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True / False
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
Correct
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.
is a bowel commensal
Correct
Correct
Correct
Correct
Correct
3-Penicillins:
True / False
Are bactericidal
Correct
Incorrect answer
Correct
Correct
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.
Correct
Correct
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
Correct
Correct
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.
Correct
Correct
Incorrect
answer selected
Correct
Correct
Correct
Correct
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
Correct
Correct
Anencephaly
Pyloric stenosis
Correct
Myotonic dystrophy
Ankylosing spondylitis
Correct
Correct
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.
Haemolytic anaemia
Correct
Correct
Correct
Correct
Correct
Correct
Correct
Correct
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.
Short stature
Correct
Mental retardation
Karyotype XXY
Correct
Correct
Correct
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.
Correct
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
16-Bone age:
True / False
Correct
Correct
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.
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
Correct
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.
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
Correct
Correct
Correct
Correct
Optic atrophy
Correct
Retinal haemorrhage
Neck stiffness
Fever
Correct
Correct
This is the
correct answer
Hypsarrhythmia - infantile spasms.
Correct
Incorrect
answer selected
3 cycle per second spike and wave discharge - petit mal seizures.
Correct
Centro-temporal spikes - benign rolandic epilepsy.
Correct
Disinhibition
Correct
Speech problems
Correct
Abnormalities of micturition
Correct
hemiplegia. Specific visual field defects may assist in localising the lesion within
the cerebral hemisphere.
Copyright 2002 Dr Colin Melville
Correct
Correct
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
Correct
Correct
Correct
Correct
Kawasaki Disease
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
Depression
Acromegaly
Hyperthyroidism
Correct
Premature thelarche
Correct
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
Correct
Correct
True / False
Incorrect answer
selected
The skull vault develops from mesenchyme
Correct
Crouzon syndrome
Apert syndrome
Carpenter syndrome
Chotzen syndrome
Pfeiffer syndrome.
Incorrect answer
selected
Radioiodine treatment should be avoided because of the risk of genetic
damage and neoplasia.
Correct
Correct
Correct
TREATMENT:
All babies who have received oxygen should have their eyes examined
until a corrected age of 44 weeks gestation.
Correct
Incorrect answer
Correct
Correct
Incorrect answer
selected
Cyanosis on crying may occur in the normal child.
Correct
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
The incidence of the idiopathic form varies inversely with gestational age.
Correct
It may be caused by neonatal sepsis.
Correct
Correct
2.
3.
Secondary hyperaldosteronism
Hyperkalaemia
Correct
Correct
Metabolic acidosis
Correct
Correct
Correct
Correct
Correct
Correct
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
Dermatitis herpetiformis
Hyposplenism
Correct
Mucosal ulceration
Constipation
Correct
Correct
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.
37-Undescended testis:
True / False
Correct
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
Correct
Correct
Correct
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.
Phenylalanine aminotransferase
Correct
Galactose-phosphate-uridyl transferase
Ornithine transcarbamylase deficiency
Fumaryl acetoacetase
Correct
Correct
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
Correct
Correct
Correct
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
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
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
Correct
Incorrect
answer selected
It is important to examine the facial features of the baby in making a
diagnosis
Correct
Correct
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.
Correct
Correct
Correct
Correct
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
Correct
Correct
Correct
Correct
Correct
2.
3.
4.
True / False
Correct
Correct
Incorrect answer
selected
Occurs in 6% of patients given neuroleptic drugs
Correct
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
Correct
Correct
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
Correct
Correct
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
Ptosis
Correct
Divergent squint
Constrictive pupil
Correct
Incorrect answer selected
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
Correct
Correct
Incorrect
answer selected
Is more severe in reactivation disease.
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
Diaphragmatic hernia
Hydrocephalus
Correct
Correct
Hydronephrosis
Correct
Pleural effusion
Correct
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