Paeds 8A

Question 1

What are the five autism spectrum disorders?

Answer 1

Autism
Asperger’s syndrome
Rett syndrome
Childhood disintegrative disorder
Pervasive developmental disorder not otherwise specified (PDD-NOS)

Question 2

Which disease in infants can present very similarly to necrotising enterocolitis?

Answer 2

Hirschsprung’s enterocolitis

Question 3

What does ‘10% glucose’ mean with regards to fluids?

Answer 3

10 g glucose per 100 mL

Question 4

What is the investigation of choice for suspected SUFE?

Answer 4

Frog-lateral hip X-ray

NOTE: they will have limited hip flexion and abduction

Question 5

List some complications of Henoch-Schonlein Purpura.

Answer 5

Acute renal impairment 
Intussusception
Arthritis involving ankles and knees commonly
Testicular pain
Pancreatitis

Question 6

What is an important first step in the management of acute lymphoblastic leukaemia?

Answer 6

Prevent tumour lysis syndrome
Hyperhydration
Start allopurinol

Question 7

Which antibiotic should be used for neutropaenic sepsis?

Answer 7

Tazocin

Question 8

List some clinical features of space-occupying lesions.

Answer 8

Headaches are worse when lying down 
Morning vomiting 
Headaches may cause night-time waking 
Change in mood or personality 
Change in educational performance
Focal neurology (e.g. weakness, visual field defect)

Question 9

Which age group is most likely to experience a febrile convulsion?

Answer 9

< 6 years

Question 10

What are some key features of frontal seizures?

Answer 10

Involves motor and pre-motor cortex

May lead to clonic movements

Question 11

What are some key features of temporal lobe seizures?

Answer 11

Aura Automatisms (e.g. lip smaking) 
Impaired consciousness

Question 12

Which diagnostic tests can be used for mumps and rubella?

Answer 12

Serology from blood or oral fluid sample

Question 13

Which investigations may be used in a child with short stature?

Answer 13

MId-parental height 
Random GH measurement 
Insulin tolerance test 
CT/MRI head scan 
Bone age (using DEXA or wrist X-ray)

Question 14

What are some complications of scarlet fever?

Answer 14

Otitis media 
Acute sinusitis/mastoiditis 
Streptococcal pneumonia
Meningitis 
Endocarditis 
Osteomyelitis 
Rheumatic fever 
Streptococcal glomerulonephritis

Question 15

In an acute asthma attack, which other medications may be used if the patient is unresponsive to nebulised SABA and ipratropium bromide?

Answer 15

Magnesium sulphate
Aminophylline
IV salbutamol

IMPORTANT: ECG and electrolytes should be monitored

Question 16

Who will receive the children’s flu vaccine?

Answer 16

Annually as a nasal spray in september/october for all children aged 2-9 years

Question 17

When is the 4 in 1 preschool booster given and which vaccines does it contain?

Answer 17

Around 3 years and 4 months 
Diphtheria
Tetanus 
Pertussis 
Polio

Question 18

When is the 3 in 1 teenage booster given and which vaccines does it contain?

Answer 18

14 years
Diphtheria
Tetanus
Polio

Question 19

What type of murmur does a VSD cause?

Answer 19

Pansystolic heard loudest at the lower left sternal edge (fifth intercostal space)

Question 20

What type of murmur does a PDA cause?

Answer 20

Continuous machinery murmur inferior to the left clavicle

Question 21

What should you do first if a child presents to paediatric A&E with infectious diarrhoea, whooping cough or symptoms suggestive of MMR?

Answer 21

Put in a side room

Question 22

What is spinal muscular atrophy?

Answer 22

Autosomal recessive disorder of anterior horn cells
Leads to progressive weakness and wasting of skeletal muscles
Due to mutation in SMN1

NOTE: Werdnig-Hoffman disease is type 1 SMD

Question 23

What is Charcot-Marie-Tooth disease?

Answer 23

Hereditary motor sensory neuropathy
Causes symmetrical, slowly progressive distal muscle wasting
May present in preschool children with tripping from bilateral foot drop

Question 24

What is a key feature of CSF studies in Guillain-Barre syndrome?

Answer 24

High protein

Question 25

What is myotonic dystrophy?

Answer 25

Autosomal dominant trinucleotide repeat (CTG) disorder

Presents with hypotonia, feeding difficulties, respiratory difficulties and abnormal facies (in older children)

Question 26

List some causes of ataxia.

Answer 26

Friedreich ataxia
Ataxia telangiectasia
Cerebellar tumour 
Cerebellar agenesis/dysgenesis 
Post-infectious cerebellitis
Toxins (e.g. ethanol)

Question 27

What is Friedreich ataxia and how does it present?

Answer 27

Autosomal recessive triplet repeat in FXN gene
Worsening ataxia and dysarthria
Distal wasting of lower limbs
Absent reflexes
Pes cavus (associated with Charcot-Marie-Tooth)
Impairment of proprioception

Question 28

What are the main clinical features of ataxia telangiectasia?

Answer 28

Telangiectasia in the conjunctiva
Mild delay in motor development 
Oculomotor problems 
Coordination problems 
Complex eye movement disorders

Question 29

What are the main features of NF1?

Answer 29

6 or more cafe au lait spots 
Neurofibromas 
Axillary freckling 
Optic glioma 
Lisch nodules 
1st degree relative with NF1

Question 30

What are the main features of NF2?

Answer 30

Multiple schwannomas
Meningiomas
Ependyomas
Bilateral acoustic neuroma

Question 31

List the main clinical features of tuberous sclerosis.

Answer 31

Cutaneous: ash leaf depigmentation, shagreen patches (roughened), angiofibromata (across the bridge of the nose)

Neurological: developmental delay, epilepsy, intellectual disability

Question 32

What are the main features of Sturge-Weber syndrome?

Answer 32

Port wine stain in the distribution of the ophthalmic division of the trigeminal nerve
Similar lesion intracranially
May have epilepsy, intellectual disability and contralateral hemiplegia

Question 33

List some neurodegenerative conditions that occur in children.

Answer 33

Lysosomal storage disorders 
Peroxisome enzyme defects 
Wilson disease 
Tay Sachs 
Gaucher 
Niemann Pick

Question 34

What is adrenoleukodystrophy?

Answer 34

Group of disorders caused by peroxisomal defects
Peroxisomes are important for breaking down fatty acids
Neonatal and X-linked forms

Question 35

What are some clinical features of growing pains?

Answer 35

Age 3-12
Symmetrical pain not limited to joints
Pain never present at the start of the day
Physical activities not limited (no limp)
Normal physical examination