample means
Correct

SD invariably falls with increasing sample size


SEM increases with sample size

Correct

Correct

if SD is greater than the mean the distribution is positively skewed


Correct
Student's t test is a non-parametric test

Correct

The Standard error of the Mean = SD/sq root n. SD does not necessarily fall with sample
size as the distribution of values may increase and hence SD increase. SEM would decrease
with sample size as can be seen in the above calculation. d - negatively distributed.
Student's T test is a parametric test comparing normally distributed data.

6-In Hodgkin's disease, an unfavourable prognosis is indicated by


True / False

A. Mixed cellularity pathology on lymph node biopsy

Correct

B. Clinical presentation with pyrexia of unknown origin


C. Presentation over the age of 60 years
D. Pruritis

Correct

Incorrect answer selected

Incorrect answer selected

E. Presence of Reed-Sternberg cells on bone-marrow trephine

Incorrect

answer selected

A. And lymphocyte depleted.


D. Nor is alcohol-induced chest pain.
E Implies spread to bone marrow!

7-Regarding the child with near drowning


True / False

On initial immersion, apnoea and tachycardia occur

Correct

If the rectal temperature on arrival is below 33C, the chances of survival


are increased

Correct

Immersion in salt water carries a worse prognosis

Correct

Associated cervical spine injury is unusual

Correct

External rewarming is usually sufficient if the core temperature is above


32C.

Incorrect answer selected

On initial immersion the diving reflex occurs, which results in apnoea and
bradycardia. Later, as apnoea continues, hypoxia results, causing tachycardia,
increase in blood pressure and acidosis. Rapid cooling of the organs to below
33C is protective and associated with a better prognosis. Immersion in salt or
fresh water carries the same prognosis. Cervical spine injury should be suspected
in all cases of near drowning as the incidence of this associated injury is high,
especially in diving accidents. External rewarming (removal of wet clothing, warm
or heating blankets, infrared lamp) is sufficient for core temperatures of 32C or
above but for lower core temperature, core rewarming becomes necessary (warm
intravenous fluids and ventilator gases,
gastric/bladder/peritoneal/pleural/pericardial lavage).
Efforts to resuscitate should be continued until the core temperature is at least
32-34C.

8-In a 6 month old child with meningitis, the following organisms should be
covered by first line antibiotic therapy:
True / False

Group B Streptococcus
Listeria monocytogenes
E. Coli

Correct
Incorrect answer selected

Incorrect answer selected

Haemophilus Influenzae Type B


Pseudomonas.

Correct

Correct

Antibiotics should be selected according to the common organisms prevalent at


various ages. Currently, in the UK these are:

<3 months of age: Group B Streptococcus, Escherichia


coli or other gram negatives, Listeria.

1 month - 6 years of age: Meningococcus,


Pneumococcus, Haemophilus influenzae. The incidence of
the latter has declined since the introduction of Hib
vaccine, but it should still be covered.

>6 years: Meningococcus, Pneumococcus.

Copyright 2002 Dr Colin Melville

9-The following are examples of multifactorial inheritance manifest in the


neonatal period:
True / False

Anencephaly

Incorrect answer selected

Pyloric stenosis

Correct

Myotonic dystrophy

Incorrect answer selected

Ankylosing spondylitis

Incorrect answer selected

Leber's optic neuropathy

Correct

Polygenical multifactorial inheritance refers to a spectrum of disorders which are


neither purely environmental in origin nor purely hereditary. They are thought to
result from the additive effect of several genes with or without the influence of
environmental or other unknown factors. Height and IQ are inherited in this way,
and these parameters show a normal distribution in the population. Relatives of
an affected person show an increased liability to the disorder so that a greater
proportion of them than in the general population will fall beyond the threshold
and will manifest the disorder. The disease may also be more severe in relatives,
particularly where there is a close relationship to the affected person and there
are multiple affected family members. In addition, a sex difference in prevalence
results in an increased risk to relatives. The phenotype may be manifest as a
congenital malformation or in adult life.

Congenital malformations: Neural tube defects,


congenital heart disease, cleft lip and palate, pyloric
stenosis, CDH, talipes, hypospadias.

Adult life: Atherosclerosis and coronary heart disease,


diabetes mellitus, asthma, epilepsy, hypertension, HLA
associated diseases.

Leber's hereditary optic neuropathy is a mitrochondrial abnormality.


Copyright 2002 Dr Colin Melville

10-Which of the following statements concerning the reporting of adverse drug


reactions (ADR) in the UK is/are correct?
True / False

ADR reporting is compulsory

Incorrect answer selected

A black triangle sign alongside a drug within the British National


Formulary (BNF) requires that all ADRs should be reported with this drug
Correct

Reporting is not required for ADRs associated with vaccinations

Correct

There is no requirement to report ADRs with Over The Counter (OTC)


drugs

Correct

When submitting a yellow card concerning an ADR, all other drugs taken
within the last 3 months should also be reported

Correct

Reporting ADRs to the Medicines Control Agency is a voluntary process but all
side effects noted with newer drugs denoted with a black triangle in the BNF
should be reported.
Even ADRs with OTC therapies should be reported to ensure adequate monitoring
and potentially ascertain whether the manufacturing of certain brands is
appropriate.
The report should also contain drugs that the patient has taken (including OTCs)
within the last three months to establish potential interactions.

11-Henoch-Schnlein syndrome is associated with:


True / False

A highest incidence in children under the age of 3


Hepatosplenomegaly

Incorrect answer selected

Haemolytic anaemia

Correct

Sudden onset of oedema in unusual places


Elbow purpura and papules

Correct

Incorrect answer selected

Correct

75 % of the children are above two years but below 11 years.


Hepatosplenomegaly is a rare feature of active disease.
Haemolysis is not present, full blood count and clotting are normal.
Subcutaneous oedema of the feet, hands, scalp and ears are seen. Scrotal
oedema also may occur. Gastrointestinal bleeding may occur and haematuria and
proteinuria also may occur. Abdominal pain, intussusception and arthritis are
features.
Purpura and papules occur in the elbows but the characteristic sites are thighs
and buttocks.
Further reading:
Henoch-Schnlein syndrome eMedicine

12-Regarding sickle cell disease


True / False

The Sickledex test involves adding a reagent to blood, which identifies


the type of haemoglobinopathy

Correct

It is caused by the substitution of glutamic acid by valine at position 6 on


the beta chain of haemoglobin

Incorrect answer selected

The erythrocytes of Haemoglobin AS patients can sickle at a PO 2 of 5 - 6


kPa (40 - 50 mmHg)

Correct

The erythrocytes of Haemoglobin SC patients may sickle at a PO 2 of 4 kPa


(30 mmHg)

Correct

Osteomyelitis is typically caused by E. coli bacteria

Correct

Sickle cell disease in a haemoglobinopathy caused by the substitution of glutamic


acid by valine at position 6 (from the N-terminal) of the beta chain. Inherited as
an autosomal gene, heterozygous (HbAS) and homozygous (HbSS) forms exist. A
low partial pressure of oxygen (PO2) causes HbS to polymerise and precipitate,
resulting in sickling of the erythrocyte. HbSS patients sickle at PO2 of 5 - 6 kPa
and HbAS patients sickle at PO2 of 2.5 - 4 kPa (not 5 - 6 kPa).
A mild disease is produced when heterozygotes for HbS combine with other
haemoglobins e.g. Haemoglobin C, creating HbSC, with sickling occurring at
around 4 kPa. Osteomyelitis is typically caused by unusual organisms, e.g.
Salmonella.
Diagnosis of sickle cell disease requires the detection of HbS. The Sickledex test
involves the addition of reagent to blood; turbidity only confirming the presence
of HbS, but it gives no information on other haemoglobins. Haemoglobin
electrophoresis is the only investigation that can determine the nature of the
haemoglobinopathy.

13-The following are recognised features of Down's syndrome:


True / False

Coarctation of the aorta

Correct

Overlapping of the 3rd and 4th fingers


Hoarse cry
Hypotonia

Correct

Correct
Correct

Corneal opacities

Correct

The following are clinical features: third fontanelle, brachycephaly, sparse hair,
upslanted palpebral fissures, epicanthal folds, brushfield spots, large tongue in a
small mouth, transverse palmar creases, brachydactyly, fifth-finger clinodactyly,

hypotonia and cataracts. The cardiac defects that can occur are: endocardial
cushion defects, ventricular septal defect, patent ductus arteriosus, atrial septal
defect and Tetralogy of Fallot. Coarctation is associated with Turner's syndrome
and not Down's Syndrome. Corneal opacity can occur secondary to glaucoma.

14-Characteristic features of Turner's syndrome include:


True / False

Short stature

Correct

Mental retardation
Karyotype XXY

Correct

Correct

Webbing of the neck

Correct

Increased incidence of coarctation of the aorta

Correct

Short stature (with height of >2.5 standard deviations below the mean height
value for age) is an important phenotypic feature of the condition and it is not
due to deficiency of growth hormone, thyroid hormone, adrenal or gonadal
steroids but may improve following the administration of growth hormone. In
general patients with gonadal dysgenesis do not differ from siblings in overall
intelligence. The karyotype is XO or XO/XX in mosaics. Webbing of the neck,
lymphoedema of the distal extremities, ascites and pleural effusions are features.
Cardiovascular abnormalities other than coarctation include bicuspid aortic valve,
mitral valve prolapse and aortic root dilatation.

15-The following applies to rotavirus gastroenteritis:


True / False

It typically affects infants older than 18 months

Correct

The Rotazyme test to detect virus particles is a direct enzyme-linked


immunosorbent assay

Correct

Blood and mucus is found in the stools of about 50% of affected babies
Correct
It occurs most often in the summer months
Protection is not conferred by breast feeding

Correct
Correct

The peak incidence of rotavirus infection is 3-15 months.

ELISA is performed on the stool samples to detect virus particles.


Watery diarrhoea is common and blood and mucus is rare.
Epidemics occur in the cooler months.
Breast feeding protects against rotavirus diarrhoea and other infections e.g.
pneumonia.

16-Bone age:
True / False

Is more advanced in girls than in boys of the same age

Correct

Is characteristically retarded in girls with untreated congenital adrenal


hyperplasia

Correct

Is a better predictor of final adult height than height for age


May be retarded by emotional deprivation
May be retarded in chronic renal failure

Correct

Correct

Correct

Bone age and skeletal maturity are more advanced in females compared to males of the
same age, and they reach peak height velocity earlier. Bone age is advanced in girls with
untreated congenital adrenal hyperplasia due to the effects of the excessive androgens.
Bone age (which can be measured with an x-ray of the left wrist) determines the timing of
puberty and can be used to predict how much additional growth is possible, therefore it is
an excellent predictor of final adult height. Extreme emotional deprivation may retard
growth by causing reversible hypothalamic-pituitary depression. Normal skeletal growth
resumes rapidly after removal from the oppressive environment. Chronic renal failure is
also a well known cause of growth failure and delayed skeletal maturation. Improved
metabolic control, nutrition and growth hormone therapy improves growth in such
patients.

17-The following definitions are true:


True / False

Still birth rate is the rate of fetal deaths after 28 completed weeks of
pregnancy per 1000 total pregnancies.

Correct

The perinatal mortality rate is the total of still births plus deaths within
the first month per 1000 live and still births.

Correct

The neonatal mortality rate is the deaths of live born infants less than 28
days of age per 1000 live births.

Correct

An abortion is a premature expulsion from the uterus of the products of

conception before 26 completed weeks gestation.

Correct

A miscarriage is the loss of the products of conception from the uterus


before 16 completed weeks.

Incorrect answer selected

A still birth is defined as a fetal death after 24 completed weeks of pregnancy. A


miscarriage is fetal loss before 24 completed weeks. Perinatal mortality rate is the
still births plus deaths within the first 6 days per 1000 live and still births.
Neonatal mortality rate is the deaths of live born infants.

18-Regarding the Central Limit Theorem:


True / False

It provides information on the sampling distribution of x.

Incorrect

answer selected
It states that the sampling distribution of the mean is based on repeated
random samples from a population will be approximately bell shaped.
Incorrect answer selected
Is a way of defining the mode.

Incorrect answer selected

It states that the median is usually larger than the mode.

Correct

It implies that, for a large number of measurements, the mean will tend
to the population mean.

Correct

The Central Limit Theorem states that the distribution of the mean of N random
variables tends to a normal distribution as N tends to infinity. The main condition
is that the variance of any one variable should not dominate. An important
application is to the mean of a random sample of N independently identically
distributed random variables, each with a mean u and a standard deviation o. For
a large N, the theorem implies that this mean will be N (, o2/N). In practice,
convergence is usually very rapid. The standard error of the mean equals u/pN.
The significance of the Central Limit Theorem is two-fold: 1. It explains why many
measurements have bell-shaped frequency distributions. 2. It is useful in
statistical inference.
Copyright 2002 Dr Colin Melville

19-The following suggest a diagnosis of Friedreich ataxia rather than ataxia


telangiectasia:
True / False

Increased chromosomal fragility

Correct

Increased susceptibility to infection


Raised serum alphafeta protein
Pes cavus

Correct

Correct

Correct

Optic atrophy

Correct

Ataxia telangiectasia is a DNA repair defect inherited as an autosomal recessive.


It presents with difficulty with balance and co-ordination and occular motor
dyspraxia is evident by school age. Telangiectasia develops from about 4 years of
age, particularly on the conjunctivae, shoulders and neck. There is increased
susceptibility to infection from a variable immune defect, and increased incidence
of malignant disorders, a raised alphafeta protein, and increased DNA fragility to
irradiation which is useful diagnostically. Friedreich's ataxia is the most common
form or spinal cerebellar degeneration. It is inherited as an autosomal recessive
and presents with progressive clumsiness. There is ataxia of the limbs and trunk
with distal wasting in the legs, diminished reflexes, and pes cavus. The above are
similar to other hereditary motor sensory neuropathies, but in Friedreich ataxia
there is impairment of propreception and often optic atrophy. There is progressive
cerebellar degeneration with scoliosis and cardiomyopathy, the latter causing
death at 40-50 years of age.
Copyright 2002 Dr Colin Melville

20-The following suggest a subarachnoid haemorrhage rather than an


extradural haemorrhage:
True / False

Retinal haemorrhage
Neck stiffness
Fever

Correct

Incorrect answer selected

Incorrect answer selected

Focal neurological signs

Incorrect answer selected

Accompanying skull fracture

Correct

An extradural haemorrhage results from arterial or venous bleeding into the


extradural space following direct head trauma, and is often accompanied by a
skull fracture. There may be a lucid interval until the conscious level deteriorates
and seizures occur secondary to the space-occupying lesion formed by the
enlarging haematoma. Focal neurological signs, anaemia, and shock may occur.
Subdural haematomata seen almost exclusively in infants and toddlers due to
non-accidental injury caused by shaking, and they are accompanied by retinal
haemorrhages. Subarachnoid haemorrhage is usually caused by an aneurysm or
arteriovenous malformation, and presents acutely with head pain, neck stiffness
and occasional fever. Retinal haemorrhage is usually present and seizures and
coma may develop.

Copyright 2002 Dr Colin Melville

21-The following EEG abnormalities are correctly paired:


True / False

Burst-suppression - myoclonic epilepsy of adolescents.

This is the

correct answer
Hypsarrhythmia - infantile spasms.

Correct

Temporal lobe abnormalities - herpes simplex encephalitis.

Incorrect

answer selected
3 cycle per second spike and wave discharge - petit mal seizures.
Correct
Centro-temporal spikes - benign rolandic epilepsy.

Correct

An EEG is indicated whenever epilepsy is suspected. If the standard EEG is


normal, an abnormality may be revealed from a sleep deprived recording or an
EEG within an hour of a clinical seizure, or a 24 hour ambulatory recording.
Typical EEG patterns in epilepsy are hypsarrhythmia, 3 cycle per second spike and
wave discharge, centro-temporal spikes. In addition, EEG recording during
piridoxine administration may be diagnostic. Burst-suppression pattern may be
seen in hypoxic ischaemic encephalopathy and in the myoclonic stage of SSPE,
and is the pattern desired when inducing a pentothyone coma for status
epilepticus.
Copyright 2002 Dr Colin Melville

22-A lesion of the prefrontal cortex can present with:


True / False

Disinhibition

Incorrect answer selected

Loss of appreciation of smell

Correct

Spatial orientation disruption

Incorrect answer selected

Speech problems

Correct

Abnormalities of micturition

Correct

Lesions of the cerebral cortex result in dementia, dysphagia, apraxia,


astereognosis and other forms of agnosia. If Broca's area is involved (inferior
frontal gyrus) then speech can be affected. Because of the diffuse radiation of
nerves beyond the internal capsule, upper motor neurone signs tend to be more
localised. A paresis confined to one limb or to one side of the face is likely than a

hemiplegia. Specific visual field defects may assist in localising the lesion within
the cerebral hemisphere.
Copyright 2002 Dr Colin Melville

23-Compared with truants- school refusers are more likely to:


True / False

Come from large families.

Correct

Have a specific reading disability.


Have over anxious parents.

Correct

Incorrect answer selected

Have behavioural problems at school.


Have a low IQ.

Correct

Correct

School phobia is when a child will not attend school, and occurs in 1-2% of
children. The mother/child relationship is one of hostile dependency, and a large
proportion of such children suffer from depression or anxiety, and in particular
separation anxiety disorder (SAD). There are unrealistic or persistent worries
about harm befalling primary care givers, reluctance to go to school or to sleep
without being near their parents, persistent avoidance of being alone, nightmares
involving themes of separation, and numerous somatic symptoms and complaints
subjective to stress. Children tend to come from middle to lower social classes.
Management involves treating underlying psychiatric problems, family therapy,
parent management training, and liaison with the school. Truancy is never
developmentally appropriate. They may skip school because they are afraid of
peers or teachers, or because of humiliation secondary to learning difficulties.
Occasionally, there is separation anxiety. There is often disorganisation within the
home or developing personality problems. Younger children may run away to get
back at their parents, whereas older children with nowhere to go are almost
always expressing serious underlying problems. This may be related to abuse or
neglect. Disagreements with parents, developing personality problems, may also
be implicated.
Copyright 2002 Dr Colin Melville

24-Regarding tricyclic overdose:


True / False

Sinus tachycardia is a recognised feature.


Pin-point pupils are usual.

Incorrect answer selected

Correct

Hypertension is possible due to adrenergic stimulation.


Emesis may be useful even in presentation is delayed.

Correct
Correct

Arrhythmias should be aggressively treated with drugs.

Correct

Tricyclics give rise to sinus tachycardia and conduction disorders. Their


anticholinergic effects also produce a dry mouth and blurred vision from pupillary
dilatation. Agitation, confusion, convulsions, drowsiness, coma, respiratory
depression and hypotension are also features. Emesis is no better than activated
charcoal. The latter should be given as early as possible after ingestion. Cardiac
monitoring is mandatory though arrhythmias should be treated conservatively
with sodium bicarbonate to correct metabolic acidosis. Convulsions may require
Diazepam or lorazepam.
Copyright 2002 Dr Colin Melville

25-The following are recognised causes of anterior uveitis:


True / False

Kawasaki Disease

Incorrect answer selected

Pauciarticular rheumatoid arthritis


Sarcoidosis

Correct

Herpetic keratitis
Trauma

Correct

Correct

Correct

The uveal tract consists of the inner vascular coat of the eye (iris, ciliary body,
choroid). Inflammation results from both infectious and non-infectious causes,
including a number of systemic diseases and trauma. Either part or the whole of
the uveal tract may be preferentially involved. Iritis may be caused by:
pauciarticular rheumatoid arthritis, Kawasaki Disease, sarcoidosis, secondary to
corneal disease (herpetic keratitis, bacterial or fungal corneal ulcer, corneal
abrasion, foreign body), traumatic iritis of iridocyclitis.
Choroiditis is inflammation of the posterior portion of the uveal tract, and
invariably involves the retina (choreoretinitis). Causes include toxoplasmosis,
histoplasmosis, CMV, sarcoid, syphilis, TB, toxocariasis. Secondary complications
include retinal detachment, glaucoma, phthisis.
Copyright 2002 Dr Colin Melville

26-Hyperprolactinaemia is associated with:


True / False

Polycystic ovary syndrome

Incorrect answer selected

Depression
Acromegaly

Incorrect answer selected


Correct

Hyperthyroidism

Correct

Premature thelarche

Correct

Hyperprolactinaemia may be manifest by a milk discharge from the breasts.


Causes include, prolactinoma, hypothyroidism (far increased TRH), and
occasionally craniopharyngioma. Treatment consists primarily of Dopamine
agonists such as bromocriptine, with a rare requirement for transsphenoidal
surgery. Prolactin levels will also be increased in normal puberty. In premature
thelarche (isolated breast development) plasma levels of LH and oestrodial are
below the limits of normal assays, but basal levels of FSH and their responses to
gonadotrophin releasing hormone stimulation are greater than those seen in
normal controls. In contrast, children with true precocious puberty secrete
predominately LH. Ultrasound examination of the ovaries reveals a normal size,
but a few small cysts are not uncommon.
Copyright 2002 Dr Colin Melville

27-The following are would confirm the diagnosis of insulin dependent diabetes
mellitus in a symptomatic 6-year-old child on the 25th centile for weight and
height:
True / False

A random plasma glucose of >6.5mmol/L


Finding of 3+ ketonuria
An HbA1c of 7.8%

Correct

Incorrect answer selected

Correct

A fasting plasma glucose of 6.5mmol/L


An abnormal glucose tolerance test

Incorrect answer selected

Incorrect answer selected

The diagnosis is usually relatively easy to confirm in a symptomatic child. A


random glucose of >11.1mmol/L or a fasting glucose of >7.0mmol/L would be
regarded as confirmatory. There is usually glycosuria in addition to ketonuria.
Isolated ketonuria suggests fasting. A raised glycosolated haemoglobin (HbA1c) is
also highly suggestive. A glucose tolerance test is rarely needed.
Copyright 2002 Dr Colin Melville

28-Regarding the development of the skull sutures:

True / False

Craniosynostosis is due to dysfunctioning osteoblasts

Incorrect answer

selected
The skull vault develops from mesenchyme

Incorrect answer selected

Scaphocephaly develops from premature fusion of the coronal suture


Correct
Occipital plagiocephaly is usually due to infant positioning

Correct

Scaphocephaly develops from premature fusion of the lamdoid suture


Correct

Craniosynostosis is defined as premature closure of the sutures, and may be


primary or secondary to failure of brain growth. The majority are idiopathic, with
genetic syndromes accounting for 10%. The skull bones develop from
mesenchyme, and craniosynostosis may be due to abnormal skull base
development disrupting suture development. Osteoblasts and osteoclasts are not
thought to be abnormal. Most cases are evident from birth, and a prominent bony
ridge from the affected suture (S) may be found, confirmed by skull x rays.
Specific forms include:

Scaphocephaly - sagittal suture.

Frontal plagiocephaly - coronal/sphenofrontal suture.

Occipital plagiocephaly - infant positioning.

Trigonocephaly - metopic suture.

Turricephaly - coronal/sphenofrontal sutures.

Single suture involvement is rarely associated with neurological problems.


Surgery is only needed for cosmetic appearance. Genetic disorders involving
multiple sutures include:

Crouzon syndrome

Apert syndrome

Carpenter syndrome

Chotzen syndrome

Pfeiffer syndrome.

Copyright 2002 Dr Colin Melville

29-Regarding hyperthyroidism in the adolescent:


True / False

Usually resolves with 2 years of medical treatment.

Incorrect answer

selected
Radioiodine treatment should be avoided because of the risk of genetic
damage and neoplasia.

Correct

May present with heart failure.

Correct

May be associated with increased stature and advanced bone age.


Correct
May present with chronic diarrhoea.

Correct

Neonatal hyperthyroidism is transient and is due to transferred thyroid


stimulating immunoglobulins from mother. Juvenile hyperthyroidism is from
Grave's Disease and is commonest in teenage girls.
Symptoms include:

Systemic: anxiety, increased appetite, diarrhoea, weight


loss; sweating, tremor, tachycardia, warm vasodilated
peripheries; goitre; rapid growth with advanced bone
age; behavioural problems.

Eye signs (not in variable): exophthalmos,


ophthalmoplegia, lid retraction, lid lag.

TREATMENT:

Medical: carbimazole, propylthiouracil. Betablockers can


be used for symptomatic relief, but not on their own.
50% relapse after 2 years when treatment is stopped.

Surgical: sub-total thyroidectomy.

Radioiodine: not considered to result in neoplasia or


genetic damage. Follow-up, and subsequent thyroxine
therapy, is often needed.

Copyright 2002 Dr Colin Melville

30-Regarding retinopathy of prematurity:


True / False

All babies who have received oxygen should have their eyes examined
until a corrected age of 44 weeks gestation.

Correct

It occurs in 50% of very low birth weight infants.


selected

Incorrect answer

Cryosurgery or laser therapy may be indicated for grade 3 or 4 disease.


Correct
It is first detected at the equivalent of 32-38 weeks gestational age.
Incorrect answer selected
It may progress extremely rapidly.

Correct

Retinopathy of prematurity (ROP, retinalentral fibroplasia) affects vessels at the


junction of the vascular and non-vascularised retina. Follow-up only needs to take
place until the retina is fully vascularised. Vascular proliferation may progress to
retinal detachment, fibrosis and blindness. It was previously the commonest
cause of blindness in children, but careful monitoring has reduced its incidence to
a much smaller proportion of very low birth weight infants (more in extremely
preterm infants). This is usually only grade 1 or 2 (reversible) rather than grade 3
or 4 (requiring treatment). It is first detected between 32 and 38 weeks of age,
but may progress rapidly. Severe visual impairment occurs in only 1% of low birth
weight infants.
Copyright 2002 Dr Colin Melville

31-Regarding the neonatal cardiac examination:


True / False

A chest x-ray and ECG are routinely indicated.

Correct

A grade 3/6 murmur is likely to be pathological.

Incorrect answer

selected
Cyanosis on crying may occur in the normal child.

Correct

Babies with weak femoral pulses should be re-examined a few hours


later.

Incorrect answer selected

A loud second heart sound may be normal.

Incorrect answer selected

Heart murmurs are often audible in the neonatal period, but resolve shortly
afterwards. Only occasionally are these caused by congenital heart disease.
Innocent murmurs are usually soft and either blowing or buzzing in character.
They are usually localised to the left sternal edge with no diastolic component and
no radiation. The heart sounds are normal and there are no accompanying thrills,
cyanosis nor abnormal pulses. Cyanosis may occur in the neonatal period when
crying due to shunting through a patent foramen ovale. A loud second heart
sound or weak femoral pulses should always be taken as significant until proven
otherwise, as neonates can deteriorate rapidly if they have significant heart
disease.
Copyright 2002 Dr Colin Melville

32-Regarding apnoea of prematurity:


True / False

The incidence of the idiopathic form varies inversely with gestational age.
Correct
It may be caused by neonatal sepsis.

Correct

It increases in frequency during non-REM sleep.


Bag and mask ventilation is often required.
May be treated with blood transfusion.

Correct

Incorrect answer selected

Incorrect answer selected

Periodic breathing must be distinguished from prolonged apnoeic pauses, since


the latter may be associated with serious illness. The aetiology includes:
1.

CNS depression (hypoglycaemia, meningitis, drugs, haemorrhage).

2.

Abnormal oxygen delivery (shock, sepsis, anaemia).

3.

Ventilatory defects (pneumonia, HMD, PFC, muscle weakness).

It is described as idiopathic when identifiable causes are absent. The idiopathic


form varies inversely in frequency with gestational age. It is rare on day 1, but
tends to start between day 2 and 7 of life. Sudden onset of severe apnoeas and
bradycardia deserve investigation. Apnoea is defined as cessation of breathing for
longer than 20 seconds or cessation of breathing is associated with cyanosis and
bradycardia.
Treatment includes cutaneous stimulation, with bag and mask ventilation for
recurrent or prolonged apnoea. Oxygen and Theophylline or caffeine should be
prescribed if they recur. The haemoglobin should be maintained with transfusion
or erythropoietin. Occasionally nasal CPAP is required for mixed or obstructive
apnoeas (assists in splinting the upper airway). Unless severe, recurrent, or
refractory to therapy, the prognosis is related to underlying conditions (IVH, BPD,
ROP etc.). Apnoea of prematurity usually disappears by 36 weeks post-conceptual
age, and does not predict future episodes of SIDS.
Copyright 2002 Dr Colin Melville

33-The following are characteristic of Bartter's Syndrome:


True / False

Secondary hyperaldosteronism
Hyperkalaemia

Correct

Correct

Metabolic acidosis

Correct

Reduced renal concentrating ability


Diarrhoea

Correct

Correct

Barter's Syndrome is a rare form of renal potassium wasting characterised by


hypokalaemia, normotension, and elevated renin and aldosterone levels. It is
occasionally autosomal recessive. There is hyperplasia of the juxtaglomerular
apparatus in most cases. It is postulated that the primary defect is in chloride
reabsorption in the ascending limb, resulting in sodium chloride excessively
presented to the distal tubule, with sodium reabsorption in exchange for
potassium, resulting in urinary sodium wasting. There is secondary stimulation of
prostaglandin synthesis, which activates the renin angiotensin aldosterone system
which exacerbates the renal potassium wasting. Growth failure, muscle
weakness, constipation, polyuria and dehydration are typical in younger children
with muscle weakness, cramps or carpopedal spasms present in older children.
The potassium is <2.5mmol/L, there is metabolic alkalosis, and
hyperammonaemia with hyperaldosteronism. There are high levels of urinary
potassium and chloride. The high urinary chloride level is helpful in distinguishing
it from similar presentations which have low urinary chloride levels, such as
liquorice, laxative, or diuretic use, persistent vomiting or diarrhoea,
pyelonephritis, or diabetes insipidus. Oral potassium and indomethacin may be
used.
eMedicine article
Copyright 2002 Dr Colin Melville

34-The following are recognised causes of non-organic failure to thrive:


True / False

Maternal eating disorders


Maternal depression
Poor housing

Incorrect answer selected

Correct

Correct

Poor social support

Incorrect answer selected

Lack of extended family

Correct

Under-nutrition is the final common pathway, usually from a combination of


inadequate or inappropriate feeding and psychosocial factors. Maternal depression
or psychiatric illness, and poor maternal education may contribute.
Accompanying, but probably not causative, factors include poor housing, poverty,
inadequate social support, and lack of extended family.
Copyright 2002 Dr Colin Melville

35-Human milk contains more of the following than cows' milk formula.
True / False

Lactalbumin
Calcium
Iron

Correct

Correct

Correct

Folic acid
Sodium

Correct
Correct

Cow's milk formula has been adjusted to have a similar amount of lactalbumin to
breast milk. There is less iron, but about half is bio-available compared with only
4% bio-availability of iron from cow's milk. The latter therefore is supplemented
with iron.
Copyright 2002 Dr Colin Melville

36-The following are characteristic of gluten enteropathy:


True / False

Dermatitis herpetiformis
Hyposplenism

Correct

Mucosal ulceration
Constipation

Correct

Incorrect answer selected

Correct

Increased risk of oesophageal carcinoma

Incorrect answer selected

The mode of presentation of coeliac disease is variable; the majority present with
diarrhoea. Children can have failure to thrive or vomiting as the only
manifestation. Perhaps as many as 10% of children referred to endocrinologists
for growth retardation without an endocrine or overt gastrointestinal disorder
have gluten sensitivity. Anorexia is common and may be the major cause of
weight loss or lack of weight gain. Infants with gluten-sensitive enteropathy are
often, but not always, clingy, irritable, unhappy children who are difficult to
comfort. In contrast to infants with cystic fibrosis, they are not interested in food,
although this is not always the case. Pallor and abdominal distension are
common. Large, bulky stools suggestive of constipation have been described in
some children with this condition. Digital clubbing can occur.

There is an increased prevalence of gluten-sensitive enteropathy in children with


selective IgA deficiency or diabetes mellitus compared with unaffected children.
Lymphocytic gastritis occurs rarely.
Appropriately-diagnosed gluten-sensitive enteropathy is a lifelong condition
requiring lifelong treatment.
There is an increased risk of oesophageal carcinoma, small bowel lymphoma,
melanoma and non-Hodgkin lymphoma in long-standing enteropathy, especially
with poor adherence to diet.
Dermatitis herpetiformis is seen most commonly in children 2-7 yr of age. It is
characterised by symmetric, grouped, small, tense, erythematous, stinging,
intensely pruritic papules and vesicles.
Aetiology is unknown; however, an association with gluten-sensitive enteropathy
is found in 75-90% of patients. Aggressive gluten challenge generally unmasks
the condition in the remainder of patients with dermatitis herpetiformis.
Copyright 2002 Dr Colin Melville

37-Undescended testis:
True / False

Is present in 5% of the male population at 6 months of age


Is often associated with a hernia

Correct

Incorrect answer selected

Should be operated on before the age of 6 years

Incorrect answer

selected
The testis and epididymis are usually not affected
The risk of malignancy is not significantly increased

Correct
Correct

Failure to find one or both testes in the scrotum may indicate any variety of congenital or
acquired conditions e.g. ectopic testes, maldescended testes , retractile or absent testes.
4.5% of males have an undescended testis at birth, falling to 0.8% bu 6 months.
Maldescended or ectopic testes and true undescended testicles are differentiated from
each other surgically. The ectopic testis has completed its descent through the inguinal
canal but ends up in a subcutaneous location. Spontaneous testicular descent does not
occur after the age of 1 year. Complications include infertility in adulthood, associated
hernias and torsion and tumour development in the affected testis (if not operated before
11 years). The patient with cryptorchidism has a 20-40% chance of developing
malignancy, and those most at risk are those untreated or those whose surgery was
carried out during or after puberty.

38-Lidocaine

True / False

inhibits the generation of neuronal impulses

Correct

inhibits the conduction of neuronal impulses

Correct

is only effective via parenteral administration

Correct

is effective for the treatment of both supraventricular and ventricular


tachycardia

Incorrect answer selected

is associated with haemolytic anaemia

Incorrect answer selected

Lidocaine is associated with the inhibtion of the conduction of neuronal impulses rather
than inhibting the generation of impulses. It an effective local anaesthetic agent when
used topically in urinary catheterisation for instance. It is associated with
methaemoglobinaemia.

39-The following enzyme defects are associated with a characteristic body


odour in infants:
True / False

Phenylalanine aminotransferase

Correct

Galactose-phosphate-uridyl transferase
Ornithine transcarbamylase deficiency
Fumaryl acetoacetase

Correct
Correct

Incorrect answer selected

Branched chain alpha ketoacid decarboxylase

Incorrect answer selected

The following inborn errors of amino acid metabolism are associated with
abnormal odours: Glutaric acidaemia type II (sweaty feet), hawkinsinuria
(swimming pool), isovaleric acidaemia (sweaty feet), maple syrup urine disease
(maple syrup), methionine malabsorption (cabbage), multiple carboxylase
deficiency (tomcat urine), oasthouse urine disease (hops-like), phenylketonuria
(mousy or musty), trimethylaminuria (rotting fish), tyrosinaemia (rancid, fishy or
cabbage-like). In addition, ketones may be smelt in diabetic ketoacidosis, and
some intermediary disorders of fatty acid metabolism can result in fishy smells.
The general rule is that if a child smells peculiar he requires a metabolic work-up.
Copyright 2002 Dr Colin Melville

40-In relation to Maple Syrup Urine Disease:


True / False

It may present with prolonged jaundice.

Correct

It usually presents with severe metabolic acidosis, hypoglycaemia and


seizures.

Correct

The urine has a characteristic fishy smell.

Correct

There is increased excretion of branch chain amino acids in the urine.


Correct
There is a high risk of early death during acute illnesses.

Correct

Maple Syrup Urine Disease usually presents with severe metabolic acidosis,
hypoglycaemia, and seizures. There is increased excretion of the branch chain
amino acids, leucine, isoleucine and valine, resulting in a characteristic maple
syrup smell in the urine. Delayed diagnosis can result in learning difficulties and
neurological dysfunction, and acute illnesses may precipitate a rapid decline.
Copyright 2002 Dr Colin Melville

41-Regarding bronchopulmonary dysplasia:


True / False

Children have higher than average weight gain because of fluid retention.
Correct
Children are susceptible to recurrent wheezing and chest infections.
Correct
Cor pulmonale is a recognised complication.
Doxopram therapy may be required.

Correct

Correct

It is associated with an increased incidence of congenital heart disease.


Incorrect answer selected

Bronchopulmonary dysplasia is thought to result from a combination of oxygen


toxicity, pressure (alveolar stress and volume trauma), hypocapnic
surponification, absorption atelectasis, and subsequent inflammation. This results
in persistent ventilatory and oxygen requirement with hypoxia, hypocarbia,
oxygen dependency, and the development of right sided heart failure. The chest
x-ray changes from complete opacification with air bronchogram to interstitial
emphysema. Histologically, there is alveolar coalescence with atelectasis,
interstitial oedema, and focal thickening of the basement membrane with
bronchial and bronchiolar mucosal metaplasia and hyperplasia. There is severe
maldistribution of ventilation. It is defined by oxygen dependency at 36 weeks
gestational age. Some patients require prolonged hospitalisation, but often they
can be discharged on home oxygen therapy. Right heart failure and viral
bronchiolitis are the major causes of death. The children also fail to thrive and
require nutritional supplementation.

Treatment includes nebulised bronchodilators, Theophylline, diuretics, fluid


restriction, and aggressive treatment of infection, and food supplementation.
CPAP may be required for tracheomalacia and Dexamethasone may be helpful in
reducing oxygen requirement and getting children off the ventilator.
Complications include: growth failure, transient psychomotor retardation, parental
stress, and nephrolithiasis, osteopenia, subglottic stenosis. Since new alveoli
continue to develop up to the age of about 7 or 8, children often improve with
age. Prognosis is good for children who have been weaned off oxygen prior to
discharge from the NICU, but prolonged ventilation, associated IVH, pulmonary
hypertension, cor pulmonale and oxygen dependence beyond 1 year of life are
poor prognostic signs. Long term broncho-reactivity may be a problem.
Copyright 2002 Dr Colin Melville

42-A full term 3.50kg baby, Apgar score 7 at one minute, develops abdominal distension and
bile-stained vomiting on the first day of life:
True / False

Neonatal necrotising enterocolitis is a likely diagnosis

Correct

It is quite safe to exclude pyloric stenosis from the differential

Incorrect

answer selected
It is important to examine the facial features of the baby in making a
diagnosis

Correct

If the baby has not passed meconium, meconium ileus is unlikely to be


the diagnosis

Correct

Rectal examination will not yield any useful information

Incorrect

answer selected
This question relates to a scenario of small bowel obstruction. Obstruction may be
complete or partial with complete obstructions leading to more dramatic presentations.
High obstructive lesions tend to be associated with vomiting and in this case the vomit is
bile stained suggesting the obstruction is distal to the ampulla of Vater.Lower lesions tend
to lead to abdominal distension and constipation. Congenital causes of duodenal
obstruction include duodenal atresia, malrotation , duodenal webs and annular pancreas.
Duodenal atresia is the most common of these lesions and frequently is found in patients
with Downs syndrome.It is therefore important to examine the facial features of the baby.
Neonatal necrotising enterocolitis is unlikely.Its cause is unknown.It primarily affects
premature or low birth weight infants.Clinical manifestations include abdominal distension,
gastric retention and bloody stools in the first two weeks of life. Meconium impaction may
cause an ileus and intestinal obstruction.

43-Regarding the Glasgow Coma Scale:


True / False

it may be modified for use in children


it provides a score from 3 to 15
is only used for head injuries
motor response is assessed

Correct

Correct

Correct
Correct

pupil response to light is a parameter

Correct

The Glasgow Coma Score (GCS) provides a score from 3 to 15, depending on the
BEST response observed in three different parameters (eye opening, verbal
response and motor response). The pupil response to light is not included. The
GCS was originally developed for use in brain trauma but is now used when
evaluating other cerebral insults. A modified version is used in young children

44-A 7-year-old girl presents with acute cough and wheeze, and is given
nebulised salbutamol. The following are indications for admission to hospital:
True / False

A peak flow rate of 80% of that predicted for height.


Continuing cough.

Correct

Respiratory rate of 40/min.


Saturation of 90% in air.
She looks tired.

Correct

Correct

Correct

Correct

Patients should be given nebulised bronchodilator therapy, and admitted if:


1.

They have not responded clinically.

2.

They are exhausted.

3.

Their peak flow is markedly reduced (severe is defined as <50% of


predicted or best value, and life-threatening as <33% of predicted
or best value).

4.

The oxygen saturation is reduced below 92%.

Copyright 2002 Dr Colin Melville

45-The neuroleptic malignant syndrome:

True / False

Can be effectively treated with bromocriptine

Incorrect answer selected

Has a similar clinical presentation to malignant hyperthermia


Has autosomal dominant inheritance

Correct

Correct

Is a contraindication to the use of suxamethonium

Incorrect answer

selected
Occurs in 6% of patients given neuroleptic drugs

Correct

The neuroleptic malignant syndrome (NMS) is a complication of neuroleptic drugs,


but suxamethonium is not a trigger agent. It has a clinical presentation similar to
malignant hyperthermia (MH), with a dantrolene responsive hypermetabolic state.
The main features of NMS include hyperthermia, muscle rigidity, altered
consciousness, and autonomic disturbances.
NMS occurs after 0.5-1.4% of neuroleptic exposures and signs may appear hours
to months after exposure. It has a mortality rate of approximately 15%. It is not
an inherited disorder and is unrelated to familial susceptibility to MH. Drug
treatment options include dantrolene and bromocryptine. Early management is
similar to MH and cessation of the neuroleptic treatment is essential.

46-A 3-year-old boy presents with fever and headache. He has received oral
Amoxicillin for 3 days. The following CSF findings exclude a partially treated
meningitis:
True / False

Negative gram stain

Correct

A CSF glucose of 45% of blood glucose


A white cell count of 50

Incorrect answer selected

Incorrect answer selected

A negative CSF culture

Correct

Negative Kernig's Sign

Correct

The assessment of children with suspected bacterial meningitis who have already
received antibiotic therapy is a diagnostic conundrum. This applies to about 2550% of children, so it is an important problem. Partial treatment may reduce the
incidence of positive CSF gram stains to <60%, and it also reduces the ability to
grow the bacteria, particularly meningococcus. CSF glucose, protein, neutrophils
and bacterial antigen testing or PCR should be completely unaffected.
Copyright 2002 Dr Colin Melville

47-In the differential diagnosis of cyanosis in the newborn:


True / False

A ground glass appearance confirms hyaline membrane disease.

Correct

An arterial PO2 above 13 kPa suggests non-cyanotic congenital heart


disease.

Incorrect answer selected

A normal cardiothoracic ratio excludes total anomalous pulmonary venous


drainage.

Correct

Persistent hypoxia in a child of 34 weeks gestation suggests transposition


of the great arteries.

Incorrect answer selected

A saturation of 91% in the right hand and 85% in the right leg suggests
significant shunting.

Correct

A ground glass appearance is not specific for hyaline membrane disease: Group B
Streptococcus can also present in this way. In the nitrogen wash-out test, an
arterial PO2 <13kPa in 100% oxygen suggests cyanotic congenital heart disease.
Other causes of cyanosis, such as pneumonia or hyaline membrane disease, can
normally be oxygenated beyond this level. A normal cardio-thoracic ration is
characteristic of total anomalous pulmonary venous drainage. This is because the
obstruction is extra-cardiac. The usual reason for hypoxia in a pre-term baby is
hyaline membrane disease or infection, with cardiac causes being rarer. A
significant difference in oxygen saturation between the pre-ductal and post-ductal
circulations suggests that hypoxic blood is shunting from the pulmonary artery to
the aorta.
Copyright 2002 Dr Colin Melville

48-Complete third nerve palsy causes:


True / False

Ptosis

Correct

Divergent squint
Constrictive pupil

Correct
Incorrect answer selected

Loss of corneal reflex


Decreased sweating

Incorrect answer selected


Correct

Causes of third nerve palsy include:

Congenital: developmental anomaly, birth trauma.

Acquired: intracranial neoplasm, aneurysm, trauma,


post-viral syndrome, migraine.

Third nerve palsy causes an exotropia (divergent squint). There may be


downward deviation of the affected eye and complete or partial ptosis of the

upper lid. This occurs because of the unopposed lateral rectus and superior
oblique actions. If the internal branch of the third nerve is involved, pupillary
dilatation also occurs. Eye movements are limited nasally, in elevation, and in
depression.
The corneal reflex is mediated by V and VII.Copyright 2002 Dr Colin Melville

49-Eczema herpeticum:
True / False

Is invariably fatal if untreated.

Correct

Usually has an indolent onset.

Incorrect answer selected

Only a single crop of vesicles usually appear.

Correct

Is typically associated with a high fever for over a week.

Incorrect

answer selected
Is more severe in reactivation disease.

Incorrect answer selected

Eczema herpeticum is the result of primary infection of eczematous skin with HSV.
The severity varies from mild to fatal. There is usually an abrupt onset with crops
appearing over 7-9 days. These may become coalesced. Typically, the child has a
high fever for 7 days, and recurrent attacks can occur. Death can result from
physiological disturbances (loss of fluid electrolytes and protein through the skin)
or dissemination of the virus to brain and other organs or from secondary
bacterial sepsis.
Copyright 2002 Dr Colin Melville

50-The following conditions can be successfully treated by surgery on the fetus:


True / False

Diaphragmatic hernia
Hydrocephalus

Correct

Correct

Hydronephrosis

Correct

Pleural effusion

Correct

Hypoplastic left heart syndrome

Correct

Fetal surgery is being attempted in specialised centres, but the results have been
generally disappointing. Attempts to repair diaphragmatic hernia have resulted in
preterm delivery, with no definite benefit on lung development. Pleural effusions
have been successfully drained, but shunting of urinary obstructions have been
disappointing. Treatment of hydrocephalus has largely been abandoned because
the survivors are severely disabled, and the treatment of stenotic heart valves
have only been successful in case reports. Currently, routine surgery is, therefore,
not an option.
Copyright 2002 Dr Colin Melville

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