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YEAR 5: Specialties (Laz) > Paeds 8 > Flashcards

Paeds 8 Flashcards

1
Q

What are the five autism spectrum disorders?

A 
Autism
Asperger’s syndrome
Rett syndrome
Childhood disintegrative disorder
Pervasive developmental disorder not otherwise specified (PDD-NOS)
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2
Q

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

A

Hirschsprung’s enterocolitis

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3
Q

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

A

10 g glucose per 100 mL

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4
Q

What is the investigation of choice for suspected SUFE?

A

Frog-lateral hip X-ray

NOTE: they will have limited hip flexion and abduction

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5
Q

List some complications of Henoch-Schonlein Purpura.

A 
Acute renal impairment 
Intussusception
Arthritis involving ankles and knees commonly
Testicular pain
Pancreatitis
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6
Q

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

A

Prevent tumour lysis syndrome
Hyperhydration
Start allopurinol

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7
Q

Which antibiotic should be used for neutropaenic sepsis?

A

Tazocin

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8
Q

List some clinical features of space-occupying lesions.

A 
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)
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9
Q

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

A

< 6 years

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10
Q

What are some key features of frontal seizures?

A

Involves motor and pre-motor cortex

May lead to clonic movements

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11
Q

What are some key features of temporal lobe seizures?

A 
Aura Automatisms (e.g. lip smaking) 
Impaired consciousness
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12
Q

Which diagnostic tests can be used for mumps and rubella?

A

Serology from blood or oral fluid sample

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13
Q

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

A 
MId-parental height 
Random GH measurement 
Insulin tolerance test 
CT/MRI head scan 
Bone age (using DEXA or wrist X-ray)
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14
Q

What are some complications of scarlet fever?

A 
Otitis media 
Acute sinusitis/mastoiditis 
Streptococcal pneumonia
Meningitis 
Endocarditis 
Osteomyelitis 
Rheumatic fever 
Streptococcal glomerulonephritis
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15
Q

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

A

Magnesium sulphate
Aminophylline
IV salbutamol

IMPORTANT: ECG and electrolytes should be monitored

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16
Q

Who will receive the children’s flu vaccine?

A

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

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17
Q

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

A 
Around 3 years and 4 months 
Diphtheria
Tetanus 
Pertussis 
Polio
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18
Q

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

A

14 years
Diphtheria
Tetanus
Polio

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19
Q

What type of murmur does a VSD cause?

A

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

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20
Q

What type of murmur does a PDA cause?

A

Continuous machinery murmur inferior to the left clavicle

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21
Q

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

A

Put in a side room

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22
Q

What is spinal muscular atrophy?

A

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

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23
Q

What is Charcot-Marie-Tooth disease?

A

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

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24
Q

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

A

High protein

25
Q

What is myotonic dystrophy?

A

Autosomal dominant trinucleotide repeat (CTG) disorder

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

26
Q

List some causes of ataxia.

A 
Friedreich ataxia
Ataxia telangiectasia
Cerebellar tumour 
Cerebellar agenesis/dysgenesis 
Post-infectious cerebellitis
Toxins (e.g. ethanol)
27
Q

What is Friedreich ataxia and how does it present?

A

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

28
Q

What are the main clinical features of ataxia telangiectasia?

A 
Telangiectasia in the conjunctiva
Mild delay in motor development 
Oculomotor problems 
Coordination problems 
Complex eye movement disorders
29
Q

What are the main features of NF1?

A 
6 or more cafe au lait spots 
Neurofibromas 
Axillary freckling 
Optic glioma 
Lisch nodules 
1st degree relative with NF1
30
Q

What are the main features of NF2?

A

Multiple schwannomas
Meningiomas
Ependyomas
Bilateral acoustic neuroma

31
Q

List the main clinical features of tuberous sclerosis.

A

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

Neurological: developmental delay, epilepsy, intellectual disability

32
Q

What are the main features of Sturge-Weber syndrome?

A

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

33
Q

List some neurodegenerative conditions that occur in children.

A 
Lysosomal storage disorders 
Peroxisome enzyme defects 
Wilson disease 
Tay Sachs 
Gaucher 
Niemann Pick
34
Q

What is adrenoleukodystrophy?

A

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

35
Q

What are some clinical features of growing pains?

A

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

36
Q

how does subluxation of the patella present?

A

Sudden lateral dislocation of the patella
Very painful
Feeling of instability/giving way
Requires reduction and immobilisation

37
Q

How many joints must be affected to be classified as polyarticular juvenile idiopathic arthritis?

A

More than 4

38
Q

Describe the difference in the iron content of breast milk and cows milk.

A

Breast milk - low iron content, high absorption

Cows milk - high iron content, low absorption

39
Q

List some clinical features of Edwards syndrome.

A 
Low-set ears 
Small chin 
Microcephaly
Overlapping fingers 
Rocker-bottom feet 
Cardiac defects (VSDs) 
Renal anomalies 
Learning disability 
90% death within 1 year
40
Q

List some clinical features of Patau syndrome.

A 
Structural brain defects 
Small eyes
Polydactyly
Cardiac/renal malformations 
Death within 1 year
41
Q

List some clinical features of Klinefelter’s syndrome.

A 
Most common cause of male hypogonadism
Small firm testes 
Tall 
Behavioural problems 
Delayed speech 
Gynaecomastia
42
Q

List some clinical features of fragile X syndrome.

A 
Moderate learning difficulties (most common genetic cause of learning difficulties)
Macrocephaly 
Large testes 
Large ears 
Long face 
Prominent mandible and forehead
43
Q

How is fragile X syndrome inherited?

A

X-linked dominant

Female carriers may have mild learning difficulties

44
Q

What is Still’s disease?

A

Systemic form of JIA thought to be of autoimmune origin

Intermittent pyrexia
Salmon-pink rash
Aches and pains of joints and muscles

45
Q

List some clinical features that are common to all congenital TORCH infection.

A 
Low birth weight 
Prematurity 
Jaundice 
Microcephaly 
Seizures 
Anaemia 
Failure to thrive 
Encephalitis
46
Q

Which medication is used to chelate lead in lead poisoning?

A

EDTA (ethylene diamine tetraacetic acid)

NOTE: it present with encephalopathic features (e.g. behavioural problems, pica) and causes a hypochromic microcytic anaemia with basophilic stippling

47
Q

Which other condition can present very similarly to acute epiglottitis?

A 
Retropharyngeal abscess (causes fever, drooling, dysphagia and stridor)
Typically has a longer history than acute epiglottitis (over a few days)
48
Q

Which murmur is typically heard in tetralogy of Fallot?

A

Ejection systolic murmur heard best in the 3rd intercostal space with a single heart sound

49
Q

Which tumour markers can be used for neuroblastoma?

A

Urinary catecholamines

50
Q

What is a craniopharyngioma?

A

Rare type of brain tumour derived from pituitary gland embryonic tissue

51
Q

Which tissue does neuroblastoma arise from?

A

Neural crest tissue in the adrenal medulla and sympathetic nervous system

NOTE: MIBG scans are used to identify neurblastoma tissue

52
Q

Which tissue does nephroblastoma arise from?

A

Embryonal renal tissue

53
Q

What is the most common type of malignant bone tumour in children?

A

Osteosarcoma

NOTE: Ewing is common in younger children

54
Q

Describe the inheritance pattern of retinoblastoma.

A

Autosomal dominant

Located on chromosome 13

55
Q

What is a common benign cause of proteinuria in children?

A

Orthostatic (postural) proteinuria

Proteinuria is only found when the child is upright during the day

56
Q

List some features of Fanconi syndrome.

A 
Polydipsia and polyuria
Salt depletion and dehydration 
Hyperchloraemic metabolic acidosis 
Rickets 
Faltering growth
57
Q

List some causes of heart failure in neonates.

A

Hypoplastic left heart syndrome
Critical aortic valve stenosis
Severe coarctation of the aorta
Interruption of the aortic arch

58
Q

Which congenital heart defect is most commonly seen in DiGeorge syndrome?

A

Interrupted aortic arch

Others include persistent truncus arteriosus, TOF and VSD

59
Q

What are some features suggestive of atypical UTI?

A 
Seriously ill
Poor urine flow 
Abdominal mass 
Raised creatinine 
Septicaemia
Failure to respond to antibiotic treatment 
Infection with non-E. coli organisms

YEAR 5: Specialties (Laz) (12 decks)

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