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Year 5 > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

80% of CF cases in UK are due to what

A

Mutation in delta508 on long arm of chr7

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2
Q

What organisms can colonise CF patients?

A

Staphylococcus aureus
Pseudomonas aeruginosa
Burkholderia cepacia
Aspergillus

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3
Q

What is the site of origin of stridor?

A

Extrathoracic

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4
Q

What is the site of origin of wheeze?

A

Intrathoracic

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5
Q

Stridor

A

Predominantly inspiratory - indicates obstruction to airflow in the extrathoracic airways down to the level of the thoracic inlet

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6
Q

What do you do if a case of whooping cough is suspected?

A

Prescribe oral azithromycin within first 21 days of symptoms and inform public health England

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7
Q

Complications of bordatella pertussis

A

subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures

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8
Q

Mx for GOR

A
  1. 30 degree position during feeds
  2. Infant sleep on back
  3. Small and mode frequent feeds, ensure no overfeeding
  4. Trial of thickened formula OR trial of alginate eg gaviscon
  5. Trial of PPI or H2RA if refusal of feeds, faltering growth or distressed behaviour
  6. Metoclopromide in specialist care
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9
Q 
Side effects of anti-epileptics 
Valproate 
Lamotrigine 
Vigabatrin
Levetiracetam
Carbamazepine
A

Valproate - weight gain, hair loss, rarely liver failure
Lamotrigine - rash
Vigabatrin - restriction of visual fields, sedation, behavioural disturbance
Levetiracetam - anorexia
Carbamazepine - visual disturbance

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10
Q

A 10-year-old boy is brought to the paediatric outpatient department for a review of his height. He was found to be on the 0.4th centile and his mid-parental height is the 98th centile. He also has widely spaced nipples, wide carrying angle, hypogonadism, pulmonary stenosis and developmental delay. What is the most likely diagnosis?

A

Noonans syndrome

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11
Q

Gain of function mutation in MAPK pathway (PTPN11) causes which condition

A

Noonan’s syndrome

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12
Q

What problem with the arms is seen in 50% of Noonan’s syndrome patients?

A

Cubitus valgus

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13
Q

Turners syndrome is associated with an increased likelihood of which GI condition

A

Coeliac disease

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14
Q

Cardiac associations with DS

A

AVSD, ventricular septal defect, tetralogy of fallot

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15
Q

GI associations with DS

A

Duodenal atresia, Hirschprung’s disease

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16
Q

Endocrine associations with DS

A

Hypothyroidism, Addison’s disease, T1DM

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17
Q

Ocular problems associated with DS

A

Cataracts

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18
Q

Malignancy associated with DS

A

Leukaemia

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19
Q

Musculoskeletal problems associated with DS

A

Atlanto-axial instability

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20
Q

What are DS patients at risk of later in life

A

Dementia

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21
Q

Treatment of status epilepticus

A

IV Phenytoin

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22
Q

Treatment for seizures in the community lasting more than 5mins

A

Buccal midazolam

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23
Q

How to treat febrile seizures?

A

If meningitis is excluded (need to ensure if first febrile convulsion under 12mths), then likely to be viral, so dress the child lightly and give regular anti-pyretics.

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24
Q

Diagnostic for absence epilepsy on EEG

A

Three spike waves per second in all leads

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25
Q

Presentation of temporal epilepsy

A

Warning aura or sensation, impaired conciousness or unresponsiveness and a focal seizure which may spread to become generalised tonic clonic

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26
Q

Presentation of Wolff-Parkinson white syndrome

A

Spontaneous onset re-entry tachycardia or SVT which may lead to dizziness, SOB and sometimes fainting. (ECG shows delta wave).

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27
Q

Presentation of MILD hypoxic ischaemic injury in a neonate

A

Irritability, startle responses, poor feeding, hyperventilation

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28
Q

Presentation of MODERATE hypoxic ischaemic injury in a neonate

A

Features of mild +++ altered tone or reduced movement and seizure activity

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29
Q

Hypoglycaemia in a neonate - range?

A

<2.5mmol/L

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30
Q

Multiple sclerosis presentation

A

Neurology consistent with multiple lesions eg arm/leg weakness (but can switch sides), diplopia, urinary incontinence, nerve palsy.

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31
Q

MRI findings - multiple sclerosis

A

multiple, hyperintense, inflammatory, white matter lesions

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32
Q

MRI findings - tuberous sclerosis

A

Subependymal calcifications and hypointense white matter lesions or tubers

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33
Q

Parvolex =

A

NAC

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34
Q

Slow-relaxing reflexes may be a sign of

A

systemic illness eg hypothyroidism or LMN diseases eg GBS

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35
Q

Features of UMN lesions

A

Increased reflexes
Increased tone
Upgoing plantars
Decreased power

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36
Q

Signs of raised ICP

A

Headache
Morning vomiting
Sunsetting eyes (pressure on CNIII, IV and VI)
Papilloedema

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37
Q

Which syndrome is Carbamazepine associated with?

A

SLE

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38
Q

Hypsarrthymia on EEG

A

chaotic and disorganized brain electrical activity with no recognizable pattern, associated with infantile spasms, west syndrome, tuberous sclerosis

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39
Q

Triad of West’s syndrome

A

Hypsarrthymia on EEG
Infantile spasms
Developmental plateau/difficulties

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40
Q

Any baby born with a port wine stain in trigeminal region or hair should have?

A

MRI brain to look for intracranial hemangiomas - they are at risk of epilepsy

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41
Q

Management of headache

A

Rescue eg
Analgesia (paracetamol and NSAIDs)
Antiemetics (prochlorperazine)
Triptans (nasal preparation available for children)
Physical treatments (e.g. cold compress, warm pads, topical forehead balms)

Prophylactic eg 
Sodium channel blockers (topiramate, valproate) 
Beta-blockers (propranolol) 
Tricyclics (pizotifen) 
Acupuncture

Psychosocial Support eg identify stressors and relaxation technique

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42
Q

Treatment of a seizure

A

If lasts >5mins rectal diazepam or buccal midazolam

- can repeat rectal diazepam once after 5mins if seizure hasn’t stopped

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43
Q

Carbamazepine can make which type of seizures work

A

Myoclonic and absence

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44
Q

Features of cerebellar disease

A 
Dysdiadochokinesia
Dysmetria
Difficulty holding posture
Wide-based gait
Ataxic
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45
Q

Features of damage to basal ganglia

A

Difficulty in initiating movement with fluctuating tone
Dystonia or dyskinesia
Chorea
Athetosis

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46
Q

Encephalomyelitis

A

Acute inflammation of spinal cord and brain

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47
Q

Gower’s sign

A

Seen in children with myopathies, need to turn prone to rise from supine position and use legs to push self up

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48
Q

What is ophthalmoplegia?

A

Paralysis or weakness of eye muscles eg seen in myasthenia gravis

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49
Q

Abx used fo treatment of bacterial meningitis (normal, allergy to penicillin, anaphylaxis to penicillin)

A

Ceftriaxone
Allergy -> cefotaxime
Anaphylaxis -> chloramphenicol

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50
Q

What is spinal muscular atrophy caused by?

A

Mutations in the survival motor neurone (SMN1) gene

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51
Q

CMT1A mutations account for 70-80% of which type of disease?

A

Charcot Marie Tooth disease

= symmetrical, slowly progressive distal muscular wasting

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52
Q

Type 1 spinal atrophy is also called

A

Werdnig-Hoffman disease. AR inheritance. Progressive weakening and wasting of skeletal muscles. Likely to die from resp failure within 12mths.

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53
Q

Charcot Marie tooth disease is?

A

Symmetrical, progressive distal muscular wasting. Caused by mutations in myelin genes.

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54
Q

What is dysautonomia and what condition is it associated with?

A

Malfunction of autonomic nervous system associated with GBS, includes:

  • Hypotension, HTN
  • Bradycardia, Tachycardia
  • Urinary retention
  • Ileus
  • Loss of sweating
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55
Q

How is GBS managed?

A

Supportive, may need resp support. IVIG or plasma exchange.

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56
Q

What infections is Bell’s palsy associated with?

A

HSV or Lyme disease

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57
Q

Why do you need to exclude HTN in paeds Bell’s palsy cases?

A

Association with renal failure and coarctation of aorta

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58
Q

How does myasthenia gravis present?

A

Ptosis, Ophthalmoplegia, loss of facial expression, difficulty chewing, generalised (esp. proximal) weakness

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59
Q

How does myaesthenia gravis present?

A

Ptosis, Ophthalmoplegia, loss of facial expression, difficulty chewing, generalised (esp. proximal) weakness

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60
Q

What is the tensilon test and for what condition would you do it?

A

Give IV edrophonium bromide to improve symptoms in myasthenia gravis

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61
Q

Management of myasthenia gravis

A
  1. Pyridostigmine, neostigmine (cholinesterase inhibitors)
  2. Immunosuppressive therapy
  3. Immune-modulating drugs eg prednisolone, azathioprine, mycophenolate mofetil, biologics
  4. Thymectomy is indicated if thymoma is present
  5. Plasma exchange may be used for crises
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62
Q

Heliotrope rash is associated with which condition

A

Dermatomyositis

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63
Q

Presentation of dermatomyositis

A

Fever + Misery + symmetrical muscle weakness + heliotrope rash (likely to have high creatinine kinase)

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64
Q

Management of dermatomyositis

A

Physiotherapy to prevent contractures
Steroids (2yrs)
Immunosuppressants eg methotrexate, ciclosporin
Risk of cancer

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65
Q

Myotonia

A

Delayed muscle relaxation after sustained muscle contraction

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66
Q

Condition caused by triple nucleotide expansion of CTG in DMPK gene

A

Dystrophia Myotonica type I

AD inheritance

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67
Q

Why might death occur in dystrophia myotonica type I?

A

Cardiac conduction defects

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68
Q

How might dystrophia myotonica type I present?

A

Hypotonia, feeding/resp difficulties, talipes, oligohydramnios, thin ribs, reduced foetal movements

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69
Q

Miller fisher syndrome

A

A rare, acquired nerve disease characterised by abnormal muscle coordination, paralysis of the eye muscles and absence of the tendon reflexes. Variant of GBS.

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70
Q

A triplet repeat in FXN gene causes a lack of what protein and subsequently what disease?

A

Frataxin protein, Friedreich ataxia

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71
Q

Presentation of Friedreich ataxia

A

o Worsening ataxia and dysarthria
o Distal wasting in the lower limbs
o Absent reflexis
o Pes cavus
o Impairment of joint position and vibration sense (posterior columns affected)
o Extensor plantars (pyramidal involvement)
o Optic atrophy
o Cerebellar component becomes more apparent with age

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72
Q

Volume of males testes by puberty

A

Around 4ml

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73
Q

Volume of adult male testes

A

12.5-19ml

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74
Q

Constitutional delay in puberty

A

Much more common in males. Isolated finding of delay in skeletal growth ie height. May see delayed puberty and growth spurt. Typically seen around puberty.

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75
Q

Constitutional delay in puberty

A

Much more common in males. Isolated finding of delay in skeletal growth ie height.

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76
Q

Typical presentation of muscular dystrophy in young child

A

Clumsy and walk with waddling gait. May cry when standing for long periods of time.

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77
Q

What age is a child who builds a tower of 6 blocks, feeds themselves with a spoon and starts to potty train?

A

18 months

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78
Q

What age is a child who feeds themselves with a spoon and starts to potty train?

A

18 months

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79
Q

A child walking unsteadily with 2-3 words is likely to be how old?

A

12 months

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80
Q

A child who scribbles and walks well is likely to be how old?

A

15 months

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81
Q

How old is a child who can build a tower of 8 blocks?

A

2.5 years

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82
Q

Wasted buttocks is a classic sign of which disease in a child?

A

Coeliac

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83
Q

Most common cause of CAH

A

21-hydroxylase deficiency

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84
Q

How can CAH present?

A

May present with ambiguous genitalia or bilateral undescended testicles. Kids are at risk of a salt-losing adrenal crisis (vomiting, weight loss, floppy unwell infant), typically around 1–3 weeks of age.

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85
Q

Presentation of Kleinfelter’s

A

XXY, tall stature, delayed puberty, mild learning difficulties

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86
Q

Normal time taken for testes to descend

A

3 months, most by 6months. Worried after 9months.

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87
Q

Obesity

A

May present with weight AND height above average. May see gynaecomastia with obesity.

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88
Q

How to predict height of child? (Predicts adult height of the child ± two standard deviations)

A

Mean parental height +7cm for boys, -7cm for girls.

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89
Q

Recurrent coughs and colds in a child who has speech delay might suggest?

A

Hearing difficulties eg glue ear

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90
Q

How to manage meningococcal septicaemia?

A

High flow O2 and IV ceftriaxone. Check for dehydration (eg CR time, dry mucous memranes etc) as patient most likely needs IV fluid bolus.

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91
Q

Definition of shock

A

Inadequate perfusion of tissues which is insufficient to meet cellular metabolic needs

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92
Q

Why is IM adrenaline given in anaphylaxis?

A

To prevent the capillary leak of fluid into airway tissues (Otherwise oedema closes the airway off)

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93
Q

What is distributive shock?

A

Rapid shifts in fluid to the interstitium result in intravascular hypovolaemia

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94
Q

How does hypovolaemic shock present?

A

Cold, pale and poorly perfused patient. (usually caused by haemorrhage or dehydration).

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95
Q

What is obstructive shock caused by?

A

Blockage of blood flow from the heart eg cardiac tamponade, fluid in pericardial sac compressing the heart or tension pneumothorax.

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96
Q

Paediatric BLS guidelines for CPR

A

Two inflation breaths per 15 chest compressions. If the child is intubated: continuous inflation breaths about 10-12 per minute and compressions 100-120 per minute

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97
Q

Management of status epilepticus

A

IV phenytoin (don’t give more than two doses of barbituates due to respiratory depression risk). If child’s epilepsy is managed with phenytoin then give IV phenobarbital instead.

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98
Q

Presentation of iron overdose

A

Two phase illness: 1) early vomiting and diarrhoea due to gastric irritation, may present with haematemesis or malaena. Then period of 24hrs improvement. 2) Then deterioration with liver failure, drowsiness and coma. Liver failure can produce hypoglycaemia and seizures.

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99
Q

Presentation of paracetamol overdose

A

Gastric irritation, Hx child took tablets, Liver failure on day 3-5

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100
Q

TCA overdose presentation

A

Tachycardia, Anticholinergic symptoms (dry mouth, blurred vision, agitation). Shock with seizures or coma. Severe metabolic acidosis (indication for giving IV bicarbonate).

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101
Q

Anaphylaxis management

A

IM adrenaline

  • call anaesthetist to open airway
  • salbutamol nebs
  • IV access to give hydrocortisone and antihistamine

When discharging be aware of possible Type IV hypersensitivity, so send home with two further doses of prednisolone over next 2 days to cover this.

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102
Q

Presentation of biliary atresia

A

Prolonged jaundice in neonate, acholic stools, dark urine

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103
Q

Biliary atresia increases the risk of

A

Liver cirrhosis

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104
Q

How can GBS manifest itself?

A

Sepsis, pneumonia, meningitis, urinary tract infection and septic arthritis

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105
Q

Most common pathogens in neonate

A

group B Streptococcus, E. coli and Listeria monocytogenes

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106
Q

What is the genetic cause of Prader Willi syndrome?

A

Imprinting. To get PW syndrome, there is loss of part of paternal chromosome 15.

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107
Q

What are examples of two syndromes do microdeletions cause?

A

DiGeorge’s and Williams’

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108
Q

Trisomy 13 =

A

Patau’s

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109
Q

Trisomy 18 =

A

Edward’s syndrome

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110
Q

What defects do glycogen storage disorders present with?

A

Liver, Muscle and Cardiac

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111
Q

Galactosaemia cause

A

Deficiency in galactose-1-phosphate uridyl transferase

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112
Q

Presentation of galactosaemia

A

Illness with lactose-containing milks (ie could be seen in exclusively formula fed babies), with vomiting, cataracts and recurrent episodes of E Coli sepsis.

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113
Q

How can fructose intolerance present?

A

Metabolic acidosis and vomiting

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114
Q

Presentation of phenylketonuria

A

Developmental delay when child is older and musty smelling urine (not acute illness)

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115
Q

Inheritance of Marfan’s syndrome

A

Autosomal dominant, affects fibrillin gene. (Connective tissue disorder affecting musc, ocular and cardiac systems).

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116
Q

Hypophosphataemic rickets

A

X-linked dominant condition, can present with genu varum and short stature

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117
Q

Phenylketonuria inheritance

A

Autosomal recessive

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118
Q

Phenylketonuria presentation and cause

A

Metabolic condition resulting in defect in enzyme phenylalanine hydroxylase. If undetected can present with musty smelling urine, seizures, microcephaly and learning difficulties.

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119
Q

What are patients with oculocutaneous albinism at higher risk of?

A

Defects in melanin production so higher risk of skin malignancy

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120
Q

Both DMD and Becker’s are due to defects in which gene?

A

Dystrophin

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121
Q

Features of Down Syndrome

A

Dysmorphic facies eg low set ears, epicanthic folds, protruding tongue, flattened nasal bridge, single palmar crease, sandal gap toes

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122
Q

Management of salt losing crisis in CAH

A

IV dextrose and IV hydrocortisone

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123
Q

What is haematochezia?

A

Passage of fresh blood through anus (usually with stools)

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124
Q

What is pneumatosis intestinalis? What is it a sign for?

A

Gas cysts in the bowel wall. Sign for NEC

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125
Q

What are the 3 key radiographical findings for NEC?

A
  • Intestinal dilation
  • Portal venous gas
  • Pneumatosis intestinalis
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126
Q

Pneumonitis - what is it?

A

Occurs as part of meconium aspiration syndrome - is due to irritation to the lung caused by chemicals in meconium

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127
Q

What congenital conditions are associated with cleft palate?

A

DS or DiGeorge’s syndrome

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128
Q

VACTERL association?

A 
Vertebral
Anal imperforation
Cardiac
Tracheo-oesophageal fistula
Renal and Limb abnormalities
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129
Q

What is CHARGE syndrome?

A 
Colomba
Heart defects
Atresia choanae
Retardation of growth and development
Genitourinary abnormalities
Ear abnormalities syndrome
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130
Q

What in the history would suggest incoordinated swallowing reflex?

A

Concern about the neurological or neuromuscular status

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131
Q

A term baby is awaiting his discharge check when you are called to see him at 10 hours of age. His mother reports that he has turned a dusky colour and is not
as alert as he has been. On examination he has central cyanosis, pulse 150bpm regular, and both brachial and femoral pulses are palpable. He has normal heart
sounds with no murmur. His oxygen saturations are 65per cent in air. What is the most likely underlying diagnosis?

A

Transposition of the great arteries

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132
Q

What needs to co-exist/occur for compatibility of life with transposition of the great arteries?

A

A mixing defect eg VSD, ASD, persistent ductus arteriosus. (NB: when managing these patients, give prostaglandin infusion to keep duct open then perform surgery)

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133
Q

Complication of gastroschisis

A

Dehydration, hypothermia, electrolyte losses

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134
Q

What are infants of diabetic mothers at risk of?

A

Congenital malformation eg if glucose control was poor around conception, post-natally hypoglycaemia and macrosomia

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135
Q

What is gastroschisis?

A

Herniation of bowel through a defect in the anterior abdominal wall of the developing foetus (NB: if with membrane then called exomphalos)

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136
Q

Causes of asymmetrical IUGR

A

Placental insufficiency eg late in pregnancy insult like maternal diabetes or preeclampsia

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137
Q

Causes of symmetrical IUGR

A

Chromosomal anomaly
Maternal smoking
Congenital infection
Maternal alcohol use

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138
Q

How to manage croup?

A

Give oral dexamethasone

If complication eg heart disease, BPD, neuromusc disorders, immunodeficiency etc present then admit to ward

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139
Q

Common cause of croup

A

Parainfluenza virus

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140
Q

Causative organism in epiglotittis

A

Haemophilus influenzae type B

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141
Q

Epiglottitis management

A

Don’t touch child, leave with parent, secure airway

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142
Q

Pertussis management

A

Oral azithromycin (doesn’t affect course of disease though) + can go back to sch AFTER 48HRS on the abx

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143
Q

Pertussis management

A

Oral azithromycin (doesn’t affect course of disease though) + can go back to sch AFTER 48HRS on the abx (or 3wks after symptoms if child doesn’t qualify for Abx)

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144
Q

What is the centor criteria used for?

A

To know whether to prescribe Abx in tonsillitis or not. Need score of 3/4/5

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145
Q

Jenny, a 3yr old girl, was at a village fete. She suddenly developed swollen cheeks and lips and a widespread urticarial rash. She is rushed to the nearby GP surgery. How do you treat her?

A

IM adrenaline, 150mcg. (as 150mcg if <6yrs)
If doesn’t resolve, repeat after 5mins.
Then do IV fluid challenge, chlorpheniramine and hydrocortisone.

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146
Q

What reflex is lost in testicular torsion?

A

Cremasteric reflex

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147
Q

What sign to do with the testes suggests epididymo-orchitis over testicular torsion?

A

Pain relived on elevation of affected testis

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148
Q

Presentation of pyloric stenosis

A

Projectile vomiting, 1 month year old, more common in males (vomit will be normal milky colour). Child may be lethargic, pass less urine and stool. Child may often be hungry after the vomit.

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149
Q

What is there usually a history of in patients who present with NEC at term?

A

Birth asphyxia or

Severe growth restriction.

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150
Q

What is the pathological process of Coeliac disease?

A

Villous atrophy of the small intestine

and corresponding malabsorption and malnutrition

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151
Q

Wasting of the buttocks is a sign of

A

Coeliac disease

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152
Q

Kayser–Fleischer and (at least 5) spider naevi are a sign of what disease?

A

Wilson’s disease

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153
Q

What is the inheritance of Wilson’s disease?

A

Autosomal recessive

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154
Q

What are some red flags for a constipation history?

A 
Growth failure
A history of delayed passage of meconium
Distended abdomen with vomiting
Anal pathology
Neurological complications affecting the lower limbs
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155
Q

Management of chronic constipation (NICE guidelines)

A

1) maintenance therapy (medical bowel disimpaction with non-stimulant laxatives) with polyethene glycol
++++ encourage pt to increase fluid intake, dietary fibre and exercise
++++ behavioural management eg star charts once stool is softer to help avoid toilet avoidance

Continue medication at maintenance dose for several weeks after regular bowel habit is established – this may take several months

2) Add a stimulant laxative if that doesn’t work

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156
Q

Swelling in the groin which is associated with a tense abdomen in a vomiting baby?

A

Incarcerated inguinal hernia (needs urgent surgical management)

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157
Q

Abdominal distention + stained nasogastric aspirates in a premature infant =

A

NEC

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158
Q

Treatment of umbilical hernias in paeds

A

None, they will self resolve

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159
Q

A 5-year-old girl is brought to accident and emergency with a 24-hour history of vomiting and diarrhoea and now her eyes and skin have gone very yellow. She has been taking oral rehydration salts and is still passing urine. She is normally healthy and there is no family history of jaundice. On examination her heart rate is 130 and respiratory rate is 26. She is alert, warm and well perfused. The chest is clear, heart sounds are normal
and the abdomen is soft with a 2cm liver edge. What is the diagnosis and what should the management be?

A

Hepatitis A
- Take bloods to test for liver function, hepatitis and U&Es; inform the Health Protection Agency and discharge home with follow-up to review results
(she is not dehydrated so doesn’t need IV fluids and is well enough to go home and not infect other patients)

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160
Q

What are some common autoimmune diseases that T1DM patients suffer from?

A

Coeliac
Graves’
(screen for them yearly)

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161
Q

Antibodies in autoimmune hepatitis

A

ASMA (70%) and ANA (80%)

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162
Q

Presentation fo autoimmune hepatitis

A

Women, aged 10-30, usually presents with chronic liver failure or acute hepatitis

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163
Q

Presentation of mesenteric adenitis

A

Diagnosis of exclusion, may see 1/2 day Hx of vomiting, abdominal pain and fever. Preceding viral illness. ==> Enlarged mesenteric glands on USS

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164
Q

Causes of PR bleeding

A

Constipation with anal fissure
Intussuception
Meckel’s diverticulum
Bacterial gastroenteritis

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165
Q

Toddler’s diarrhoea

A

Likely due to intestinal immaturity, usually resolves by 5. History should be relatively normal.

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166
Q

A younger child with IBD is more likely to have…

A

Crohn’s disease

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167
Q

Mouth ulcers are associated with which IBD

A

Crohn’s disease

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168
Q

Associations of UC

A

Erythema nodosum
Pyoderma gangrenosum
Arthritis
Spondylitis

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169
Q

Histology finding of Crohn’s

A

Non-caseating granuloma and full thickness lesions.

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170
Q

What is choanal atresia?

A

Congenital blockage of the nasal airway which

presents with newborn cyanosis and respiratory distress, as infants are obligate nose breathers.

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171
Q

Most common cause of non-passing of meconium

A

Meconium plug syndrome - manage with anal stimulation with glycerine chip

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172
Q

Presentation of cow’s milk protein intolerance

A

Worsening eczema, vomiting and diarrhoea, failure to thrive, irritability, colic

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173
Q

Hyper IgE syndrome

A

= Job’s syndrome

AD immunodeficiency associated with severe eczema and skin boils

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174
Q

Wiskott–Aldrich syndrome

A

X-linked recessive disorder associated with thrombocytopenia, eczema and lymphopenia

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175
Q

Hypochloraemic metabolic alkalosis with low plasma sodium and potassium

A

Pyloric stenosis vomiting

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176
Q

O/E a olive shaped mass in the RUQ suggests?

A

Pyloric stenosis

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177
Q

What would you see on abdominal USS for pyloric stenosis?

A

Increase in pyloric muscle thickness, increase in transverse diameter

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178
Q

What procedure fixes a pyloric stenosis?

A

Ramstedt pyloromyotomy (cut made in longitudinal and circular muscles of pylorus)

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179
Q

Management of pyloric stenosis

A
  1. IV fluid resuscitation
    - Essential to correct the fluid and electrolyte disturbance before surgery
    - Provide at 1.5 x maintenance rate with 5% dextrose + 0.45% saline
  2. Ramstedt pyloromyotomy
    - Cut in longitudinal and circular muscles of the pylorus
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180
Q

What is McBurney’s point tenderness?

A

Guarding in the RIF, indicates appendicitis (may be absent if appendix is retrocaecal)

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181
Q

Management of appendicitis

A
  1. NBM from time of diagnosis
  2. IV fluids should be started
  3. Admit
  4. Appendicectomy
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182
Q

Most common cause of gastroenteritis in developed countries

A

Rotavirus

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183
Q

Most common bacterial cause of gastroenteritis in developed countries

A

Campylobacter jejuni

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184
Q

Prozotoan causes of gastroenteritis

A

Cryptosporidium

Giardia

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185
Q

Normal presentation of Hirschsprungs

A

Neonatal period, failure to pass meconium within 24hr, vomiting (bile stained) + abdo distention.

Occasionally can present with severe, life threatening H enterocolitis = usually due to C difficile

If later in life - chronic constipation (and growth failure)

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186
Q

Findings on full-thickness rectal biopsy in Hirschsprungs

A

Absence of ganglion cells + large ACh-esterase (+) nerve trunks

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187
Q

Management of Hirschsprungs

A

Bowel irrigation + anorectal pullthrough

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188
Q

Non-caseating epithelioid cell granulomata on histology

A

Crohn’s

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189
Q

What is the most common cardiac abnormality in Turner’s syndrome?

A

Bicuspid aortic valve (aortic stenosis is 2nd)

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190
Q

Which IBD does smoking make worse?

A

Crohn’s

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191
Q

Treatment for UC

A

o Colectomy with an ileostomy or ileojejunal pouch

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192
Q

What is the MOST COMMON cause of intestinal obstruction in infants after the neonatal period?

A

Intussusception

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193
Q

What is Meckel’s diverticulum?

A

Ileal remnant of the omphalomesenteric duct

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194
Q

For what is a Ladd procedure performed?

A

Intestinal malrotation eg Volvulus etc: involves detorting the bowel and surgically dividing the Ladd bands

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195
Q

Acrodermatitis enteropathica

A

Autosomal recessive metabolic disorder characterised by the malabsorption of Zinc resulting in diarrhoea, inflammatory rash around the mouth +/- anus and hair loss.

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196
Q

What are the risks of not adhering to a gluten free diet in Coeliac disease?

A

1) Micronutrient deficiency, esp osteopaenia

2) Bowel cancer, especially small bowel lymphoma

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197
Q

What type of malignancy are patients with Coeliac disease at risk of?

A

Small bowel lymphoma

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198
Q

What products are removed from the diet in Coeliac disease?

A

Wheat, Barley, Rye

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199
Q

What is the Marsh criteria used for?

A

Grading of small intestine tissue histologically in Coeliac

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200
Q

What are inguinal hernias usually caused by?

A

A persistent processus vaginalis

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201
Q

What is the difference between an obstructed vs a strangulated hernia?

A

Both are irreducible, but the blood supply is compromised in strangulated hernias vs not compromised in obstructed hernias

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202
Q

Treatment of umbilical hernias

A

If <1.5cm then most spontaneously resolve (80%), if not then after 4-5years surgical repair due to incarceration risk

If >1.5cm then unlikely to spontaneously repair –> elective repair at 2-3years

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203
Q

What are some signs of hepatic dysfunction?

A 
§ Coagulopathy(PT>20s) not correctable with vitamin K 
§ Hypoglycaemia
§ Hypoalbuminaemia
§ Increasing bilirubin
§ Hyperammonaemia
§ High lactate
§ Electrolyte disturbance
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204
Q

How to treat cerebral oedema?

A

Fluid restriction and mannitol (3% saline)

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205
Q

What tests are used to look for H pylori?

A

CLO test and C-13 breath test

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206
Q

Investigations for suspected H pylori

A

CLO and C-13 breath test - looks for urease release from the Bac
Gastric antral biopsies
Stool testing for H pylori

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207
Q

What eradication therapy can be used for detected H pylori? (nb: not peptic ulceration)

A

Metronidazole + Amoxicillin OR Clarithromycin

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208
Q

What are symptoms of functional dyspepsia?

A 
Epigastric pain
Early satiety
Bloating
Post-prandial vomiting 
Delayed gastric emptying (due to gastric dysmotility)
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209
Q

Rose spots indicate which infection

A

Salmonella typhoid

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210
Q

What are some complications of CMV Infection discovered during pregnancy?

A

Hearing loss,Low-birth-weight, hydrocephalus, Microcephaly, seizures

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211
Q

Which part of the bowel does Crohn’s commonly affect?

A

Terminal ileum

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212
Q

Possible primary causes of constipation

A
  • Hirschsprung disease
  • Lower spinal cord problems
  • Anorectal abnormalities
  • Hypothyroidism
  • Coeliac disease
  • Hypercalcaemia
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213
Q

What is Apley’s criteria?

A

If recurrent abdo pain is peri-umbilical, doesn’t wake the child up from sleep, lasts no more than a few hours and has not been present for a considerable period of time then no Ix are necessary + non-organic cause is more likely.

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214
Q

What are the most common causes of meningitis in the UK?

A

Streptococcus pneumoniae

Neisseria meningitidis

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215
Q

If meningitis is caused by the classic bacteria ie strep pneu. or neisseria menin. then what results would you expect on an LP?

A

Raised WCC
Normal protein
Glucose <2/3 of serum glucose

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216
Q

What is the causative organism likely for meningitis in the immunosuppressed? eg co-exisiting HIV infection

A

Cryptococcus neoformans

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217
Q

Parasaetemia levels in malaria?

A

<1% mild, 1-2% moderate, >2% esp higher warrants careful observation

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218
Q

When should you perform a splenectomy in malaria?

A

If there is evidence of a ruptured spleen eg hypovolaemic shock and peritonism

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219
Q

Features of kawasaki disease

A

Fever, bilateral non-purulent conjunctivitis, red fingers/toes, swollen lymph nodes, dry/cracked lips, rash (blanching, erythematous, confluent). (can have cardiac involvement in form of coronary artery aneurysms)

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220
Q

What should one consider if there are petechial spots in the presence of fever and clinical signs of shock?

A

Meningococcal sepsis (commence IV ceftriaxone immediately and consider the need for a fluid bolus)

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221
Q

How does scarlet fever present?

A

Sandpaper rash, STRAWBERRY TONGUE, erythematous mucous membranes, high fever, swollen lymph nodes, sore throat

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222
Q

What are the complications of periorbital cellulitis?

A 
Visual loss
Abscess
Septicaemia
Orbital cellulitis
**Meningitis** (spread to CSF around optic nerve)
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223
Q

Presentation of lymphoma

A

Enlarged lymph node, increasing in size, dry cough/prolonged fever/night sweats/weight loss, reduction in appetite, CXR may show mediastinal mass

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224
Q

What should one consider if there are petechial spots in the presence of fever and clinical signs of shock?

A

Meningococcal sepsis

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225
Q

Definition of juvenile idiopathic arthritis

A

6 weeks of joint pain and swelling which persists after other diagnoses have been excluded

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226
Q

What is the most common congenital infection acquired in pregnancy?

A

CMV

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227
Q

What are mothers routinely screened for in early pregnancy?

A

Rubella
HIV
Syphilis
Hepatitis B

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228
Q

Which congenital infection is associated with congenital heart defects>

A

Rubella

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229
Q

Rose spots may indicate which type of infection

A

Salmonella typhi eg Typhoid

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230
Q

What is late complement deficiency and what illness does it usually cause?

A

Deficiency of C5-C9. It is typically characterised by meningitis due to often recurrent meningococcal infections eg N. meningitidis.

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231
Q

Whats the difference between a simple and a complex seizure?

A

Simple <5mins, self-resolving

Complex >15mins, requires medical treatment

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232
Q

What is DiGeorge syndrome caused by?

A

22q11 deletion

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233
Q

What is Wiskott/Aldrich syndrome?

A

X-linked recessive disease: involves microthrombocytopenia, atopic dermatitis and immunodeficiency resulting in recurrent infections

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234
Q

What is late complement deficiency and what illness does it usually cause?

A

Deficiency of C5-C9. It is typically characterised by meningitis due to often recurrent meningococcal infections.

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235
Q

In what situation would you give Adrenaline IV?

A

Cardiac arrest protocol (give IV or through endotracheal tube)

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236
Q

Why do you have to admit and observe a child who has had an anaphylactic reaction?

A

There is a biphasic response - second reaction may occur 6-12hrs later despite no contact with the allergen

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237
Q

What is erythema multiforme?

A

Rash described with target lesions with a surrounding red ring. Can be caused by drugs eg penicillin or sulphonamides, infection eg atypical pneumonia or idiopathic

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238
Q

Features of DiGeorge’s syndrome?

A 
Cleft palate
Aortic arch and other cardiac abnormalities eg tetralogy of fallot/VSD
Thymic hypoplasia
Typical facial features
Hypocalcaemia
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239
Q

What are the likely immune test results for SCID patients?

A

Low B cells
Low T cells
Low immunoglobulins

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240
Q

Presentation of cow’s milk protein intolerance

A

Diarrhoea (can be bloody) upon introduction to cow’s milk based formula or breast milk if mother drinks/eats dairy products. also associated with eczema on the face

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241
Q

How does fructose intolerance present?

A 
Vomiting
Hypoglycaemia
Failure to thrive 
Hepatomegaly
Jaundice
Renal complications
Severe metabolic acidosis
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242
Q

Features of DiGeorge’s syndrome?

A 
Cleft palate
Aortic arch and other cardiac abnormalities
Thymic hypoplasia
Typical facial features
Hypocalcaemia
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243
Q

What is ELISA used for?

A

Detection of known proteins eg autoantibodies or bacterial/viral detection

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244
Q

What are the two most important complications of eczema?

A

Cellulitis -> bacterial superinfection (Gram + cocci eg staph and strep)
Eczema herpeticum –> (HSV)

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245
Q

Causative organisms bacterial meningitis in 1mth-6yr olds

A

NHS
Neisseria meningitidis
Haemophilus influenzae
Streptococcus pneumoniae

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246
Q

Patients who are immunocompromised eg on steroids or chemo are at risk of infections caused by which organisms?

A

Mycobacterium tuberculosis

Pneumocystis jiroveci

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247
Q

What does a septic screen include?

A 
Blood culture  
FBC including differential WCC 
Acute phase proteins (e.g. CRP) 
Urine sample  
If indicated: 
CXR, LP, Rapid antigen screen on blood/CSF/urine, Meningococcal and pneumococcal PCR on blood/CSF, PCR for viruses in CSF (especially HSV and enteroviruses)
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248
Q

Causative organisms for bacterial meningitis in neonates-3mth year olds

A

GEL
Group B Strep
E. Coli
Listeria monocytogenes

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249
Q

Causative organisms bacterial meningitis in 1mth-6yr olds

A

NHS
Neisseria meningitidis
Haemophilus influenzae
Streptococcus pneumoniae

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250
Q

To whom is prophylactic treatment given for bacterial meningitis?

A

Rifampicin or ciprofloxacin given to ALL household contacts for meningococcal meningitis or H.influenzae infection

Household contacts of patients with group C meningococcal meningitis should be vaccinated with the meningococcal group C vaccine

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251
Q

What type of drug should NOT be used in meningococcal septicaemia?

A

Steroids

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252
Q

What organisms are associated with the complication of subdural effusion in meningitis?

A

H. influenzae

Pneumococcal meningitis

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253
Q

Complications of meningitis

A
  1. Hearing impairment
  2. Local vasculitis: focal neuro deficits or cranial nerve palsies
  3. Local cerebral infarction: focal or multifocal seizures, later on epilepsy
  4. Subdural effusion
  5. Hydrocephalus
  6. Cerebral abscess
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254
Q

To whom is prophylactic treatment given for bacterial meningitis?

A

Rifampicin or ciprofloxacin given to ALL household contacts for meningococcal meningitis or H.influenzae infection

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255
Q

Viral causes of meningitis

A

o Enteroviruses
o EBV
o Adenoviruses
o Mumps

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256
Q

Causative organism in Lyme disease meningitis

A

Borrelia burgdorferi

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257
Q

Risk factors for early onset neonatal sepsis

A
  • Prolonged or premature rupture of membranes

* Chorioamnionitis (mother has fever during labour)

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258
Q

What is a complication of toxoplasmosis ?

A

Chorioretinitis

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259
Q

Most common pathogen causing late onset neonatal sepsis?

A

Coagulase negative staph

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260
Q

Risk factors for late onset neonatal sepsis

A
  • Indwelling central venous catheters for parenteral nutrition
  • Invasive procedures that break skin barrier
  • Tracheal tubes
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261
Q

Risk factors for early onset neonatal sepsis

A
  • Prolonged or premature rupture of membranes

* Chorioamnionitis (mother has fever during labour)

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262
Q

What is the choice of aminoglycoside in the UK and for what pathogen class is it used?

A

Gentamicin

Gram negatives

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263
Q

What are the investigations for food allergy?

A

IgE mediated:

1) Clinical investigation
2) Skin prick tests
3) Patch testing
4) IgE antibodies

Non-IgE mediated:
More based on clinical Hx and observation. Can involve endoscopy + intestinal biopsy if necessary.

If any doubt in both: gold standard = EXCLUSION of relevant food under clinical observation

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264
Q

How does IgE mediated food allergy usually present?

A

Urticaria
Facial swelling
Can progress to anaphylaxis

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265
Q

How does non-IgE mediated food allergy usually present?

A

Diarrhoea
Vomiting
Abdominal swelling
Faltering growth (if left for a long time)

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266
Q

What is food protein induced enterocolitis syndrome?

A

Results from non-IgE mediated food allergy causing blood in the stools due to proctitis in the first few weeks of life or severe vomiting which can lead to shock

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267
Q

Management of cow’s milk allergy

A
  1. Consider referral to secondary care for skin prick/specific IgE antibody blood test
  2. Strict cow’s milk elimination diet for at least 6mths, until child is 9-12mths old (if breast fed then mum avoid dairy, if formula then use hypoallergenic kind, if older children then no dairy)
    3) Regularly monitor growth + consider reassessing for tolerance + ‘Milk ladder’ for reintroduction
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268
Q

Most effective treatment for allergic rhinitis

A

Intranasal corticosteroid

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269
Q

What to do with ANY FEBRILE CHILD with a purpuric rash ?

A

Treat immediately with
IM penicillin OR
IV 3rd generation cephalosporin

THEN ADMIT TO HOSPITAL

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270
Q

What tool can be used to help identify urticaria severity?

A

Urticaria Activity Score (UAS7)

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271
Q

Children who are at risk of streptococcal infections (e.g. due to hyposplenism due to SCD or something like nephrotic syndrome) should be given what?

A

Daily prophylactic penicillin to prevent infection

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272
Q

What are examples of some streptococcal infections that are immune mediated?

A

Post-streptococcal glomerulonephritis

Rheumatic fever

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273
Q

Since the meningitis vaccine in the UK, what are most cases caused by?

A

Group B Meningococci

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274
Q

Children who are at risk of streptococcal infections (e.g. due to hyposplenism) should be given what?

A

Daily prophylactic penicillin to prevent infection

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275
Q

What are examples of some streptococcal infections that are immune mediated?

A

Post-streptococcal glomerulonephirtis

Rheumatic fever

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276
Q

What is the management of impetigo?

A

Advice: usually heals without scarring, stop sch until lesions crusted/dried over.
Medical:
topical > topical fusidic acid 3-4day for 7days
oral >flucloxacillin 4/day for 7days

Bullous: oral flucloxacillin or clarithromycin/erythromycin

277
Q

What is Nikolsky’s Sign?

A

Areas of epidermis will separate on gentle pressure, leaving denuded areas of skin

278
Q

Treatment of staphylococcal scalded skin syndrome

A

IV antibiotics (flucloxacillin)

Analgesia

Monitoring hydration and fluid balance

279
Q

What are the human herpes viruses?

A 
HSV1 
HSV2 
VZV 
CMV 
EBV 
HHV-6 
HHV-7 
HHV-8
280
Q

What prevention can be used for primary VZV in immunocompromised patients?

A

• Human varicella zoster immunoglobulin if they come into contact with VZV eg pregnant mother not exposed in contact with child exposed etc

281
Q

What treatment should be considered for chickenpox and when?

A

Oral acyclovir 800 mg 5/day for 7 days

  • Within 24hrs onset of the rash
  • If the chickenpox is severe
282
Q

What are three serious complications of chicken pox?

A

Secondary bacterial infection
Encephalitis
Purpura fulminans

283
Q

What prevention can be used for primary VZV in immunocompromised patients?

A

• Human varicella zoster immunoglobulin if they come into contact with VZV eg pregnant mother not exposed to child

284
Q

What treatment should be considered for chickenpox and when?

A

Oral aciclovir 800 mg 5/day for 7 days

  • Within 24hrs onset of the rash
  • If the chickenpox is severe
285
Q

What does EBV have a tropism for?

A

B lymphocytes and epithelial cells of the oropharynx

286
Q

What medication should be avoided in children with EBV?

A

Ampicillin and amoxicillin (can cause a florid maculopapualr rash)

287
Q

Signs of severe anaemia

A 
Impaired consciousness 
Pallor --> severe anaemia 
Acidotic breathing 
Resp distress 
Hypoglycaemia 
Prostration
288
Q

What are the thick and thin blood smears used for in malaria?

A

Thick for diagnosis

Thin for species identification

289
Q

Chloroquine should not be used for which type of malaria

A

Falciparum due to widespread resistance

290
Q

First line treatment for uncomplicated Plasmodium falciparum malaria

A

ACT- artemisinin combination therapy

291
Q

In the Indian subcontinent, what medication are most cases fo typhoid resistant to?

A

Ciprofloxacin. So use ceftriaxone or azithromycin instead (or combo of both if no response)

292
Q

What malaria drug should NOT be given to children <12yrs?

A

Doxycyline

293
Q

What are some complications of malaria?

A 
Cerebral malaria
ARDS
Coagulopathy + spotaneous bleeding + splenic rupture
Septicaemia
Severe anaemia 
Metabolic acidosis, hypoglycaemia 
Jaundice 
Nephrotic syndrome, AKI
294
Q

What infectious cause is suggestive of these blood results?

  • Increase in Hct
  • Rapid decrease in pct count
  • Leukopenia

+ raised AST/ALT

A

Dengue fever

295
Q

What is dengue?

A

Arboviral infection transmitted by Aedes genus of mosquito

296
Q

When does dengue shock syndrome occur?

A

When a previously infected child is infected with a different strain of the virus. A partially effective host response augments the severity of infection by causing capillary leak syndrome, hypotension and haemorrhagic complications.

297
Q

What infectious cause is suggestive of these blood results?

  • Increase in Hct
  • Rapid decrease in pct count
  • Leukopenia
A

Dengue fever

298
Q

How can parvovirus B19 be transmitted?

A

Resp secretions
Vertical transmission
Infected blood products

299
Q

What can parvovirus B19 precipitate in children with chronic haemolytic anaemias?

A

Aplastic crisis

300
Q

What effect can parvovirus B19 have on foetuses?

A

Rarely, foetal hydrops + death due to severe anaemia

301
Q

Coxsackie A16 causes

A

Hand, foot and mouth disease

302
Q
  • Low-grade fever
  • Painful oral ulcers
  • Vesicles on the hands and feet

What condition?

A

Hand, foot and mouth disease

303
Q

Most common cause of viral meningitis in developed countries

A

Enteroviruses

304
Q

Most common complication of measles

A

Otitis media

305
Q

Most serious complication of measles

A

Subacute panencephalitis / encephalitis

306
Q

Clinical signs of rubella

A

Maculopapular rash, begins on face and spreads to whole body (often first sign of infection)

307
Q

Cardinal features of Kawasaki disease

A 
CRASH and Burn
Conjunctivitis
Rash
Adenopathy (usually cervical)
Strawberry tongue
Hand (swelling/erythema of hands and feet)
Burn (fever)
308
Q

Why should TB never be diagnosed on CXR alone in HIV+ children?

A

Risk of confusing it with lymphoid interstitial pneumonitis

309
Q

What stains can be used to identify TB?

A

Ziehl Neelsen or Auramine stains

310
Q

What is the tuberculin skin test (TST)?

A

Inject purified protein derivative intradermally into the forearm and observing 48-72 hours later –> measure the induration in millimetres (beware result may be because of past BCG not necessarily had infection)

311
Q

How should a TST of 5+mm be considered?

A

≥5 mm considered positive in situation of HIV infection, contact with infectious TB case within past 2 years, fibrotic opacities on chest x-ray consistent with untreated but healed TB, severely immunosuppressed patients (e.g., organ transplant, tumour necrosis factor-alpha-blocker, prednisolone ≥15 mg/day for 1 month or longer)

≥10 mm considered positive in situation of TST conversion within 2 years, medical or social conditions associated with increased risk of progression to active TB (e.g., diabetes, malnutrition, cigarette smoking, alcohol consumption >3 drinks/day, intravenous drug users, leukaemia, lymphoma, head and neck cancer, lung cancer, chronic renal failure), recent immigrants from countries with high prevalence of TB, residents and employees of high-risk congregate settings (e.g., nursing homes, prisons), TB laboratory personnel

≥15 mm considered positive in people with no risk factors for TB

312
Q

What is the treatment for tuberculosis?

A

RIPE and key worker for all +ve individuals:
Rifampicin + Isoniazid (6months)
Pyrazinamide + Ethambutol (2 months)

If adolescent - give pyridoxine (vitB6) weekly to prevent peripheral neuropathy due to isoniazid

If TB meningitis - give dexamethsone

Then do screening/contact tracing

313
Q

What is the prevention/contact tracing procedure for TB in the UK?

A

o UK RECOMMENDATION: BCG is given at birth for high-risk groups (but never HIV+ or immunocompromised individuals)
o As most children are infected by a household contact, it is important to screen other family members
o NICE GUIDELINES: children < 2 years who had close contact with a sputum smear positive pulmonary TB person should be started on prophylactic isoniazid
• If TST and IGRA are negative at 6 weeks, isoniazid should be discontinued and BCG should be given

314
Q

What test should be performed in a patient with Pneumocystic jirovecii pneumonia?

A

HIV test

315
Q

What prophylaxis is given to HIV + patients and which patients qualify for this?

A

PCP prohylaxis of co-trimoxazole

  • infants
  • older ppl with lower CD4+ cell counts
316
Q

Erythema migrans suggests

A

Lyme disease, caused by Borrelia burgdorferi

317
Q

What is X-linked agammaglobulinaemia caused by?

A

Abnormal Tyrosine Kinase gene, essential for B-cell maturation - leads to B-cell/antibody defects

318
Q

SPUR infections are indicative of what?

A 
Primary immunodeficient
(Severe, prolonged, unusual, recurrent infections)
319
Q

How do immunodeficiencies tend to present?

A

Prolonged or recurrent diarrhoea, faltering growth, recurrent infections eg pneumonia, severe infections eg meningitis, osteomyelitis, recurrent warts, candidiasis, complications following live vaccinations eg disseminated BCG etc

320
Q

Treatment of emergency asthma attack (in hospital)

A

1) 10 puffs of salbutamol via spacer
2) Oral prednisolone
3) Salbutamol and atrovent nebuliser (can be oxygen driven if sats are low) - try max 3 doses of salbutamol and one of Atrovent
4) MgSO4
5) Consider IV salbutamol or aminophylline
6) Consider intubation and ventilation

321
Q

What are some complications of primary ciliary dyskinesia (kartagener’s syndrome)?

A
  • Sinusitis
  • Otitis media
  • Infertility
  • Bronchiectasis
  • Dextrocardia +/- situs inversus (cilia are necessary for determination of sites of internal organs during development)
322
Q

How is chronic lung disease defined?

A

Oxygen requirement at 36 weeks corrected gestation or 28 days post-term

323
Q

What may be seen in a neonate with CF?

A

Meconium ileus

Prolonged jaundice

324
Q

Where are foreign bodies most likely to get lodged in the lung?

A

Right middle lobe - terminal one of three branches of right main bronchus, which is widest, shortest + most vertical of the bronchi.

325
Q

What is the most common congenital heart defect?

A

VSD - accounts for 1/3 of them (Second is patent ductus arteriosus)

326
Q

What are some signs of congenital heart disease?

A

Respiratory distress with feeds
Cyanosis
Hepatomegaly (+ potentially splenomegaly as a result of back-pressure secondary to right-sided heart failure)
Sweating with feeds

327
Q

What score is used to determine the severity of croup?

A

Westley score
=2 is mild - barking cough but no stridor at rest
3-5 is moderate easily heard stridor but few other signs
6-11 is severe - obvious stridor and chest wall indrawing
=/>12 - impending respiratory failure, cough and stridor may not be prominent at this stage anymore

328
Q

What is the most common cause of congenital heart disease in DS?

A

Atrioventricular septal defect

329
Q

What is the most common congenital heart defect?

A

VSD - accounts for 1/3 of them

330
Q

What are some signs of congenital heart disease?

A

Respiratory distress with feeds
Cyanosis
Hepatomegaly
Sweating with feeds

331
Q

How do loop diuretics work and give an example of one?

A

Furosemide/Frusemider

Inhibition Na reabsorption in ascending limb of loop of henle

332
Q

What type of shunt do ventricular septal defects produce?

A

Left to right - hence the child will NOT be cyanosed unless left untreated - Eisenmengers syndrome then develops in teenage years - pul HTN results in Right to left shunt + cyanosis

333
Q

How do loop diuretics work and give an example of one?

A

Furosemide/Frusemider

Inhibition Na reabsorption

334
Q

What type of shunt do ventricular septal defects produce?

A

Left to right - hence the child will NOT be cyanosed.

335
Q

What cardiac defects are associated with down syndrome?

A

Atrioventricular septal defect
VSD
ASD
Tetralogy of fallot

336
Q

When should indomethacin be used in children?

A

To close the ductus arteriosus if child has significant resulting respiratory distress or impaired systemic oxygen delivery

(it inhibits prostaglandin production)

337
Q

What congenital heart conditions will cause a baby to go blue on day2/3 and why?

A

Closure of ductus arteriosus into ligamentum arteriosum means circulation of blood from left-right shunting into systemic circulation can no longer continue

  • Tetralogy of fallot
  • Pulmonary atresia
  • Tricuspid atresia
  • Transposition of the great arteries
  • Total anomalous pulmonary venous drainage
338
Q

When should indomethacin be used in children?

A

To close the ductus arteriosus if child has significant resulting respiratory distress or impaired systemic oxygen delivery

339
Q

What is the most appropriate diagnosis in a child eg 7yrs who presents with intercurrent illness, increased HR eg 180bpm? Child may be pale + sweaty but alert. RR may be increased.

A

SVT

340
Q

What is suggestive of a delta wave and short PR interval on ECG?

A

Wolff-Parkinson-White syndrome

341
Q

Management of a SVT in a child

A

1) If child alert: Vagal manoeuvres eg blow a syringe like a balloon, put head in ice, unilateral carotid massage
2) Adenosine
3) Sedated synchronized cardioversion

342
Q

When should non-synchronized cardioversion be used?

A

Ventricular fibrillation
Ventricular tachycardia

(in all other situations it should be synchronised, otherwise the shock may land on QRS complex which can put a patient into VF)

343
Q

Inflammation of the BCG scar is common in what condition?

A

Kawasaki’s disease

344
Q

What is Ebstein’s anomaly?

A

Abnormal tricuspid valve + hypoplastic right ventricle + pulmonary stenosis

345
Q

What CHD would not allow a child to develop to their teens if left untreated?

A

Ebstein’s anomaly

Tetralogy of fallot

346
Q

Investigation for renal artery stenosis

A

Abdominal ultrasound with doppler

347
Q

Discrepancy of BP between arms, radio-femoral delay and systolic murmur heard in the aortic area is suggestive of what?

A

Coarctation of the aorta

348
Q

Investigation for renal artery stenosis

A

Abdominal ultrasound with renal doppler

349
Q

Which of these is NOT a sign of cardiac insufficiency?
A) Scattered wheeze on auscultation of the chest
B) Central cyanosis
C) Sacral oedema
D) Tachypnoea with the apex beat palpable in the 7th ICS just lateral to the mid-clavicular line
E) Hepatomegaly

A

Cardiac insufficiency = heart failure

Central cyanosis is not a feature

350
Q

Why does hepatomegaly occur in heart failure?

A

Back pressure in the venous system leading to congestion in the portal vein

351
Q

A low rumbling murmur heard above the nipple line with a normal echocardiogram suggests?

A

Venous hum murmur, normal in children

352
Q

What may be seen on an ECG of a patient with secundum ASD?

A

RBBB

Right axis deviation

353
Q

What may be seen on an ECG of a patient with partial AVSD?

A

Superior axis

354
Q

Continuous murmur below the left clavicle in a baby suggests?

A

Patent ductus arteriosus

355
Q

What do the following radiology signs suggest?

  • Boot shaped heart
  • Small heart
  • Pulmonary artery bay
  • Decreased pulmonary vascular markings
  • Up-tilted apex
A

ToF

356
Q

Steeple sign

A

PA view of a child with croup shows subglottal narrowing

357
Q

When is methylphenidate prescribed?

A

For ADHD, given as first line on a 6 week trial basis. Acts as dopamine/NA reuptake inhibitor. SEs of stunted growth, abdo pain, nausea and dyspepsia. Potentially cardiotoxic.

358
Q

What is more likely to present at birth - ToF or TGA?

A

TGA. ToF presents at 1-2mths.

359
Q

What are Salam attacks?

A

repeated flexion and extension movements seen in infants suggestive of infantile spasms. Confirm diagnosis on ecg by looking for hysarrythmia

360
Q

Presentation of west’s syndrome

A

=infantile spasms, usually presents in first 4-8 months of life. ECG shows hypsarrythmia. CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)

361
Q

Football sign and Rigler sign may be positive in what condition?

A

NEC
Football sign - air outlining falciform ligament
Rigler sign - air inside and outside of bowel wall

362
Q

What does Balloon atrial septostomy do?

A

Can be used to encourage mixing of blood in the heart in Transposition of the Great arteries, by breaking the foramen ovale flap.

363
Q

How does a complete AVSD present?

A

Cyanosis at birth or heart failure after 2-3wks. Usually picked up on antenatal USS.
NO MURMUR

364
Q

If a murmur is heard in tetralogy of fallot, what will it be?

A

Loud, harsh ejection systolic murmur

365
Q

What do the following radiology signs suggest?

  • Boot shaped heart
  • Small heart
  • Pulmonary artery bay
  • Decreased pulmonary vascular markings
  • Up-tilted apex
A

ToF

366
Q

What does Balloon atrial septostomy do?

A

Can be used to encourage mixing of blood in the heart in Transposition of the Great arteries, by breaking the foramen ovale flap

367
Q

How does a complete AVSD present?

A

Cyanosis at birth or heart failure after 2-3wks. Usually picked up on antenatal USS.

368
Q

Features of DiGeorge syndrome

A 
Chr22q11.2 microdeletion
Hypocalcemia
Absence of thymus 
Immunodeficiency
Palatal defects
369
Q

A carotid thrill is always present in which cardiac condition?

A

Aortic stenosis

370
Q

Transcatheter balloon dilatation can be used for which conditions?

A

Aortic or pulmonary valve stenosis

371
Q

What are the indications for exchange transfusion?

A

Acute chest syndrome
Stroke
Priapism

372
Q

Features of DiGeorge syndrome

A

Chr22q11.2 microdeletion
Hypocalcemia
Absence of thymus
Immunodeficiency

373
Q

Auer rods are suggestive of which diagnosis?

A

Acute myeloid leukaemia

374
Q

What can painful crises be triggered by in SCD?

A

Cold weather
Infection
Dehydration
Hypoxia

375
Q

What are the indications for exchange transfusion?

A

Sickle cell crisis
Stroke
Priapism

376
Q

What is the inheritance of vWF disease?

A

Types 1 and 2 - Autosomal dominant

Type 3 - Autosomal recessive

377
Q

How does HUS present?

A

AKI - raised urea and creatinine
Haemolytic anaemia - low Hb
Thrombocytopenia - low platelets

Secondary to E.Coli or Shigella gastroenteritis so Hx of exposure ie child went to petting farm + child may have diarrhoea etc

378
Q

What are the causes of clubbing?

A 
CF
Bronchiectasis 
Cyanotic congenital heart disease
IBD
Liver cirrhosis
379
Q

What are some long term complications of childhood cancer/ cancer treatment?

A
  • Educational difficulties (due to time missed off school or hearing/seeing difficulties due to malignancy or Tx)
  • Infertility
  • Growth problems
  • Blood malignancies eg leukaemias and lymphomas
380
Q

What is the inheritance of vWF disease?

A

Autosomal dominant

381
Q

How does HUS present?

A

AKI - raised urea and creatinine
Haemolytic anaemia - low Hb
Thrombocytopenia - low platelets

382
Q

Prolonged breastfeeding and lack of weening may cause what blood results in a child?

A

Abnormal iron/haem studies ie

  • Low Hb
  • Low ferritin
  • Low MCV
  • Normal B12 and folate
383
Q

What is the normal blood volume for healthy term infants?

A

80ml/kg

384
Q

A 1-year-old infant has an intake of iron of about

A

8mg/day

385
Q

Causes of microcytic anaemia

A

Iron deficiency anaemia
Beta thalassemia trait
Anaemia of chronic disease

386
Q

Raised HbA2 is suggestive of what condition?

A

B-thalassemia trait. There may also be raised HbF.

387
Q

Management of hereditary spherocytosis

A

Largely supportive

  • Blood transfusion
  • Folic acid supplements
  • Splenectomy + Vaccination for encapsulated bacteria eg H influenzae, Strep pneu., Men C
  • Cholecystectomy
  • Pneumococcal prophylaxis (Oral pen)
388
Q

Who gets G6PD?

A

Mostly boys - it is X-linked recessive (although can rarely happen in girls)

389
Q

Why would hydroxycarbamide be given to SCD patients?

A

Stimulates HbF production (NB: hydroxycarbamide = hydroxyurea)

390
Q

Raised HbA2 is suggestive of what condition?

A

B-thalassemia trait

391
Q

Indications for an unilateral undescended testicle at 8 weeks

A

If the testis is undescended by 3 months of age, the child should be referred to a paediatric surgeon and seen before 6 months of age. Orchiplexy often done around 1 Year.

392
Q

What are some of the causes of aplastic anaemia?

A

Can be acquired or inherited eg

  • Drugs (sulphonamides, chemotherapy)
  • Viruses (hepatitis)
  • Toxins (benzene)
  • Idiopathic
  • Fanconi’s anaemia
  • Shwachman-Diamond syndrome
393
Q

Neonates have lower levels of all clotting factors except…

A

Factor 8 and fibrinogen

394
Q

Bilateral undescended testes need

A

urgent review within 24hr by senior paediatrician

395
Q

What should be avoided in patients with haemophilia and vWF disease?

A

IM injections
Aspirin
NSAIDs

396
Q

What may be useful to give in Haemophilia A?

A

Desmopressin - stimulates release of endogenous factor 8 and von willebrand

397
Q

Why are patients with vWF disease also deficient in factor 8?

A

One of the roles vWF plays is to act as a carrier protein for factor 8, protecting it from inactivation and clearance

398
Q

What other conditions is T1DM associated with?

A

Addison’s

Coeliac

399
Q

What would the triple test results be for Downs Syndrome?

A

Low AFP, estriol, high hCG (and inhibin-A if quadruple test)

400
Q

What would the triple test results be for Edwards Syndrome?

A

Low AFP, estriol, hCG

401
Q

What would the triple test results be for NTDs?

A

High AFP

402
Q

What would the results be for a T21 foetus in combined test?

A

NT high
hCG high
PAPP-a

403
Q

Management of hyperthyroidism

A

Carbimazole or propylthiouracil (usually given for around 2years)
- Beta-blockers can be used symptomatically

Other options include

  • Radioiodine
  • Surgery
404
Q

Management of hypocalcaemia

A 
ACUTE
- IV Ca gluconate
CHRONIC
- Oral Ca
- High doses Vit D analogues
405
Q

What ECG change may be seen with hypercalcaemia?

A

Shortened QT interval

406
Q

Management of hypercalcaemia

A

Rehydration - IV NaCl
IV bisphosphonates eg zoledronic acid
IV diuretics

407
Q

Biochemical presentation of a salt losing crisis/addison’s

A

LOW Na
HIGH K
Metabolic acidosis
Hypoglycaemia

408
Q

Cause of achondroplasia

A

Mutation in fibroblast growth factor 3

409
Q

What are the classic facts of Kallman syndrome?

A

Deficiency of LHRH

Inability to smell

410
Q

What is global developmental delay?

A

Delay in acquisition of at least 2 domains of development

411
Q

Form of spastic cerebral palsy that affects arms over legs

A

Hemiplegia

412
Q

Form of spastic cerebral palsy that affects legs over arms

A

Diplegia

413
Q

Presentation of cerebral palsy

A 
Floopiness
Feeding difficulties
Hand preference before 1 yr
Poor trunk control 
Spasticity
Speech impairment
Delayed motor milestones 
May have evident signs depending on type ie increased tone/brisk reflexes if spastic or choreic movements if dyskinetic
414
Q

What are most cases of ataxic cerebral palsy determined by?

A

Genetically determined

415
Q

What is dorsal rhizotomy?

A

Some of nerve roots of spinal cord are cut to reduce spasticity

416
Q

What is baclofen?

A

Muscle relaxant and anti-spasmodic

417
Q

If urethral obstruction is suspected in a child, what examination should be performed?

A

MCUG

418
Q

Should you start Abx in a child with neg nitrites but leucocyte exterase positive?

A

NO, only if strong suspicion of UTI/other confirmatory tests. NB: you would start Abx if nitrites positive but leukocyte esterase neg.

419
Q

Management for enuresis before 5years

A

1) Reassure parents likely to resolve
2) Ensure easy access to toilet
3) Encourage bladder emptying before bed
4) Consider positive reward system

420
Q

Management of enuresis after 5years

A

1) If infrequent eg 2x week, reassure
2) Enuresis alarm + positive reward system
3) Desmopressin (ensure no fluid intake 1hr before, can also be used for short term control)

421
Q

What is the normal protein-to-creatinine ratio ?

A

< 20 mg/mmol

422
Q

What is a common, benign cause of proteinuria?

A

Orthostatic proteinuria

423
Q

What is usually the earliest sign of nephrotic syndrome?

A

Peri-orbital oedema

424
Q

Examples of steroid sparing agents

A

Cyclophosphamide
Tacrolimus
Ciclosporin A
Mycophenolate mofetil

425
Q

What accounts for 70-90% of nephrotic syndrome in childhood?

A

Minimal change disease

426
Q

What are the findings for renal biopsy below in minimal change disease?
LM:
Immunoflorescence:
ECM:

A

LM: minimal change
Immunofloresence: Occasional IgM in mesangium
ECM: effacement of podocyte foot processes

427
Q

What are the findings for renal biopsy below in membranous nephropathy?

A

LM: thickening of capillary walls
ImmunoF: IgG and C3
ECM: thickening of GBM

428
Q

What result needs to be seen under a microscope for haematuria to be confirmed?

A

> 10 red blood cells per high-power field

429
Q

Key features of Alport’s syndrome

A
  • Hearing loss
  • Eye problems
  • Renal disease (haematuria)
430
Q

Indications for a renal biopsy

A

Significant persistent proteinuria
Recurrent macroscopic haematuria
Renal function is abnormal
Complement levels are persistently abnormal

431
Q

If a patient presented with haematuria and haemoptysis, what diagnosis may you suspect?

A

Goodpasture’s disease, caused by anti-GBM antibodies

432
Q

Two tests that can aid in diagnosing a post-strep acute nephritis

A

Anti-streptolysin O titre

Anti-DNAse B titre

433
Q

What antibody is diagnostic for these vasculitides PAN, MPA, Wegener’s?

A

(c-)ANCA

434
Q

What are the 3 key points for managing vasculitides?

A
  1. Corticosteroids
  2. Plasma exchange
  3. IV Cyclophosphamide
435
Q

Anti-ds-DNA and low C3/4 levels indicate?

A

SLE

436
Q

What is the distribution of eczema in a child <18mths?

A

Trunk, flexure-SPARING, cheeks

437
Q

What are the most common type of renal stone for children?

A

Phosphate, associated with infection (Proteus)

438
Q

Definition of oliguria

A

<0.5ml/kg/hr

439
Q

Consider referral for renal replacement therapy if any of the following are not responding to therapy

A

1) Hyperkalaemia
2) Fluid overload and pulmonary oedema
3) Acidosis
4) Uraemia (eg pericarditis, encephalopathy)

440
Q

If there are ECG changes due to hyperkalaemia, what is the management?

A

IV calcium gluconate

Otherwise, can give salbutamol (nebs or IV), ca exchange resin, glucose and insulin, dietary restriction, dialysis

441
Q

What are the most common causes of acute renal failure in the UK?

A

HUS

Acute tubular acidosis

442
Q

What is the triad of HUS?

A

1) Acute renal failure
2) MAHA
3) Thrombocytopaenia

443
Q

What drugs are currently given to achieve immunosuppression in children with transplanted kidneys?

A

Tacrolimus
Mycophenolate mofetil
Prednisolone

444
Q

What does a septic screen include?

A

Blood culture, urine cultures,

cerebrospinal fluid cultures, chest x-ray and C-reactive protein

445
Q

How do you manage a child that has a high blood pressure reading in GP?

A

Repeat on 3 separate occasions (manually), if still high start investigations and refer to paediatric nephrologist.

446
Q

What is the treatment of choice for malignant HTN?

A

Sodium nitroprusside

447
Q

What is HUS caused by?

A

Verocytotoxin produced by E Coli O157 and Shigella gastroenteritis

448
Q

HSP involves what key 4 features?

A

1) Purpuric rash on extensor surfaces
2) Arthralgia
3) Abdominal pain
4) Renal involvement - haematuria

449
Q

Causes of post-renal AKI?

A

Ureteric stones
Bladder outflow obstruction
Neuropathic bladder

450
Q

Posterior urethral valves are diagnosed with

A

Micturating cystourethrogram

451
Q

What medication is given to renal transplant patients?

A

Septrin
Immunosuppression eg Tacrolimus, Mycophenolate mofetil, Cyclosporin
If impaired growth - Consider need for GH
EPO recombinant

DON”T GIVE NSAIDS - nephrotoxic

452
Q

Presentation of autosomal recessive kidney disease

A

Presents in childhood

  • Pulmonary hypoplasia -> respiratory distress
  • Bilateral renal masses
  • congenital hepatic fibrosis
  • Pulmonary HTN
453
Q

A 5-day-old baby is brought to see the GP because she has had a rash for the past 3 days which started on her chest, is spreading to her face and getting worse. On
examination she handles well and is alert. There is an erythematous rash on her face, torso and right arm with little pustules. What is the most likely diagnosis?

A

Erythema toxicum

454
Q

What distribution does the rash present in for Sturge Weber syndrome?

A

Port-wine naevus, trigeminal distribution

455
Q

Causes of petechiae in the superior vena cava distribution

A

Strangulation
Coughing
Vomiting
(later two due to pressure)

456
Q

Casuses of erythema nodosum

A 
NO
Drugs eg Abx - sulphonamides, amoxicillin
Oral contraceptives
Sarcoidosis
Ulcerative colitis, Crohn's, Behcet's
Micro: HepC, TB, Strep, EBV
457
Q

What is molluscum contagiosum caused by?

A

Pox virus

458
Q

What eye problems can excess steroids cause?

A

Steroids

459
Q

What conditions can be diagnosed using FISH?

A

DiGeorge’s
Cri du chat
Williams’

460
Q

What cardiac defects are Turner’s syndrome patients liable to getting?

A

Coarctation of the aorta

Aortic stenosis

461
Q

What cardiac valve problem can marfan’s patients get?

A

Mitral valve prolapse

462
Q

What test is important in an Addisonian crisis which will help determine your management?

A

Renal function tests
- Help identify hyperkalaemia and hyponatraemia

Need to most likely give
IV hydrocortisone and IV dextrose

463
Q

What test is important in an Addisonian crisis which will help determine your management?

A

Renal function tests
- Help identify hyperkalaemia and hyponatraemia

Need to most likely give
IV hydrocortisone and IV dextrose

464
Q

What is the management of hydrocele?

A

If <2yrs then observation, should resolve spontaneously
If 2-11yrs then Open repair, laparoscopic exploration, bilateral repair, if abdominal then surgery through abdominal incision

If >11yrs and familial –> removal of tunica vaginalis, observe if idiopathic, surgery if necessary

465
Q

What side of the body are varicocele more likely?

A

LEFT (left gonadal and left adrenal veins drain directly into left renal vein, rather than into IVC like right hand side)

466
Q

What is a bell clapper deformity?

A

A testis that is lying transversely on its attachment to the spermatic cord

467
Q

What is likely to present as testicular torsion but of less severity?

A

Torsion of testicular appendage - Hydatid of Morgagni

468
Q

What is a bell clapper deformity?

A

A testis that is lying transversely on its attachment to the spermatic cord

469
Q

What is likely to present as testicular torsion but of less severity?

A

Torsion of testicular appendage - Hydatid of Morgagni

470
Q

Management of Chalmydia or Gonococcal Epididymo-orchitis

A

Ceftriaxone +/- doxycycline

471
Q

Neurofibromatosis is associated with increased risk of which cancer?

A

Glioma

472
Q

HHV8 causes what type of cancer?

A

Kaposi’s sarcoma

473
Q

Increased vanillylamandelic acid and homovanillic acid in the urine suggests

A

Neuroblastoma (both are catecholamines)

474
Q

What triad suggests non-accidental injury?

A

Subdural haematoma
Retinal haemorrhages
Encephalopathy

475
Q

Risk factors for non-accidental injury

A 
Hx of maltreatment
Mental health problems
Domestic violence
Drug or alcohol abuse
Disability or long-term chronic illness
476
Q

Consequences of smoking during pregnancy

A

Increased risk of IUGR
Low BW
Placental abruption
Miscarriage

477
Q

What are the 4 most common congenital infections?

A 
TORCH
TOxoplasmosis
Rubella
Cytomegalovirus
Herpes Simplex Virus
478
Q

t(8;11) is associated with which type of malignancy?

A

Burkitt’s lymphoma

479
Q

HTLV-1 is associated with what malignancy?

A

Non-Hodgkin, adult T-cell lymphoma

480
Q

t(11;14) is associated with which type of malignancy?

A

Mantle cell non-hodgkin’s lymphoma

481
Q

What are the three types of aggressive, B cell, Non-Hodgkin’s lymphoma?

A
  1. Burkitt’s (8;11)
  2. Mantle cell (11;14)
  3. Diffuse large B cell
482
Q

What are the three types of indolent, B cell, Non-Hodgkin’s lymphoma?

A
  1. Follicular
  2. Marginal zone
  3. Small lymphocytic
483
Q

Popcorn cells under the microscope suggest what malignancy?

A

Nodular Hodgkin’s lymphoma, lymphocyte predominant

484
Q

What are the four types of classical Hodgkin’s lymphoma?

A
  1. Nodular sclerosis
  2. Mixed cellularity
  3. Lymphocyte rich
  4. Lymphocyte depleted
485
Q

What malignancy involves lacunar cells?

A

Hodgkin’s lymphoma, classical, nodular sclerosis (most often seen in young women)

486
Q

How does neuroblastoma often present in children? especially in children >2yrs?

A

Abdominal mass

In children >2years, presentation is often due to metastases ie bone pain, bone marrow suppression, malaise

487
Q

High urine catecholamine metabolites (VMA and HVA) is suggestive of what?

A

Neuroblastoma

488
Q

What should a MIBG scan be performed for?

A

Neuroblastoma - check for mets (can also be used as therapeutic intervention as targeted radiotherapy)

489
Q

MOST COMMON type of soft tissue sarcoma in childhood

A

Rhabdomyosarcoma

490
Q

What is the inheritance of retinoblastomas?

A

All bilateral retinoblastomas are hereditary, 20% of unilateral are hereditary

491
Q

How can a retinoblastoma be identified on fundoscopy?

A

White pupillary reflex replaces red reflex

492
Q

What is a skin manifestation of systemic langerhans cell histiocytosis?

A

Seborrhoiec rash, can be confused with eczema or seborrhoeic dermatitis

493
Q

If the baby is clinically well, gestational age > 38 weeks and >24 hours old with a bilirubin level that is within 50 micromol/L of the phototherapy threshold what do you do?

A

Repeat bilirubin measurements
within 18hr if risk factors for neonatal jaundice
within 24hr if no risk factors

494
Q

What are the risk factors for neonatal jaundice?

A

Visible jaundice within 24hr of birth
Gestational age <38wks
Previous sibling with neonatal jaundice requiring phototherapy
Exclusive breast feeding

495
Q

How should phototherapy be given/what is the process?

A

Repeat serum bilirubin 4-6hrs after starting, then every 6-12 hours
Stop once serum bilirubin is 50+micromol/L under the threshold
Check for rebound hyperbilirubinaemia 12-18hours after stopping
Encourage short breaks (30mins) for feeding, cuddling, changing nappies etc

496
Q

When should you consider intensified phototherapy?

A

If serum bilirubin level is rising rapidly (>8.5micromol/L/hr)
If serum bilirubin is within 50micromol/L of threshold for exchange transfusion after 72+hrs after birth
If not responding to phototherapy within 6hrs

497
Q

Assessment of underlying disease in jaundice

A 
Transcutaneous/serum bilirubin
FBC and blood film
Blood group
Haemocrit
LFTs
Coombs test
Blood G6PD levels
Septic screen if indicated
Did mum receive anti-D immunoglobulin?
498
Q

What is the harm in high bilirubin levels in a baby?

A 
Kernicterus = deposition of bilirubin in basal ganglia
Can present as tetra
- auditory neuropathy 
- neuromotor symptoms
- oculomotor paresis of upwards gaze
- enamel dysplasia
499
Q

Top investigation for diagnosing biliary atresia?

A

Cholangiogram (TIBIDA)

500
Q

Procedure for biliary atresia

A

Ideally perform within 60days, KASAI HEPATOPORTOENTEROSTOMY

- ligate biliary ducts and directly join part of duodenum to porta hepatis of liver

501
Q

Why may ursodeoxycolic acid be given to patients after a kasai hepatoportoenterostomy?

A

Promotes bile flow

502
Q

What are some of the complications of biliary atresia?

A

Growth failure
Portal HTN
Cholangitis
Ascites

503
Q

What is a choledochal cyst? How are they detected and treated?

A

Cystic dilatation of extrahepatic biliary system
Check with USS or MRCP
To treat need to surgically excise with a roux-e-y anastamosis

504
Q

What are some of the features of alagille syndrome?

A 
Triangular facies
Butterfly vertebrae 
CHD esp peripheral pulmonary stenosis
Eye abnormalities
Renal tubular disorders
505
Q

Why does alpha-1 anti-trypsin deficiency lead to emphysema?

A

Alpha-1 antitrypsin usually neutralises neutrophil elastase. Treat similarly to COPD ie inhaled bronchodilators, ICS, pulmonary rehabilitation and vaccination

506
Q

What are some of the features of galactosaemia?

A

Liver failure, cataracts, developmental delay

507
Q

What can happen if people with galactosaemia get gram negative sepsis?

A

Rapidly fatal shock with haemorrhage, DIC

508
Q

What can you measure in red cells to diagnose galactosaemia?

A

Galactose-1-phosphate-uridyl transferase

509
Q

What can result from prolonged parenteral nutrition

A

Neonatal hepatitis

510
Q

What happens to coagulation in hepatitis?

A

It remains normal

511
Q

How can hepatitis A be detected?

A

Anti-Hep A IgM Abs

512
Q

IgM HBcAb indicates?

A

Acute infection with hepatitis B virus

513
Q

HBsAg indicates?

A

Ongoing infection with hepatitis B (surface antigen detection)

514
Q

What percentage of children go on to develop chronic HBV liver disease?

A
  • 30-50% of asymptomatic carrier children will develop chronic HBV liver disease which may progress to cirrhosis
515
Q

What virus does hepatitis D depend on?

A

Hepatitis B

516
Q

What do 50-70% of chronic hepatitis D sufferers get?

A

Cirrhosis

517
Q

What is seronegative hepatitis?

A

When a viral hepatitis is suspected but not identified

518
Q

Who can get severe illness from hepatitis E?

A

Pregnant women can get fulminant hepatic failure

519
Q

What are the CATS guidelines for liver failure?

A

INR>/=1.5 + encephalopathy
OR
INR>/=2 w/t encephalopathy but due to liver cause NOT correctable by IV vit K

520
Q

What is fulminant liver failure?

A

onset of hepatic encephalopathy and coagulopathy within 8wks of onset of liver disease in the absence of any pre-exisiting liver disease in any form

521
Q

Management of acute liver failure

A

Early referral to a national paediatric liver centre
Key: maintain blood glucose, prevent coagulopathy, sepsis and cerebral oedema
Initial management:
- High flow oxygen
- Secure airway
- Venous access (ideally femoral central access for high conc IV dextrose)
- Prevent coagulopathy: IV vitamin K 2-10 mg, H2 blocking drugs/PPIs
- Maintain blood glucose 4-8 mmol/l (IV dextrose)
- Maintenance 0.9% NaCl + 10% dextrose (fluid restrict to 2/3 maintenance)
- NG tube
- Neuro observations  prevent cerebral oedema by fluid restriction and mannitol diuresis
- Prevent sepsis: Broad spec antibiotics and antifungals and parenteral high dose aciclovir in neonates
- N-acetylcysteine in paracetamol poisoning

522
Q

What are some of the findings in autoimmune hepatitis/PSC?

A
  • high total serum protein
  • hypergammaglobulinaemia
  • autoAbs
  • low C4
    biopsy –> typical histology
523
Q

What can be used to treat autoimmune hepatitis and Primary sclerosing cholangitis?

A

AH: Pred + Azathioprine
PSC: Ursodeoxycholic acid

524
Q

What are some of the hepatic consequences of CF?

A

Hepatic steatosis

Progressive biliary fibrosis

525
Q

What is wilson’s disease?

A

Autosomal recessive condition in which a basic gene defect results in reduced synthesis of caeruloplasmin (copper-binding protein) and defective copper excretion of copper in the bile –> leads to build up in liver, brain, kidney and cornea

526
Q

A 15 year old presents with increasing aggression and decline in school performance. Her mum has noticed a slight speech impediment. O/E the doctor notices a slight tremor in the right hand and incoordination in pass-pointing. The legs also looked slightly bowed. What is the likely diagnosis?

A

Wilson’s disease

can get deposits in brain causing parkinsonian type symptoms, renal deposits, haemolytic anaemia and rickets

527
Q

o Low serum caeruloplasmin
o Low serum copper
o Increased urinary copper excretion
===

A

Wilsons

528
Q

How is a diagnosis of Wilson’s disease confirmed?

A

Elevated hepatic copper on biopsy
OR
Gene mutation identified

529
Q

Mx of wilsons

A

1) Zinc (blocks intestinal copper absorption)
2) Trientine (increases urinary copper excretion)
3) Pyridoxine (vitamin B6)

530
Q

Why would Pyridoxine (vitamin B6) be given in Wilsons disease?

A

Prevent peripheral neuropathy

531
Q

What drug can be given to improve bile flow?

A

Ursodeoxycholic acid

532
Q

What medication can be given to aid pruritis in liver disease?

A
  • Phenobarbital (stimulates bile flow)
  • Cholestyramine (bile salt resin to absorb bile salts)
  • Ursodeoxycholic acid (oral bile acid that solubilises the bile)
  • Rifampicin (enzyme inducer)
533
Q

What is the finding of plasma ammonium in hepatic encephalopathy?

A

Likely to be elevated

534
Q

What diet may you put a patient on with hepatic encephalopathy?

A

Low protein - reduce nitrogenous load

535
Q

What are some features of chronic liver disease?

A
  • Jaundice
  • Palmar/plantar erythema
  • Telangiectasia
  • Spider naevi
  • Malnutrition
  • Hypotonia
  • Portal hypertension (caput medusae, splenomegaly)
536
Q

How is acute oesophageal varices managed?

A

Blood transfusions and H2 antagonists or omeprazole

537
Q

How do you manage ascites?

A

Na and fluid restriction

Diuretics

538
Q

If undiagnosed fever, abdominal pain, tenderness or an unexplained deterioration in hepatic or renal function what needs to be excluded?

A

Spontaneous bacterial peritonitis

539
Q

What should be done if spontaneous bacterial peritonitis is suspected?

A

o Diagnostic paracentesis should be performed and sent for WCC, differential and culture
o More than 250 neutrophils/mm3 is diagnostic of spontaneous bacterial peritonitis

Treat with broad spec Abx

540
Q

What is the management for cow’s milk protein allergy or intolerance?

A

If formula fed:

  • extensive hydrolysed formula (eHF) milk (first line replacement) if mild/moderate symptoms
  • amino acid based formula if severe CMPA or no response to extensive hydrolysed formula

If breast fed:

  • continue breast feeding
  • eliminate cow’s milk protein from maternal diet + consider providing mum Ca replacement
  • use eHF once breast feeding stops until 1yrs old and for at least 6mths
541
Q

What are three suggestive features of developmental problems?

A

doesn’t smile at 10 weeks
cannot sit unsupported at 12 months
cannot walk at 18 months

542
Q

In what direction is the neck of the femur displaced in SCFE?

A

Anterolaterally

Superiorly

543
Q

A shortened, externally rotated leg is suggestive of what condition?

A

SCFE

544
Q

What is looked for on a Plain X-ray of the hips in SCFE?

A

Klein’s line intersecting less of the femoral head

545
Q

What direction is displacement of the femoral head epiphysis in SCFE?

A

Postero-inferiorly

546
Q

When is methlyphenidate indicated and what needs to be monitored during its use?

A

First line pharm treatment for ADHD - give on 6wk trial basis

CNS stimulant - dopaminine/NA reuptake inhibitor

Can affect growth - monitor height and weight every 6mths

If not tolerated, switch to lisdexamfetamine

547
Q

What are the poor prognostic factors of ALL?

A 
age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex
548
Q

When would you admit a baby for IV glucose therapy?

A

Give 10% dextrose

If glucose <1mmol/L or symptomatic hypoglycaemia

549
Q

What can cause neonatal hypoglycaemia?

A 
Preterm birth (<37wks)
Maternal Diabtes mellitus
IUGR
Hypothermia
Neonatal sepsis 
Inborn errors of metabolism 
Nesidioblastosis
Beckwith-Wiedemann Syndrome
550
Q

What are some features of hypoglycaemai in a neonate/baby?

A

May be asymptomatic

Autonomic

  • Jittery, irritable
  • Tachypnoea
  • Pallor

Neuroglycopenic

  • Poor feeding/sucking
  • Weak cry
  • Drowsy
  • Hypotonia
  • Seizures

Other

  • Apnoea
  • Hypothermia
551
Q

What are the risk factors for SIDS?

A
  • Prone sleeping
  • Parental sleeping
  • Bed sharing
  • Hyperthermia and head covering
  • Prematurity
  • Male sex
  • Social classes IV and V
552
Q

Early-onset (<72hr) neonatal sepsis in the UK is most commonly caused

A

GBS or otherwise known as Streptococcus agalactiae

553
Q

What is group a strep also known as and what disease does it cause?

A

Strep pyogenes

Scarlet fever

554
Q

What organism is associated with late onset (7-28days) neonatal sepsis?

A

Staph epidermis
or
Gram neg bac eg Pseudomonas aeruginosa, Klebsiella and Enterobacter, Fungal species

555
Q

What is the incidence of neonatal infection?

A

8 per 1000 live births

556
Q

What are the risk factors for neonatal sepsis?

A
  • Mother with previous baby with GBS infection who has current GBS colonisation
  • Premature <37wks
  • Low birth weight (<2.5kg)
  • Evidence of maternal chorioamnionitis
557
Q

Presentation of neonatal sepsis

A
  • Respiratory distress
  • Tachycardia
  • Apnoea
  • Jaundice
  • Seizures
  • Poor or reduced feeding
  • Abdominal distention
  • Vomiting
558
Q

Investigations for neonatal sepsis

A 
Blood culture (try to do 2)
FBC
CRP
Blood gases
Urine microscopy
LP
559
Q

First-line regimen for suspected or confirmed neonatal sepsis

A

IV benzylpenicillin with gentamicin

560
Q

A 28-year-old primiparous woman who is 20 weeks pregnant presents after her foetal anomaly scan. The scan showed polyhydramnios and a midline sac containing bowel. She takes no regular medications and has no significant past medical history. She was planning on having a home birth and would like to know how this will affect her delivery.

Which of these is the most appropriate plan of action for this condition?

A

Exomphalos
= weakness of abdominal wall leads to protrusion of GI contents

need to perform elective C section to prevent risk of rupture, infection and atresia

561
Q

A 12-year-old female presents to her GP with bilateral knee pain, swelling and stiffness. On examination, a salmon-pink rash is noted on the legs.

What is the most likely diagnosis?

A

Still’s disease aka juvenile idiopathic arthritis, has to be for more than 6wks

may also have
pyrexia
lymphadenopathy 
arthritis
uveitis
anorexia + weight loss

ANA may be positive

562
Q

What is the rigler sign?

A

double wall on x-ray - pneumoperitoneum

563
Q

What is the only anti-depressant that you can give to children?

A

Fluoxetine

564
Q

What does a collapsing pulse indicate?

A

Aortic regurgitation or

patent ductus arteriosus

565
Q

What pulse is associated with patent ductus arteriosus?

A

Large volume, bounding, collapsing pulse

566
Q

What congenital heart defect can result in later cyanosis in the lower extremities (=differential cyanosis)?

A

Patent ductus arteriosus (initially not a cyanotic heart disease)

567
Q

At what age does hand preference emerge?

A

18 months

568
Q

What is talipes equinovarus?

A

Congenital club foot, may be noted on newborn examination, can see a rigid foot posture that proves difficult to correct

569
Q

What is pigeon toe?

A

Condition where the toes turn in while you’re walking or running. Most cases resolve by teens but some may need surgery.

570
Q

What is genu varum?

A

Bowing of legs, genu valgum is opposite (ie knock-knees)

571
Q

What is pes planus?

A

Flat foot (loss of medial longitudinal arch of the foot)

572
Q

What is transient synovitis of the hip and what causes it?

A

Inflammation in hip joint, most commonly occurs in pre-pubescent children, causes pain, limp, refusal to bear weight

Associated with decreased internal rotation of the hip

Mx:
NSAIDs + observe -> should improve within 48hrs

573
Q

What is legg-calve-perthes disease? Who gets it? How is managed?

A

Idiopathic avascular necrosis of the proximal femoral epiphysis
4-8yr olds, M:F 5:1, prognosis better if <6yrs

Can do traction or surgery - aim is to keep femoral head well located in acetabulum

574
Q

In Legg-Calves-Perthes disease, up to 75% of affected patients have some form of what?

A

Coagulopathy

575
Q

What are the most common causative organisms for septic arthritis and osteomyelitis (coincidentally the same) in

  • neonates
  • children over 2 years
A

Neonates: GBS

Children >2yrs: Staph aureus

576
Q

What are bony spurs?

A

Grow on the bones of the spine or around the joints, can be seen in osteoarthritis

577
Q

What GI manifestation can children suffering from HSP have?

A

Tarry stool ie blood in stool

578
Q

“Golf-ball” appearance of the red cells indicates what condition

A

Inclusion of HbH - indicates alpha thalassemia

579
Q

From what age is a palmar grasp observed?

A

6 months

580
Q

What are the primitive reflexes?

A

Moro
Grasp
Rooting
Walking

581
Q

Between what ages is jumping seen?

A

36 and 48 months

582
Q

What other abnormality is associated with pes planus?

A

Genu valgum

583
Q

Where are the common sites of osteomyelitus?

A

Metaphysis of distal femur

Proximal tibia

584
Q

What are the characteristic findings for osteomyelitis on

  • X-ray
  • Bone scan
A

X-ray: subperiosteal bone formation

Bone scan: hot spots

585
Q

What is the first line treatment of juvenile idiopathic arthritis?

A

NSAIDs

586
Q

What are the features of tumour lysis syndrome?

A
  • Hyperkalaemia
  • Hyperphosphatemia
  • Hypocalcaemia
  • Hyperuricaemia
587
Q

Which brain tumour in children is benign and associated with solid and cystic elements with calcification?

A

Cranipharyngioma

588
Q

A boy is recently diagnosed with neuroblastoma. What additional manifestation is he likely to demonstrate on examination?

A

An abdominal mass

589
Q

On what chromosome are abnormalities linked to wilm’s tumour?

A

11

590
Q

What is erysipelas and what is it caused by?

A

Form of cellulitis, results in red rash/discolouration over face or more commonly over lower limbs with associated swelling, can spread in superficial cutaneous lymphatics

-> most caused by group A beta-haemolytic strep

591
Q

Flesh coloured, dome shaped lesions

A

Molluscum contagiosum

592
Q

What drug can be given to those suffering from vWD?

A

Desmopressin (can increased vWF and factor 8 levels)

593
Q

What is the gold standard investigation for aplastic anaemia?

A

Bone marrow aspiration

594
Q

Unilateral lymphadenopathy in children is often caused by?

A

Staph or Strep

595
Q

What accounts for 50% of brain tumours in children and arises from astrocytes?

A

Glioma

596
Q

“dancing eyes” or jerky eye movements in a cachetic child is characteristic of what?

A

neuroblastoma

597
Q

X-ray findings of a sunburst appearance in a child with bone pain is characteristic of what?

A

Osteosarcoma

598
Q

X-ray findings of an onion skin-type appearance in a child with bone pain is characteristic of what?

A

Ewing’s sarcoma

599
Q

What organisms can cause erythema nodosum?

A

Streptococcus
Mycobacterium tuberculosis
Mycoplasma

600
Q

Green fluorescence under UV light of itchy lesions on the head + a Hx of hair falling out in a child indicates

A

Tinea capitis

601
Q

How do we define precocious puberty?

A

Onset before
8 in a girl
9 in a boy

602
Q

How do we define delayed puberty?

A

No signs of secondary sexual characteristics by 14 years

603
Q

What are the two categories of delayed puberty?

A

1) Hypogonadotrophic hypogonadism

2) Hypergonadotrophic hypogonadism

604
Q

What are streak gonads?

A

Gonads that don’t produce any hormones eg in Swyer syndrome / XY gonadal dysgenesis

605
Q

When does the ductus arteriosus close?

A

2 days

606
Q

What is the most common cause of croup?

A

Parainfluenza virus types one and two are responsible for 75% of cases

607
Q

What until Recently was the most common cause of epiglottitis and why has this changed?

A

Haemophilus influenza type B, we now give the Hib vaccine so other bacterial causes are now more common. Treat with steroids + 3rd gen cephalosporins eg ceftriaxone (or vancomycin/clindamycin) IV

608
Q

Typical presentation of ring worm?

A

Red patches in the groin and armpits which extends to the trunk

609
Q

Typical presentation of scabies?

A

Very itchy head face and hands. Red papules and vesicles with evidence of crusting.

610
Q

What is malathion solution used to treat?

A

Scabies

611
Q

Treatment of strawberry naevus? Complications of the condition?

A

Cryotherapy or laser, can also use steroids and interferon. Associated with increased bleeding and thrombocytopenia.

612
Q

How long does it take for the pulmonary pressure in a newborn to drop?

A

It drops over the first 1 to 2 months of life.

613
Q

Most common cause of a middle ear infection?

A

Streptococcus most commonly causes otitis media

614
Q

Continuous machinery murmur under the left clavicle and bounding pulse suggests well?

A

Patent ductus arteriosus

615
Q

Injection click and a murmur over the aortic area suggest what? Where can this murmur also be heard?

A

Aortic stenosis. Neck.

616
Q

A mother brings her son to A&E. She comments that he has been experiencing bouts of coughing which is associated with him going blue. In addition she states that he has been vomiting on occasion. Routine blood investigations demonstrate a lymphocyte count of 60×10 to the nine. What is the most likely diagnosis? And why are the coughing bouts happening? What is this condition Associated with?

A

Bordetella pertussis. The coughing belts are due to severe necrosis within the pulmonary epithelium. It is associated with pneumonia and seizures.

617
Q

What Kallmann’s syndrome?

A

Lack of GnRH

  • delayed/absent puberty
  • lack of smell
618
Q

What drug is specifically associated with CHD in newborn?

A

Lithium

619
Q

What is retinoblastoma? How does it present? How is it managed?

A

Most common ocular malignancy in children, presents around 18mths, 10% hereditary (AD). Loss of function of reTinoblastoma TSG in chr13. Can present with absence of red reflex, Leukocoria, strabismus and visual problems. Manage via enucleation and consider external beam therapy, chemo and photocoagulation. >90% survive into adulthood.

620
Q

What causes fragile X syndrome and how do patients present?

A

CGG triplet repeat within the FMRI gene on X chromosome - results in silencing of this part of gene and deficiency in resulting protein FMRP which is needed to form neuronal connections. Children have macropcephaly with long and narrow face, LARGE EARS, macroorchidism, intellectual impairment and commonly associated with ADHD. May also get recurrent otitis media and mitral valve prolapse. Tends to present around the time of puberty.

621
Q

What criteria is used to determine the risk of septic arthritis?

A

Kocher’s criteria

622
Q

What joints are most affected by septic arthritis?

A

Hip, knee and ankle

623
Q

A boy with learning difficulties is noted to be extremely friendly and extroverted. He has short for his age and has supravalvular aortic stenosis. What is the diagnosis?

A

Williams syndrome

624
Q

Autism is a triad of what?

A

Communication impairment
Impairment of social relationships
Ritualistic behaviour

625
Q

Attention deficit hyperactivity disorder comprises of what three major domains?

A

Hyperactivity
Inattention
Impulsivity

626
Q

What type of murmur is associated with Turners?

A

Systolic, loudest over aortic valve

627
Q

A short fourth metacarpal is associated with what genetic condition?

A

Turners

628
Q

What is the most common renal abnormality in Turner’s syndrome?

A

Horseshoe kidney

629
Q

Hand, foot and mouth disease is most commonly caused by?

A

Coxsackie A16 and Enterovirus

630
Q

Another telephone her GP following concerns about her daughter. Her daughter has had a temperature of greater than 40° for four days that has settled. But she has developed a pale red-spot-like rash on her body which has spread to her arms, legs and neck. What is the most likely diagnosis?

A

Roseola infantum. Febrile convulsions are common. Caused by HHV6B

631
Q

A young child is rushed to A&E following a fever for more than five days. On examination you no evidence of a maculopapular rash on the child’s arms and neck. In addition you no evidence of cervical lymphadenopathy and a strawberry like tongue. What is the most likely diagnosis?

A

Kawasaki’s disease

632
Q

Streptococcus pyogenes is responsible for what condition? How does it present?

A

Scarlet fever. Presents with lymphadenopathy, fever, Sore throat, strawberry tongue with a white coating, rash which spares the face, Palms and soles and has a sandpaper texture. Treat with penicillin

633
Q

How does slapped cheek syndrome present and what is the cause of it?

A

starts with a low-grade fever, headache, rash, and cold-like symptoms, such as a runny or stuffy nose. Then symptoms stop and a couple of days later a red rash appears all over the body but most prominently on the cheeks. It is caused by parvovirus b19. The child stops being infectious when the rash appears.

634
Q

What is the Paul Bunnell test?

A

The Monospot test

635
Q

How is Asperger’s syndrome characterised?

A

Social impairment without evidence of impaired language

636
Q

What platelet count is a contra indication of performing a lumbar puncture in a child?

A

Less than 50x10(9)/L

637
Q

What do all children with Kawasaki’s disease need?

A

Urgent Echo To check for coronary artery aneurysms

638
Q

When should the Hep B vaccine be given to babies born to Hep B positive mothers?

A

Birth

639
Q

When should the BCG vaccine be given to babies born in TB prevalent areas?

A

Birth

640
Q

When are the HiB and Men C vaccines given together?

A

12mths

641
Q

At what stage are both the MMR and pneumococcal vaccine given together?

A

12 months
MMR - first dose
Pneumococcal - second dose

642
Q

What does the 6 in 1 vaccine include and when are the doses given?

A

2, 3, 4 months

  • Diptheria
  • tetanus
  • pertussis
  • polio
  • Hib
  • Hep B
643
Q

What condition is associated with bouts of coughing which may result in subsequent bronchopneumonia?

A

Bordatella pertussis. Treat with azithromycin if they qualify ie <21days etc.

644
Q

How does a temporal lobe seizure present?

A

Lip smacking and unusual sensation in stomach. May also experience other sensory phenomenon, deja vu or jamais vu.

645
Q

What type of seizure is common in first year of life and associated with flexion spasms typically every 10 seconds?

A

West’s syndrome

646
Q

At what age should a child demonstrate palmar grasp?

A

3-6 months

647
Q

At what age should a child demonstrate pincer grasp?

A

10-18months. Children should also be able to hold pencils and scribble at this age.

648
Q

At what age should a child be able to hold a spoon and transfer food into the mouth?

A

14months - 2years

649
Q

At what age should children demonstrate polysyllabic babbling?

A

6-10months. They should also be able to point to objects they want to play with.

650
Q

At what age should a child build a tower of 3-4 bricks?

A

16-24months

651
Q

Which tumour is associated with jerky eye movements and small blue-coloured lumps in the skin?

A

Neuroblastoma

652
Q

Wilm’s tumour presents how and is associated with what chromosome abnormality?

A

Chromosome 11. Presents with abdominal mass and haematuria.

653
Q

What tumour arises from Astrocytes and accounts for over 50% of all childhood brain tumours?

A

Glioma

654
Q

Which murmur is associated with a harsh pan systolic murmur and splitting of the second heart sound?

A

VSD

655
Q

What is associated with a loud continuous machinery murmur, best heard under the left clavicle? A collapsing pulse may also be felt.

A

Patient ductus arteriosus

656
Q

What is associated with a slow rising pulse and an ejection systolic murmur at the apex?

A

Aortic stenosis

657
Q

A four-year-old boy is reviewed by his GP. On examination there is evidence of white spots on a red coloured background within the boys mouth. What is the most likely diagnosis?

A

Measles. These are Koplik spots and are a precursor to the development of a rash which is maculopapular in nature.

658
Q

Inflammation of the cerebellum resulting in subsequent ataxia can be caused by what childhood infection?

A

Chickenpox which is caused by varicella zoster

659
Q

A four-year-old girl is reviewed by her GP who notices the presence of a white left people which does not react to light. What is the most likely diagnosis? How can it be treated?

A

Retinoblastoma. A tumour of photoreceptor cells associated with gene deletion on chromosome 13. Treatment involves cryotherapy or radiotherapy.

660
Q

What is conjunctivitis mostly caused by and how should it be treated?

A

Most cases are viral so treated with salt water bathing. If bacterial treat with chloramphenicol eyedrops.

661
Q

What conditions are associated with exomphalos?

A

Beckwith-Wiedemann syndrome
Down’s syndrome
cardiac and kidney malformations

662
Q

In newborns/infants <3mths old what are the causes of meningitis?

A

GBS
E coli
Listeria

663
Q

In children 3mths-5yrs old what are the causes of meningitis?

A

Meningococcus
Pneumococcus
H influenzae

664
Q

What Tx should be given to meningitis caused by E Coli?

A

Cefotaxime

665
Q

What Tx should be given to meningitis caused by Pneumococcus or H influenzae?

A

Ceftriaxone

666
Q

A 5yo undergoes a routine eye assessment. Slit lamp examination reveals the presence of an irregular right pupil + anterior chamber appears cloudy. What is the most likely diagnosis?

A

Iritis = anterior uveitis

  • Need to treat with Abx
  • Risk of glaucoma and cataracts
667
Q

6yr is brought into A&E. Erythema around right eye, right eyed proptosis and reduced eye movements. What the diagnosis and how should it be treated?

A

Orbital cellulitis. Tx with IV Abx and urgent orbital CT.

668
Q

4yo seen by GP and has excessive blinking/lacrimation of right eye. Boy also has difficulty looking at bright lights. What is diagnosis?

A

Glaucoma. Tx with surgery.

Excessive blinking = blepharospasm

669
Q

A child with low set ears, micrognathia, rocker bottom feet has what condition?

A

Edward’s syndrome

670
Q

When should you admit a child with croup?

A

Moderate or severe croup based on CKS guidelines (eg stridor at rest, wall recessions etc)

  • <6mths of age
  • Known upper airway abnormalities eg laryngomalacia or down’s
  • Uncertainty about diagnosis (important differentials include acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation)
671
Q

What is a loss of function mutation on WT1 gene, chromosome 11 associated with?

A

Wilm’s tumour

672
Q

If there is any concern about baby, how often should APGAR scores be monitored?

A

APGAR scores are assessed at 1, 5 and 10 minutes of age

673
Q

The most common cause of hypothyroidism in children (juvenile hypothyroidism) in the UK is?

A

Autoimmune thyroiditis

674
Q

The most common cause of hypothyroidism in children (juvenile hypothyroidism) in the developing world is?

A

Iodine deficiency

675
Q

What is the most useful investigation to screen for the complications of Kawasaki disease?

A

Echo - check for coronary artery aneurysms

676
Q

Treatment of Kawasaki’s disease

A

1) High-dose Aspirin (despite Reye’s syndrome risk)
2) IVIG
3) Echocardiogram

677
Q

What is the most common cause of acute epiglottitis in an unvaccinated child?

A

Haemophilus influenzae type B

Now due to the HiB vaccine its commonly caused by Strep pneumoniae, Strep pyogenes, Staph aureus

678
Q

Scaphoid abdomen in a child with displaced apex beat and decreased air entry

A

Congenital diaphragmatic hernia

679
Q

In what conditions does malrotation occur in

A

Exomphalos
Congenital diaphragmatic hernia
Intrinsic duodenal atresia

680
Q

Conditions associated with Hirschprung’s disease?

A

DS
Neurofibromatosis
MEN2

681
Q

Gold standard for Hirschprung’s diagnosis

A

Rectal biopsy

682
Q

What investigation should be performed once a diagnosis of Kawasaki disease is made and why?

A

Echocardiogram at time of diagnosis and 6mths after

- look for coronary artery aneurysms

683
Q

Where should a pulse be taken in an infant vs child during life support?

A

Infant: brachial or femoral
Child: femoral

684
Q

Risk factors for more severe bronchiolitis

A

Bronchopulmonary dysplasia
Cystic fibrosis
Congenital heart disease

685
Q

Treatment of kawasaki disease

A

IVIG

Aspirin (one of only times in childhood you give due to risk of Reye’s syndrome)

686
Q

Treatment of UTI children

A

<3mths: admit and IV Abx

>3mths: 1st line trimethoprim, 2nd line: nitrofurantoin, amoxicillin, cefalxein

687
Q

How should children with HSP be monitored long term and why?

A

Weekly (1-4wks): BP, Urine dipstick
Weekly (5-12wks): fortnightly review BP/Urine dipstick
Month 6 and 12: BP, urine dip

Checking for long term renal damage

688
Q

3-5 day high fever followed by a 2 day maculopapular rash which starts on the chest and spreads to the limbs is suggestive of what diagnosis?

A

Sixth disease ie Roseola infantum (rash generally occurs as the fever is disappearing)

Year 5 (5 decks)

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