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Phase 3A 2021 > Paeds new > Flashcards

Paeds new Flashcards

1
Q

Pneumonia defintion?

A
  • inflammation of the lung parenchyma (the part of lungs involved in gas transfer e.g. alveoli + resp bronchioles)
  • congestion caused by viruses or bacteria or irritants
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2
Q

Causes of pneumonia in children?

A

Viral more common

Viral: RSV, adenovirus, rhinovirus, influenza

Bacterial: Strepmpneumoniae, Hib

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3
Q

Presentation of pneumonia in children

A
  • temperature (over 38.5)
  • rapid breathing/difficulty breathing
    cough
  • chest pain
  • vomiting
  • decreased activity
  • loss of appetite/poor feeding
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4
Q

Investigations for paediatric pneumonia?

A
  • Sputum sample (can be difficult)
  • Blood cultures.
  • CXR: look for consolidation.
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5
Q

Describe the treatment of pneumonia.

A

PO amoxicillin.
Co-amoxiclav if complicated or unresponsive.

O2, analgesia, IV fluids if indicated.

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6
Q

Bronchiolitis:

  • causative agent?
  • age?
A
  • RSV
  • Babies 0-2
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7
Q

Investigations for bronchiolitis?

A

Nasopharyngeal aspirate or throat swab
RSV rapid testing and viral cultures
FBC

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8
Q

Home or hospital for bronchiolitis?

A

Hospital if severe apnoea/resp distress: grunting, RR>70, central cyanosis, sats<92%,

Consider hospital if rr>70, inadequate fluids, clinical dehydration

No role for antibiotics, steroids or bronchodilators

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9
Q

What is asthma?

A

Chronic obstructive resp disease characterised by episodic exacerbations of bronchoconstriction

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10
Q

Presentation of asthma?

A
  • Cough - nocturnal
  • SOB
  • wheeze/whistling
  • chest congestion/tightness
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11
Q

Diagnosis of asthma in children?

A
  • If under 3: may use wait and see approach
  • If under 5: need to go off the history
  • If over 5: spirometry, PEF
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12
Q

Acute management of asthma?

A
  • Oxygen if needed
  • SABA
  • Prendisolone 1mg/kg IV

If no improvement:

  • IV salbutamol bolus
  • Aminophylline/MgSO4/salbutamol infusion
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13
Q

Long-term asthma management in under 5s?

A
  1. SABA for wheeze episodes (salbutamol prn)
    • ICS (beclametasone)
    • Leukotrine-receptor agonist (montelukast)
  4. stop LTRA and refer to asthma specialist
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14
Q

List three possible side effects of inhaled corticosteroids

A
  • Adrenal suppression
  • Growth suppression
  • Osteoporosis (although this has not been shown to affect bone fractures)
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15
Q

Wheeze vs stridor?

A

Wheeze = expiratory, polyphonic

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16
Q

Inhalers: name 2 ‘preventers’.

A

ICS act as ‘preventers’ e.g. beclamethasone, budenoside.

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17
Q

Inhalers: name 2 ‘relievers’.

A

Beta agonists e.g. salbutamol.
Muscarinic antagonists e.g. ipratropium bromide.

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18
Q

Why might asthma treatment fail in children?

A
  1. Adherence.
  2. Wrong diagnosis.
  3. Environmental factors.
  4. Choice of drug.
  5. Bad disease.
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19
Q

Non-resp causes of wheeze?

A
  1. GORD
  2. bronchomalacia
  3. cystic fibrosis
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20
Q

What constitutes the upper airway?

A

Nose, pharynx, larynx

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21
Q

Name 3 URTI.

A
  1. Rhinitis.
  2. Otitis media.
  3. Pharyngitis.
  4. Tonsillitis.
  5. Laryngitis.
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22
Q

Name 3 LRTI.

A
  1. Bronchitis.
  2. Croup.
  3. Epiglottitis (bacterial).
  4. Tracheitis.
  5. Bronchiolitis.
  6. Pneumonia.
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23
Q

Would you expect a patient with bronchitis or with bronchiolitis to be hypoxic and tachypnoeic? Explain why.

A

Bronchiolitis.

Bronchiolitis affects the respiratory portion of the airway, where gas exchange takes place therefore you may see hypoxia and tachypnoea.

Bronchitis affects the conducting portion of the airway and so is unlikely to have these effects.

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24
Q

Name 4 LRTI that could be caused by RSV.

A
  1. Acute bronchiolitis.
  2. Wheezy bronchitis.
  3. Asthma exacerbation.
  4. Pneumonia.
  5. Croup.
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25
Q

Why are infants more susceptible to descending infection?

A

Infants have a poor innate immune response and so are more susceptible to descending infections.

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26
Q

What is croup?

A

Acute larngotracheobronchitis.

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27
Q

Describe the cough that is associated with croup.

A

A barking seal like cough - often worse at night.

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28
Q

How do you treat croup?

A

usually self-limiting

can give steroids e.g. dexamethosone, beclamethasone.

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29
Q

Describe the aetiology of recurrent wheeze.

A
  1. Persistent infantile wheeze.
  2. Viral episodic wheeze.
  3. Asthma.
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30
Q

What is persistent infantile wheeze normally associated with/exacerbated by?

A

Persistent infantile wheeze tends to affect the small airways. It is associated with parental smoking or post-viral infection.

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31
Q

Are inhalers likely to help a child with persistent infantile wheeze?

A

No. Inhalers are unlikely to help; symptoms will improve as the child gets older.

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32
Q

Are inhalers likely to help a child with viral episodic wheeze?

A

Bronchodilators may help but there is no benefit from inhaled steroids.

Symptoms are likely to improve with age.

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33
Q

Viral induced wheeze:

  • common causes?
  • affects which age?
A
  • RSV, rhino virus
  • children aged 2-5
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34
Q

VIW vs bronchiolitis?

A

Bronchiolitis: babies, 3-5 days gradual build up

VIW: 2-5 yrs, viral prodrome for 1-2 days then suddenly worsens

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35
Q

How to manage a viral induced wheeze?

A

Reliever inhaler for the minority=SABA=salbutamol
If SABA 2-10 puffs PRN to max 4 hourly via a spacer
Otherwise, supportive/comfort care

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36
Q

How to identify viral induced wheeze (how does it differ from asthma)?

A
  • No wheeze with exercise
  • No interval symptoms
  • No excess of atopy
  • No benefit from regular inhaled steroids
  • No wheeze except with viral infections
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37
Q

Pathaphysiology of cystic fibrosis

A

Autosomal recessive

Reduced airway surface liquid impedes mucus clearance allowing excessive bacterial growth
Pancreas: duct occluded in utero leading to pancreatic insufficiency (can lead to CF-related DM)
GI: increased mucus can cause meconium ileus
Biliary tree: can have cholestasis leading to neonatal jaundice

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38
Q

Neonatal test to identify CF

A

Heel prick- Guthrie test

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39
Q

Investigations for CF:

A

Sweat chloride over 60mmol/L

CXR, sweat test, glucose tolerance test, microbiology, LFT, coag, bone profile, PFTs- all generally involved in annual assessments

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40
Q

Name 2 respiratory illnesses that can present with stridor.

A
  1. Croup - often a louder stridor.
  2. Acute epiglottitis - often a quieter stridor due to inflamed epiglottis blocking oesophagus and trachea.
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41
Q

Why are children more at risk of epiglottitis than adults?

A

Epiglottis is floppier, broader, longer and angled more obliquely to trachea and have a larger tongue
Therefore higher risk of acute airway obstruction

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42
Q

Aetiology of epiglottitis?

A

Reduced since the Hib vaccination
Normally Haemophilus infleunzae and Streptococcus pneumoniae which locally invade

V rarely due to trauma or non-infectious causes

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43
Q

Presentation of epiglottitis

A
  1. S.O.B
  2. Drooling
  3. Difficulty swallowing (dysphagia)
  4. Muffled voice (dysphonia)
  5. Typically no cough
  6. Stridor is a late sign
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44
Q

Describe the immediate management for acute epiglottitis.

A

Secure the airway - anaesthetist, ENT surgeon.

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45
Q

Management of epiglottitis

A

Oxygen, nebulised adrenaline, IV antibiotics (3rd gen cephalosporins eg ceftriaxone), IV steroids, nil by mouth until airway improved

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46
Q

Name a bacteria that causes acute epiglottitis.

A

H.influenzae B.

Acute epiglottitis is a severe acute illness.

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47
Q

You do a lumbar puncture and find raised proteins and low glucose. Is this likely to be due to a bacterial or a viral infection?

A

Bacterial.

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48
Q

Give 3 potential consequences of hearing loss.

A
  1. Speech and language delay.
  2. Social problems e.g. behavioural issues.
  3. Academic underachievement.
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49
Q

How does hearing loss in children often present?

A
  1. Parental concern.
  2. Speech, behavioural or educational problems.
  3. Incidentally on screening.
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50
Q

What are the 3 types of hearing loss?

A
  1. Conductive hearing loss.
  2. Sensori-neural hearing loss.
  3. Mixed.
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51
Q

Give 3 causes of conductive hearing loss.

A
  1. Glue ear.
  2. Ear wax.
  3. Otitis media.
  4. Perforated ear drum.
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52
Q

Describe the management of conductive hearing loss.

A

Conductive hearing loss is usually ENT managed:

  • Wait and wait - most will resolve on their own.
  • Grommet insertion.
  • Temporary hearing aid.
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53
Q

Give 3 risk factors for sensori-neural hearing loss.

A
  1. Family history.
  2. SCBU.
  3. Consanguinity.
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54
Q

Describe the management of sensori-neural hearing loss.

A

Sensori-neural hearing loss is often managed by a paediatrician. Treatments involve hearing aids or cochlea implants.

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55
Q

How would you manage mixed hearing loss?

A

You would address the conductive problem first and then offer a hearing aid.

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56
Q

When is hearing tested in children?

A
  1. New-born hearing screen.
  2. School entry hearing test.
  3. Long term monitoring is done in high risk groups.
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57
Q

Is the new-born hearing screen an objective or subjective test?

A

It is an objective test - response or no response.

If there are concerns, the patient is followed up with evoked response audiometry.

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58
Q

What are the 3 aims of hearing testing in children?

A
  1. Measure hearing threshold (dB).
  2. To be frequency specific (Hz).
  3. Obtain single ear information if possible.
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59
Q

Name 4 types of subjective hearing testing.

A
  1. Behavioural observational audiometry.
  2. Distraction testing.
  3. Visual reinforcement audiometry.
  4. Performance testing and play audiometry.
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60
Q

Name 2 bacterial and 2 viral organisms that can cause acute otitis media.

A

Bacterial: S.pneumoniae., H.influenzae.

Viral: RSV, rhinovirus

come from nasopharyngeal area through eustachian tube

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61
Q

Give 3 symptoms of acute otitis media.

A
  1. Severe pain lasting weeks
  2. Coryzal symptoms
  3. Generally unwell.
  4. Otorrhoea.
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62
Q

Otitis media investigations?

A
  1. ALWAYS test function of facial nerve on examination
  2. Otoscopy
  3. Discharge sent for microscopy and culture
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63
Q

Describe the treatment for acute otitis media.

A

Watch and wait - most spontaneously resolve in 24 hrs
Analgesia.
If recurrent, offer antibiotics and consider a grommet.

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64
Q

Give 2 potential complications of acute otitis media.

A
  1. Extra-cranial: mastoiditis, TM perforation.
  2. Intra-cranial: meningitis, abscess.
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65
Q

What is the function of a grommet?

A

A grommet keeps the middle ear aerated and prevents the accumulation of fluid in the middle ear.

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66
Q

When might a grommet be indicated?

A
  1. Recurrent AOM.
  2. Chronic otitis media + effusion (glue ear)
  3. ET dysfunction.
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67
Q

What is the common name for otitis media + effusion?

A

Glue ear.

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68
Q

What causes glue ear?

A

Infection!
45% follow AOM.

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69
Q

Presentation of glue ear? Examination findings?

A

Difficulty hearing and pressure sensation

Examination: TM dull and light reflex is lost

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70
Q

Pathophysiology of glue ear?

A

inflammatory fluid build up leading to conductive hearing impairment via chronic inflammatory changes and eustachian tube dysfunction

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71
Q

Give 3 risk factors for glue ear.

A
  • Bottle fed
  • paternal smoking
  • atopy
  • genetic disorders egCF and Downs
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72
Q

Causes of deafness in children? (infections)

A
  • Temporary=OME
  • Meningitis, mumps and measles
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73
Q

Aetiology of tonsillitis

A

Viral mostly
Bacterial-group A strep most common (S.pyogenes)

Most common in age 5-15

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74
Q

Tonsillitis- bacterial vs viral assessment

A

Centor score:

Age, exudate, tender/swollen anterior cervical LNs, temp over 38, absent cough

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75
Q

Management of tonsillitis

A

Viral is self limiting
Bacterial- should swab for culture and commence antibiotics (usually benzylpencillin)

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76
Q

What are the criteria for considering a tonsillectomy?

A
  1. >7 episodes of acute tonsilitis in a year.
  2. OSA or sleep-deprived breathing.
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77
Q

What is the epidemiology of periorbital cellulitis?

A

Peak in under 10s
Males twice as commonly affected
Peak occurrence in later winter and early spring

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78
Q

What are the causes of periorbital cellulitis?

A

S. pneumoniae and S. aureus

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79
Q

How to manage periorbital cellulitis?

A

If mild preseptal can do from home with broad spectrum empirical abx

If more extensive- hospital, IV abx, supportive therapy

If large abscesses, intracranial complications at px and in frontal sinusitis, need urgent drainage

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80
Q

Complications of periorbital cellulitis?

A
  • Visual related: (vision loss is 11%) start to lose red colour vision (sign of optic nerve compromise)
  • Neurological complications: sepsis, intracranial abscess, cavernous sinus thrombosis
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81
Q

Describe the types of manifest and latent strabismus

A

Manifest:
Esotropia=eye turns in
Exotropia=eye turns out
Hypertropia=eye goes up
Hypotropia=eye goes down

Latent:
Esophoria=inwards under occluder
Exophoria=outwards under occluder
Hyperphoria=up under occluder
Hypophoria= down under occluder

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82
Q

what tests to identify strabismus

A 
Cover test (manifest squint) 
Cover/uncover test (latent squint)
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83
Q

What shape lens for myopia? For hypermetropia?

A

Myopia/short sighted= concave
Hypermetropia/long sighted=convex

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84
Q

Causes of microcytic anaemia?

A
  • Iron deficiency
  • thalassaemia
  • chronic inflamm
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85
Q

Two microcytic anaemias and how you could tell them apart?

A

Iron-deficiency anaemia (high TIBC on iron profile)
Thalasaemia (low TIBC on iron profile)

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86
Q

What is the most common cause of anaemia in children?

A

Iron deficiency.

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87
Q

Why are kids more susceptible to iron deficiency anaemia than adults?

A

30% of their iron comes from diet and 70% from recycled rbcs (whereas adults have 5% from diet and 95% from recycling)
Have higher expenditure

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88
Q

Give 3 signs of anaemia in children.

A
  1. Pallor.
  2. Irritable.
  3. Lethargy.
  4. SOB.
  5. Tachycardic
  6. Murmur
  7. Poor growth
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89
Q

Risk factors for iron-deficiency anaemia in infants and children?

A

Infants = maternal iron deficiency, premature, low birth weight, multiple pregnancy, exclusively breastfed after 6 months

Children=veggie/vegan, GI disorders, chronic blood loss

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90
Q

What is the treatment for iron deficiency anaemia?
Side effect?

A
  • Diet advice, ferrous sulphate tablets
  • Constipation
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91
Q

Why does the treatment for iron deficiency anaemia sometimes fail?

A

Non-compliance.

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92
Q

Give 3 signs of beta thalassaemia major.

A
  1. Severe anaemia.
  2. Jaundice.
  3. Splenomegaly.
  4. Failure to thrive.
  5. Present at 6months+ (when adult Hb takes over)
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93
Q

Describe the management of beta thalassaemia major.

A
  • Genetic counselling.
  • Blood transfusions with iron chelation to prevent overload
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94
Q

Long term sequelae of untreated thalassaemia major?

A

Progressive severe anaemia
Try to compensate with bone marrow expansion and extramedullary haematopoiesis

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95
Q

What may carriers of thalassaemia have?

A

Mild microcytic hypochromic anaemia

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96
Q

Which type of thalassaemia is generally incompatible with life?

A

alpha major (4/4 genes missing to make alpha chain)

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97
Q

What does a low reticulocyte count indicate?

A

A production problem e.g. non-haemolytic.
Infection
Chronic inflamm
Bone marrow malignancy
Bone marrow failure (aplastic)

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98
Q

What does a high reticulocyte count indicate?

A

A degradation problem e.g. bleeding or haemolysis.

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99
Q

What are some types of haemolytic anaemia?

A

Intrinsic to RBC:

  • sickle cell
  • enzyme deficiencies: G6PD, pyruvate kinase
  • spherocytosis

Extrinsic:

  • auto and alloimmune
  • DIC
  • TTP
  • malaria
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100
Q

How do haemolytic anaemias present?

A
  • Splenomegaly
  • Cholecystitis/gall stones
  • Jaundice (hyperbilirubinaemia)
  • Increased lactate dehydrogenase
  • Leg ulcers
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101
Q

Describe the molecular structure of haemoglobin.

A

Four protein chains (globin) each surrounding a non-protein group (heme).

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102
Q

What is the difference between adult and fetal haemoglobin?

A

Adult Hb: the four protein chains are made up 2 alpha and 2 beta chains.
Feta Hb: the four protein chains are made up of 2 alpha and 2 gamma chains.

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103
Q

Describe the inheritance pattern seen in sickle cell disease.

A

Autosomal Recessive.

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104
Q

When do symptoms of sickle cell disease start to present? WHy?

A

Between 3 and 6 months as HbF levels fall

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105
Q

Pathophysiology of sickle cell disease?

A

Sickling causes deformed and easily destroyed rbcs= occlusion of microcirculation and chronic haemolytic anaemia

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106
Q

Give 3 consequences of sickle cell disease.

A
  1. Life expectancy 20 years younger
  2. Anaemia.
  3. Infection.
  4. Painful crises.
  5. Stroke.
  6. Acute chest infection/infarction.
  7. Aplastic crises
  8. Splenic sequestration.
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107
Q

What is the affect of sickle cell disease on Hb and reticulocyte count?

A

Low Hb.
Raised reticulocyte count.

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108
Q

Describe the management for sickle cell disease.

A
  • Hydroxycarbamide.
  • Transfusions.
  • Stem cell transplants.

-prophylactic penicillin + pneumococcal, influenza and meningococcal vaccines to prevent infections

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109
Q

How does hydroxycarbamide help in sickle cell disease?

A

Increases foetal haemoglobin production

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110
Q

What is the advantage of being a sickle-cell carrier?

A

Protected against malaria.

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111
Q

Why are children with sickle cell more predisposed to infection?
How is this prevented?

A

Microvascular occlusion leads to splenic infarction leads to increased susceptibility to infection, specifically bacterial sepsis.
Prevented by:
-prophylactic penicillin
-pneumococcal, influenza and meningococcal vaccines.

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112
Q

What are some triggers for a painful crisis in sickle cell?

A

cold
dehydration
infection
hypoxia

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113
Q

What is acute chest syndrome in sickle cell?

A

Occurs after painful crises.
Presents with pain, resp distress, hypoxia, chest X ray signs.

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114
Q

How can you spot aplastic crises in a child with sickle cell?

A
  • very anaemic
  • low reticulocyte count (normally high in sickle cell)
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115
Q

Cause of aplastic crises in sickle cell?

A

Parvovirus B19 (slapped cheek)

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116
Q

Most common hereditary/intrinsic haemolytic anaemia in Europeans?

A

Spherocytosis.

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117
Q

How can spherocytosis be treated?

A

Splenectomy - can increase RBC survival.

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118
Q

Describe the inheritance pattern seen in hereditary spherocytosis.

A

Autosomal Dominant.

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119
Q

Describe the inheritance pattern for G6PD?

A

X-linked recessive

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120
Q

What can trigger haemolysis in G6PD?

A
  • eating broad beans
  • infection
  • antimalarials
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121
Q

What is haemolytic disease of the newborn?

A

transplacental maternal antibodies causing alloimmune haemolysis of foetal rbcs
Most commonly due to rhesus alloimmunisation (Rh + rbcs from foetus enter rh - maternal blood circulation)

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122
Q

Why does haemolytic disease of newborn occur then and not in utero?

A

In utero, bilirubin cleared by placenta
In neonate, liver does clearing and can’t handle high bilirubin load

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123
Q

Presentation of haemolytic disease of the newborn?

A

Jaundice
Pallor
Hepatosplenomegaly
Severe= oedema, ascites, petechiae

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124
Q

What anaemia will haemolytic disease of newborn present as?

A

Normocytic. Increased reticulocyte count

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125
Q

Management of haemolytic disease of newborn?

A 
50%= normal Hb and bilirubin and need monitoring for late onset anaemia at 6-8 weeks 
25%= mod disease and may require transfusion 
25%= severe disease= stillborn or have hydrops fetalis- require immediate resus, temp stabilisation, exchange transfusion.
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126
Q

What is fanconi anaemia?

A

X-linked/autosomal recessive condition
Bone marrow failure, solid tumours, leukaemia

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127
Q

How to manage fanconi anaemia?

A

Treat specific symptoms in each patient
Only curative= stem cell transplant
Cancer treatment
Surgery for skeletal malformations

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128
Q

What are the liver dependent clotting factors?

A

10, 9, 7, 2 = “1972”

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129
Q

Name 2 coagulopathies.

A
  1. Haemophilia.
  2. Von Willebrand disease
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130
Q

what factor is deficient in haemophilia a

A

clotting factor 8 (classic haemophilia, most common)

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131
Q

Describe the inheritance pattern seen in haemophilia.

A

X linked recessive.

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132
Q

what factor is deficient in haemophilia b

A

clotting factor 9 (also known as christmas disease)

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133
Q

how does severe haemophilia present?

A
  • Easy bruising
  • Mouth bleeds
  • Haematomas
  • Joint bleeds
  • Increased APTT
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134
Q

how does mild/mod haemophilia present?

A

Delayed presentation until following trauma or bleeds with surgery/dental extractions

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135
Q

Management of haemohpilia?

A

Factor VIII for haemophilia a
Factor IX for haemophilia b

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136
Q

How does von willebrand factor work?

A

Assists in platelet plug formation by attracting circulating platelets and binds to CF VIII, preventing its clearance from the plasma

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137
Q

Presentation of Von Willebrand disease?

A

Bleeding tendency from mucosa eg epistaxis/menorrhagia, spontaneous bleeding

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138
Q

How to manage von willebrand disease?

A

tranexamic acid and desmopressin
or
concetrates with vwf or factor VIII-vwf

Testing for 1st degree relatives

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139
Q

When does immune thrombocytopenia occur?

A

Commonly after viral infection or sometimes after immunisation

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140
Q

What diagnosis would you suspect in a child with a single figure platelet count but is otherwise well?

A

ITP.

They would have a normal blood film and clotting, just very low platelets.

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141
Q

Management of ITP?

A
  • Most children will not need treatment and recover spontaneously in weeks-months.
  • Avoid NSAIDs and aspirin
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142
Q

Give 3 signs of thrombocytopenia.

A
  1. Petechial rash.
  2. Bruising.
  3. Bleeding.
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143
Q

Give 2 causes of thrombocytopenia in children.

A
  1. ITP.
  2. Marrow failure.
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144
Q

What can trigger acute ITP?

A

Viral infection.

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145
Q

How to manage fanconi anaemia?

A

Treat specific symptoms in each patient
Only curative= stem cell transplant
Cancer treatment
Surgery for skeletal malformations

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146
Q

Describe rickets presentation

A

Rachitic rosary, limb deformity, weakness, misery

Metaphyseal swellings, bowing deformities, slowing of linear growth, motor delay, hypotonia, fractures, resp distress

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147
Q

Causes of low vit d/rickets?

A

Maternal vitamin D deficiency causes low stores in newborn, exclusive breastfeeding will exacerbate
Lack of dietary intake ie prolonged unsupplemented breastfeeding

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148
Q

What is rickets?

A

Severe vitamin D deficiency (child version of osteomalacia)

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149
Q

Pathophysiology of rickets?

A

lack of vit D; inadequate mineralisation of bone matrix
Decreased vit D > decreased calcium and phosphate > secondary hyperparathyroidism
In children, this occurs before the growth plates have closed.

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150
Q

Presentation of rickets?

A

Hypocalcaemic seizures or tetany, bony deformity (eg genu varum and valgum), irritable and reluctant to weight bear, severe can result in cardiomyopathy

Delayed walking, waddling gait, impaired growth, fractures, dental deformities

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151
Q

Investigating rickets?

A

Bloods, wrist X ray (long bone showing cupping, splaying and fraying of metaphysis eg champagne glass wrist) required for diagnosis

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152
Q

Management of rickets?

A

Diet, sunlight, vit D supplementation=oral calciferol and calcium supplement
Maintenance dose of calciferol is recommended for family members

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153
Q

What are the 3 main differentials for a limping child?

A
  1. Infection e.g. sepsis/osteomyelitis.
  2. Trauma e.g. NAI, fracture.
  3. Tumour.
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154
Q

What is the likely cause of a limp in a child aged 0-3?

A
  • Trauma - toddlers fracture, NAI
  • Infection - osteomyelitis, septic arthritis
  • Displasia - DDH
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155
Q

What is the likely cause of a limp in a child aged 3-10?

A
  • Trauma
  • Infection - transient synovitis, osteomyelitis
  • Perthe’s disease.
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156
Q

What is the likely cause of a limp in a child aged 10-15?

A
  • Trauma.
  • Infection - osteomyelitis
  • SUFE
  • Perthe’s disease
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157
Q

What must you remember to consider as a differential in a limping child?

A

Intra-abdominal pathology e.g. hernia, testicular torsion.

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158
Q

What investigations might you want to do on a child presenting with a limp?

A
  1. General observations e.g. HR, BP, T, RR, O2 sats.
  2. FBC, BM, ESR and CRP.
  3. XR - AP and lateral views of the the joint and the joints above and below.
  4. USS - effusion in joints?
  5. CT/MRI.
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159
Q

Give 3 signs of septic arthritis.

A
  • Children under 2
  • Systemically very unwell
  • Pain at rest
  • Raised WCC and CRP
  • Hip = flexed, abducted, externally rotated
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160
Q

Most common cause of septic arthritis?

A

S. aureus

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161
Q

Septic arthritis investigations and management?

A

FBC, ESR/CRP, synovial fluid examination and culture, blood cultures. Xray will show later changes (14-21 days)

Surgical emergency: surgical drainage and IV antibiotics- cefuroxime

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162
Q

Describe Kocher’s criteria. What is it used for?

A

For differentiating transient synovitis from septic arthritis.

  • non-weight bearing
  • temp> 38.5
  • ESR>40 mm/hr
  • WCC> 12000 cells/mm

3/4 = septic joint.

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163
Q

What is DDH?

A

DDH - developmental dysplasia of the hip.

Abnormal relationship of the femoral head to the acetabulum -> aberrant development of the hip.

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164
Q

What tests can be done on clinical examination in the neonatal period to pick up DDH?

A
  1. Ortolani test.
  2. Barlow manoeuvre.

Can be confirmed with USS.

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165
Q

Give 3 risk factors for DDH.

A
  1. Female (M:F - 1:8).
  2. First born.
  3. Breech birth.
  4. Family history.
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166
Q

age cut off for uss or pelvic x ray for DDH?

A

Under 4.5 months = USS
Over 4.5 months= pelvic XR

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167
Q

Describe the management of DDH.

A
  1. Pavlik harness.
  2. Surgical reduction.

Prevention = safe swaddling

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168
Q

What are the 2 main risks associated with the surgical management of DDH.

A
  1. Avascular necrosis.
  2. Re-dislocation.
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169
Q

What is the most common cause of hip pain/limp in children aged 3-10?

A

Transient synovitis

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170
Q

What is transient synovitis?

A

Acute onset joint inflammation following illness, often respiratory.

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171
Q

How does transient synovitis differ from septic arthritis?

A

Transient synovitis:

  • no pain at rest
  • systemically well
  • rest, physiotherapy and NSAIDs often help
  • no findings on esr/crp, xray, uss
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172
Q

Transient synovitis management?

A
  • no long term sequelae and self limiting
  • Simple analgesia, rest and physio
  • Usually resolves within 2 weeks
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173
Q

What is Perthe’s disease?

A

A self-limiting idiopathic disease characterised by avascular necrosis of the femoral head.

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174
Q

Key points in history for Perthe’s disease?

A
  • 5-10 yr olds
  • Developed over weeks
  • No history of trauma
  • All hip movements limited
  • May have referred pain to groin/thigh/knee
  • Systemically well
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175
Q

Describe the management of Perthe’s disease.

A
  • If bone age under 6 years: activity restriction, physio, nsaids
  • If bone age over 6 years: surgery
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176
Q

Give 3 risk factors for Perthe’s disease.

A
  1. ADHD.
  2. Deprivation.
  3. Passive smoking.
  4. LBW.
  5. Short stature.
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177
Q

What is SUFE?

A

Slipped upper femoral epiphysis - slippage of the femoral head

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178
Q

Who is likely to be affected by SUFE?

A

A pre-pubescent obese male.

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179
Q

How does SUFE present?

A
  • several week history of vague groin/thigh discomfort
  • Drehmann’s sign (passively flex hip, falls back into external rotation and abduction)
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180
Q

What is the treatment for SUFE?

A

Surgical pinning of the hip.

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181
Q

Give 3 signs of osteomyelitis in children.

A
  1. Joint pain.
  2. Lethargy.
  3. Fever.
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182
Q

Name an organism that commonly causes osteomyelitis in children.

A

Staphylococcus aureus.

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183
Q

Most common site of osteomyelitis in children?

A

Distal femur and proximal tibia

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184
Q

Pathophysiology of osteomyelitis?

A

Infection of bone marrow, commonly S. aureus
Inflammatory destruction of bone.
If periosteum involved: necrosis and detachment forming a sequestrum
Bony remodelling causes deformity

NB/ involucrum= viable periosteum separated from underlying bone and forms new bone around it

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185
Q

What is the likely mechanism of osteomyelitis in children?

A

Haematogenous spread- tends to occur in rapidly growing and highly vascular metaphysis of growing bones (long bones more common)

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186
Q

Investigating osteomyelitis?

A

FBC, ESR/CRP, blood cultures, bone cultures (gold standard), MRI

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187
Q

Management of osteomyelitis?

A

Local bone and soft tissue debridement, stabilisation of bone, local antibiotic therapy, reconstruction of soft tissue, reconstruction of osseous defect zone

Antibiotic therapy 4-6 weeks if acute, at least 12 weeks if chronic

IV cefuroxime or IV flucloxacillin, switch to PO if improving.

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188
Q

What is Kohler’s disease?

A

Osteochondrosis of tarsal navicular bone

A non inflammatory, none infectious derangement of bony growth, affecting the epiphyses

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189
Q

Presentation of kohler’s disease?

A

Unilateral antalgic gait, local tenderness of medial aspect of foot over navicular bone

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190
Q

Management of kohler’s disease?

A

Rest, analgesia, avoid excessive weight bearing, short leg cast for immobilisation, treat for at least 6 weeks
Chronic course but rarely over 2 years

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191
Q

3 types of discoid meniscus?

A

Incomplete (bit thicker and wider than normal), complete (tibia completely covered by meniscus) and hypermobile wrisberg (normal shape but no posterior attachment to tibia)

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192
Q

How will a discoid meniscus present?

A

More prone to injury than normal shaped meniscus
Some may never experience problems
Most cases= knee problems, vague pain, audible snap on terminal extension, swelling, locking

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193
Q

Management of discoid meniscus?

A

If asx, do nothing
otherwise, arthroscopic partial meniscectomy and rehab

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194
Q

What is osgood schlatter disease?

A

Self limiting disorder of the knee
generally in active adolescents before tibial tuberosity has finished ossification, during adolescent growth spurt

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195
Q

Presentation of osgood schlatter disease?

A

Gradual onset pain and swelling below knee, relieved by rest, provoked by knee extension or hyperflexing while prone

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196
Q

Management of osgood schlatter disease?

A

Conservative: rest, ice, physio and exercise advice, simple analgesia

Most patients able to return to activity after 2-3 weeks

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197
Q

What is the criteria for making a clinical diagnosis of juvenile idiopathic arthritis?

A

Joint swelling/stiffness >6 weeks in children <16 and no other cause is identified.

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198
Q

What symptoms are associated with JIA?

A
  1. Fever.
  2. Salmon-pink rash.
  3. Uveitis.
  4. Pain.
  5. Morning stiffness.
  6. Swelling.
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199
Q

Give 5 potential consequences that can occur if you fail to treat JIA.

A
  1. Damage.
  2. Deformity.
  3. Disability.
  4. Pain.
  5. Bony overgrowth.
  6. Uveitis.
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200
Q

Subtypes of juvenile idiopathic arthritis?

A

Oligoarticular (1-4 joints)
Polyarticular (at least 5 joints)- RF positive or negative
Systemic
Psoriatic
Enthesitis-related
Undifferentiated

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201
Q

Qualifying factors for it to be systemic JIA?

A

at least 2 weeks of fever with at least one of: rash, ln enlargement, hepato/splenomegaly, serositis= pericarditis/pleuritis/peritonitis

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202
Q

Qualifying factors for it to be psoriatic arthritis (JIA)?

A

Arthritis and psoriasis
or
arthritis and at least 2 of: dactylitis, nail pitting, onycholysis, psoriasis in first degree relative

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203
Q

Qualifying factors for it to be enthesitis related arthritis?

A

Arthritis or enthesitis and 2 of:
sacroiliac/lumbosacral pain, HLA b27 positive, family history HLA b27 related disease, anterior uveitis

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204
Q

Describe the non-medical treatment for JIA.

A
  • Education, support, liaison with school
  • Physiotherapy
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205
Q

Describe the medical treatment for JIA.

A
  1. Steroid joint injections.
  2. NSAIDS.
  3. Methotrexate.
  4. Systemic steroids.

5.biologics- etanacerpt.

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206
Q

What extra-articular features might you see in someone with JIA?

A
  1. Psoriasis.
  2. Dactylitis.
  3. Nail pitting.
  4. Rash.
  5. Fluctuating fever.
  6. Uveitis.
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207
Q

Name a severe, potentially life-threatening complication of systemic onset JIA.

A

Macrophage-activation syndrome (MAS).

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208
Q

What signs might you see in someone with macrophage-activation syndrome (MAS)?

A
  • High fever.
  • Hepatosplenomegaly.
  • CNS dysfunction.
  • Purpuric rash.
  • Cytopaenia.
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209
Q

What is the treatment for macrophage-activation syndrome (MAS)?

A
  • Supportive treatment.
  • Steroids.
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210
Q

Give 5 differentials for a painful joint.

A

Life-threatening differentials:

  • Leukaemia.
  • Septic arthritis.
  • NAI.

Pain and swelling:

  • Trauma.
  • Infection.
  • Reactive arthritis.
  • JIA.

Pain and no swelling:

  • Hypermobile joint syndrome.
  • Perthe’s disease.
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211
Q

Define child development.

A

The biological, psychological and emotional changes that occur between birth and adolescence as the individual progresses from dependency to increasing autonomy. It is a continuous process with a predictable sequence however each child’s development is unique.

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212
Q

Give 5 influences on a child’s development.

A
  1. Genetic factors.
  2. Stimulating environment.
  3. Pregnancy factors e.g. premature? Mums health?
  4. Healthy attachment.
  5. Medical conditions.
  6. Abuse/neglect/domestic violence.
  7. Healthy peer relationships.
  8. Education.
  9. Nutrition.
  10. Parenting style.
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213
Q

What are the 4 domains of child development?

A
  1. Gross motor.
  2. Fine motor and vision.
  3. Speech, language and hearing.
  4. Social interaction and self care skills.
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214
Q

What are the developmental milestones for gross motor function?

A
  • Newborn: flexed arms and legs, equal movement in all 4 limbs
  • 3m: lifts head on tummy.
  • 6m: chest up with arm support, can sit unsupported.
  • 9m: craws, pulls to stand
  • 12m: walking
  • 2 years: walks up stairs, runs
  • 3 years: jumps
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215
Q

With regards to gross motor development, at what age would you expect a child to do the following:

a) walking.
b) jumping.
c) crawling.
d) walking up stairs.

A

a) Walking - 12 months.
b) Jumping - 3 years.
c) Crawling - 8 months.
d) Walking up stairs - 2 years.

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216
Q

What are the developmental milestones for fine motor and visual function?

A
  • 6 weeks: fix and follow
  • 6 mo: palmar grasp (picks up cube)
  • 1 yr: pincer grip
  • 1.5 yrs mo: tower of 3 cubes
  • 2 yrs: tower of 6 cubes, draw straight line
  • 3 yrs: bridge of 3 cubes, draw circle
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217
Q

With regards to fine motor and visual development, at what age would you expect a child to do the following:

a) drawing with crayons.
b) building a tower of 8 cubes.
c) takes an object in each hand.
d) builds a tower of 2 cubes.

A

a) Drawing with crayons - 12m.
b) Building a tower of 6 cubes - 2 years.
c) Takes an object in each hand - 6m.
d) Builds a tower of 3 cubes - 18m.

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218
Q

What are the developmental milestones for speech, language and hearing?

A
  • newborn: startles to loud noises
  • 6 mo: babbles, turn to sound
  • 9 mo: responds to own name, ‘dada’, ‘mama’
  • 12 mo: 1 proper word
  • 18 mo: nouns e.g. body parts, teddy
  • 2 years: verbs e.g. eat, 50+ words, simple sentences (give teddy)
  • 3 years: adjectvies e.g. colours, speech mainly understandable
  • 4 years: can count 5 objects
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219
Q

With regards to speech, language and hearing, at what age would you expect a child to do the following:

a) form short sentences and name body parts.
b) knows colours and can count.
c) laughs and squeals.
d) has mainly understandable speech.

A

a) Forms short sentences and name body parts - 2 years.
b) Knows colours and can count - 4 years.
c) Laughs and squeals - 3 months.
d) Has mainly understandable speech - 3 years.

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220
Q

What are the developmental milestones for social interaction and self-care skills?

A

6 weeks: smile spontaneously
6 mo: finger feeds
9/10 mo: wave bye bye, stranger danger
12 mo: drink from cup, uses spoon/fork
2 years: undresses, dry by day
3 years: parallel play, interactive play evolving, put on t shirt
4 years: play simple board game, gets dressed themself

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221
Q

With regards to social interaction and self-care skills, at what age would you expect a child to do the following:

a) uses cutlery.
b) plays with others, names a friend.
c) smiles.
d) waves bye-bye.

A

a) Uses cutlery - 12 months.
b) Plays with others, names a friend - 3 years.
c) Smiles - 6 weeks.
d) Waves bye-bye - 9/10 months.

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222
Q

What does ‘The healthy child programme’ encourage?

A
  1. Encourages care to keep children healthy and safe.
  2. Promotes healthy eating and activity.
  3. Identifies problems in children’s development.
  4. Identifies ‘at risk’ families for more support.
  5. Ensures children are prepared for school.
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223
Q

Gross motor developmental red flags?

A
  1. Not sitting by 12 months.
  2. Not walking by 18 months.
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224
Q

Fine motor developmental red flag?

A

Hand preference before 18 months (cerebral palsy)

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225
Q

Speech and language developmental red flag?

A

No clear words by 18 months - ASD? Language problems?

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226
Q

3 social developmental red flags?

A
  • No smiling by 3 months
  • No response to carers interactions by 8 weeks.
  • No interest in playing by 3 years.
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227
Q

Give 5 causes of developmental delay.

A
  1. Genetics.
  2. Pregnancy.
  3. Factors around birth.
  4. Factors in childhood.
  5. Environmental.
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228
Q

Causes of developmental delay: give examples of genetic causes.

A
  1. Chromosomal disorders e.g. Down’s syndrome.
  2. Single gene disorders e.g. Duchenne.
  3. Polygenic e.g. ASD, ADHD.
  4. Micro-deletions or micro-duplications.
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229
Q

Causes of developmental delay: give examples of pregnancy related causes.

A
  1. Congenital infections e.g. CMV, HIV.
  2. Exposure to drugs/alcohol e.g. FAS.
  3. MCA infarct.
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230
Q

Causes of developmental delay: give examples of birth related causes.

A
  1. Prematurity.
  2. Birth asphyxia (due to hypoxia).
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231
Q

Causes of developmental delay: give examples of medical causes that may occur during childhood.

A
  1. Infections e.g. meningitis.
  2. Chronic illness.
  3. Hearing or visual impairment.
  4. Acquired brain injury.
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232
Q

How might you investigate developmental delay?

A

Thorough history and examination. Tailor any investigations to the child e.g.

  • Boys not walking by 18m check creatinine kinase for Duchenne.
  • Focal neurological signs -> MRI brain.
  • Genetic testing.
  • Unwell, failure to thrive -> metabolic investigations.

There is no ‘developmental screen’, investigations need to be tailored towards to the child.

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233
Q

Causes of developmental delay: give examples of environmental causes.

A
  1. Abuse and neglect.
  2. Low stimulation.
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234
Q

What is global developmental delay?

A

Significant delays in at least two of the four areas of development.

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235
Q

Types of epilepsy in childhood?

A

Absence seizures
Childhood epilepsy syndrome (‘benign’ if can predict from EEG that will stop by certain age)
Infantile spasms=West syndrome
Bects: Rolandic epilepsy (benign epilepsy with centro-temporal spikes)
Juvenile myoclonic epilepsy

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236
Q

Define seizure.

A

A paroxysmal abnormality of motor, sensory, autonomic and/or cognitive function due to transient brain dysfunction.

-may be epileptic or non-epileptic

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237
Q

Types of seizures? (epileptic and non-epileptic)

A

Epileptic

  • Epilepsies
  • Acute asymptomatic seizures
  • Febrile seizures

Non epileptic

  • convulsive syncope e.g. cardiac syncope, neurally-mediated, hypovolaemic, expiratory-apnoea syncope (blue breath holding spells)
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238
Q

Define convulsion.

A

A convulsion is a seizure, either epileptic or non-epileptic, with motor components e.g.

  • stiffening=tonic
  • a massive jerk=myoclonic
  • jerking=clonic
  • thrashing about=hypermotor

as opposed to a non-convulsive seizure with motor arrest e.g. unresponsive stare, drop attack

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239
Q

Define epileptic seizure.

A

What makes a seizure eplieptic is the nature of the underlying electival activity in the brain: excessive, hypersynchronous neuronal discharges in all or part of the cerebral cortex.

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240
Q

Define epilepsy.

A

An umbrella term brain disorders that predispose the patient to having epileptic seizures.

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241
Q

How long do epileptic seizures tend to last for?

A

30 - 120 seconds.

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242
Q

Give 3 signs of epileptic seizures.

A
  1. Movement.
  2. Tongue biting.
  3. Head turning.
  4. Muscle pain.
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243
Q

Causes of epilepsies?

A

-genetic (70%): caused by allelles at several lociso inhertiace is complex

-structural, metabolic: cerebral dysgenesis, damage (vascular occlusion, infection)

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244
Q

What are febrile convulsions?

A

Febrile convulsions are epileptic seizures accompanied by fever. They usually occur early in viral infection and tend to be brief generalised tonic-clonic seizures.

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245
Q

Px of febrile convulsion?

A

Last less than 5 mins, child becomes stiff, then limbs twitch, lose consciousness and may wet/soil themselves

May be sick, foam at mouth and eyes may roll back
Sleepy for up to an hour after event

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246
Q

What is a complex febrile seizure?

A

Lasting longer than 15 mins

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247
Q

How long do non-epileptic seizures tend to last for?

A

1 - 20 minutes.

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248
Q

Give 3 signs of non-epileptic seizures.

A
  1. Eyes closed.
  2. Talking/crying.
  3. Pelvic thrusting.
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249
Q

What 2 broad categories can epileptic seizures be divided into?

A

Generalised and focal.

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250
Q

Give 3 examples of generalised seizures.

A
  1. Absence.
  2. Myoclonic.
  3. Tonic.
  4. Atonic.
  5. Generalised tonic-clonic.
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251
Q

What are absence seizures?

A

Seizures where there is a transient loss of consciousness with an abrupt onset and termination. Momentary unresponsive stare with motor arrest, lasts <30s. Developmentally normal but can interfere with school.

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252
Q

Give some possible side effects of the treatment for absence seizures in children?

A
  • Sodium Valporate - weight gain, hair loss, teratogenic.
  • Ethosuxamide N+V
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253
Q

How might you investigate suspected absence seizures?

A

. Observe an episode - hyperventilation, ask the child to blow on a windmill.
2. EEG - would show 3-second spike and wave discharges.

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254
Q

Absence seizure meds?

A
  1. Sodium valproate/ ethusoxemide for girls
  2. Clobazam
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255
Q

What would you expect to see in a tonic seizure?

A

Generalised increase in tone.

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256
Q

What can absence seizures evolve into?

A

Juvenile myoclonic epilepsy (JME).

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257
Q

What are the signs of juvenile myoclonic epilepsy?

A

Clumsiness and GTCS that occur shortly after waking and are often provoked by sleep deprivation.

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258
Q

What would you expect to see in a generalised tonic-clonic seizure?

A

Sudden onset rigid phase followed by a convulsion in which the muscles jerk rhythmically.

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259
Q

Treatment for tonic-clonic seizures in children?

A
  1. Sodium valproate, carbamazepine for girls
  2. Clobazam
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260
Q
  • Side effects of first line treatment for tonic-clonic seizures?
  • Contraindication?
A
  • Sodium valproate - weight gain, hair loss, teratogenic
  • Carbmazepine - rash, hyponatraemia, can interfere with contraception. Contraindicated in absence and myoclonic seizures.
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261
Q

What would you expect to see in a myoclonic seizure?

A

Isolated muscle jerking.

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262
Q

First and second line AEDs for focal seizures?

A
  1. Lamotrigine/carbmazepine, sodium valproate (not for girls)
  2. Clobazam
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263
Q

What investigations might you want to do in someone presenting with seizures.

A
  1. Eye witness account/video is invaluable!
  2. ECG.
  3. EEG.
  4. MRI or CT.
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264
Q

Why must you do an ECG in those suffering from seizures?

A

To check for arrhythmia as the cause e.g. long-QT syndrome.

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265
Q

Give 3 potential side effects of AED’s.

A
  1. Cognitive disturbances
  2. Heart disease.
  3. Drug interactions.
  4. Teratogenic.
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266
Q

Name common epilepsy syndromes in order of increasing age.

A
  • 3-12 months: Infantile spasms (west syndrome)
  • 1-3 yrs: Lennox-Gaustat
  • 4-10 years: Benign rolandic epilepsy (15%), childhood absence epilepsy (2%)
  • 10-20 yrs: Juvenile absence, juvenile myoclonic

-name does not necesarily correspond to seizures e.g. can have absence and tonic-clonics in “juvenile myoclonic epilepsy”

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267
Q

Name 3 conditions hat are commonly misdiagnosed as epilepsy.

A
  1. Sandifer syndrome.
  2. Benign neonatal sleep myoclonus.
  3. Syncope.
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268
Q

What is syncope?

A

Insufficient blood or O2 supply to the brain causes paroxysmal changes in behaviour, sensation and cognitive processes.

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269
Q

What non-neurological disease is sandifer syndrome associated with?

A

GORD.

Patients present with GORD and a characteristic neck movement disorder.

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270
Q

Common causes of ‘funny turns’? (paroxysmal disorders)

A
  • blue breath holding spells (toddlers)
  • reflex anoxic syncope (triggered by pain, fright, discomfort)
  • syncope
  • migraine
  • vertigo
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271
Q

Describe a floppy infant.

A

Hypotonia, weakness, ligamentous laxity, increased range of joint mobility

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272
Q

What features suggest central hypotonia/UMN in children?

A

Normal strength, dysmorphic features, normal/brisk tendon reflexes, irritability +/- loud cry, history suggestive of HIE (hypoxic-ischaemic encephalopathy)/birth trauma/sxs hypoglycaemia,seizures

Central=2/3 cases, commonly HIE

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273
Q

What are indicators of peripheral hypotonia/LMN in children?

A

Decreased strength, reduced/absent reflexes, fasciculation, myopathic face, weak cry

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274
Q

Causes of floppy infant?

A

Central hypotonia=acute encephalopathies (HIE, hypoglycaemia, intracranial haemorrhage), chronic encephalopathies (cerebral malformations, metabolism errors, chromosomal disorders, endocrine disorders, metabolic disorders), connective tissue disorders (Ehler-Dahlos, OI)

Peripheral hypotonia= spinal cord (syringomyelia), anterior horn cell (spinal muscular atrophy), NMJ (MG), muscular disorders (dystrophies, myopathies), peripheral nerves, metabolic myopathies

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275
Q

What is cerebral palsy?

A

Non progressive cerebral pathology that leads to a disorder of movement and posture in children.

Damage to immature brain (most between 24 weeks and term)

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276
Q

Why might the clinical signs of cerebral palsy change over time?

A

The clinical signs may change over time as the brain matures but the underlying aetiology is not progressive.

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277
Q

Classification of cerebral palsy?

A
  • Spastic
  • athetoid (hyperkinesia)
  • ataxic
  • mixed
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278
Q

Give 3 causes of cerebral palsy.

A

80% antenatal - hypoxia, infection, haemorrhage, ischaemia.

10% peri-natal - hypoxia, infection, haemorrhage.

10% postnatal - hypoxia, infection e.g. meningitis, haemorrhage, encephalopathy, trauma.

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279
Q

Presentation of cerebral palsy?

A

Motor features- mono/hemi/para/quadriplegia
Don’t meet developmental milestones eg not sitting by 8 months, not walking by 18 months, early hand preference before 1 year
GORD, epilepsy, vomiting, constipation, bladder issues, drooling, orthopaedic problems all common

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280
Q

How to diagnose cerebral palsy?

A

Definitive dx may not come until 12-18 months
Clinical observation and parental observation

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281
Q

Describe the support that is offered to someone with cerebral palsy.

A
  1. Physiotherapists for mobility and hand function.
  2. SALT for communication.
  3. Feeding support.
  4. Sleeping support.

Support for children with disabilities needs to be holistic, child focused and with an MDT approach.

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282
Q

What drug can be used to treat hypertonia in children with cerebral palsy?

A

Botox

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283
Q

Fits, faints and funny turns causes?

A

In sleep: benign neonatal sleep myoclonus and parasomnias
On feeding: GORD and sandifer syndrome
Fever: febrile seziures, vaso-vagal syncope
Pain/shock/startle: reflex asystolic syncope, cyanotic breath holding, hyperekplexia
Tired/bored/stress: self gratification behaviour, tics, daydreaming
Excitement: shuddering spells, cataplexy

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284
Q

Potential childhood motor disease?

A

Tourette syndrome and tics, tremor, dystonia, ataxia, restless legs syndrome, myoclonus, juvenile huntington disease

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285
Q

DSM-V criteria for ADHD

A

Symptoms must be present before age 12, and present in at least 2 settings, affect functioning.

At least 6 symptoms of inattention for at least 6 months
or
At least 6 symptoms of hyperactivity for at least 6 months

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286
Q

Non drug management of ADHD

A

parent education and training
CBT can be offered
Meds not advised for pre-school children

Advice: plan day, clear boundaries, brief and specific instructions, incentive schemes, kep social situations short and sweet, exercise, healthy diet, bedtime routine, help at school

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287
Q

Medical management of ADHD

A

Stimulant drugs (increase dopamine):
Short acting methylphenidate eg ritalin
Long acting methylphenidate eg delmosart
Lisdexamfetamine

Non stimulant drugs (reduced breakdown of noradrenaline):
Atomoxetine (SNRI), guanfacine

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288
Q

Describe autism spectrum disorders

A

Abnormal development from under 3 years old in:

  • social interaction
  • communication
  • restricted, stereotyped and repetitive behaviour

Diagnosis requires at least 6 symptoms across 3 core areas

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289
Q

Management of autism spectrum disorders

A

Non-pharm management
behavioural therapies, social skills groups, OT, communication interventions, input from dietician if needed, specialised educational programs and structured support

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290
Q

ICD-10 for anorexia nervosa

A 
Deliberately keeping weight below 85% of expected via restricted diet choice, excessive exercise, induced vomiting, use of appetite suppressants and diuretics 
Dread of fatness-intrusive overvalued idea 
Endocrine effects (menstruation stops/puberty delayed if menarche not yet achieved, loss of sexual interest in men)
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291
Q

How to screen for eating disorders?

A

SCOFF

Do you make yourself sick because you’re uncomfortably full?
Do you worry that you’ve lost control over how much you eat?
Have you recently lost more than one stone (about 6kg) in 3 months?
Do you believe you are fat when others say you are thin?
Would you say that food dominates your life?

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292
Q

Potential clinical signs of anorexia nervosa?

A

Dry skin, lanugo hair, orange skin and palms, cold hands and feet, bradycardia, drop in BP on standing, oedema, weak proximal muscles (squat test)

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293
Q

Management of anorexia nervosa?

A
  • *1. Family therapy.**
    2. IPT.
    3. CBT.

Weight restoration at 0.5kg/week - monitor for re-feeding syndrome.

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294
Q

Define bulimia nervosa

A

Binges followed by compensatory weight loss behaviours eg self induced vomiting, fasting, intensive exercise, abuse of medication
BMI maintained at over 17.5

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295
Q

Describe the aetiology of ADHD.

A
  • Genetic and environmental.
  • Neuroanatomical and neurochemical factors too.
  • CNS insults e.g. FAS or premature.
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296
Q

ASD signs: communcation, social interaction and social imagination

A
  • Communication: non-verbal communication challenging, only communicate needs, no understanding of jokes
  • Interaction: poor eye contact, struggles to understand social roles
  • Imagination: no imaginative play, obsessions/rituals struggles with change
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297
Q

RF’s for developing anorexia?

A
  1. Social pressure.
  2. Perfectionist traits.
  3. Family attitudes to food.
  4. Low self esteem.
  5. Occupation/interests.
  6. Family history.
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298
Q

Give 5 symptoms of depression.

A
  1. Loss of interest.
  2. Fatigue.
  3. Poor sleep.
  4. Reduced appetite.
  5. Low concentration.
  6. Feelings of guilt and self blame.
  7. Low confidence.
  8. Agitated.
  9. Hopeless.
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299
Q

Describe the non-medical and medical treatment of depression.

A

Non-medical: Education, CBT, IPT and family therapy.

Medical: fluoxetine, sertraline, citalopram.

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300
Q

Give 3 predisposing factors for a child developing depression.

A
  1. Family history.
  2. Stress in pregnancy.
  3. Poor attachment.
  4. Poverty.
  5. Isolation.
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301
Q

Give 3 precipitating factors for a child developing depression.

A
  1. Trauma.
  2. Drugs.
  3. Infections.
  4. Puberty.
  5. Exam stress.
  6. Sexual abuse.
  7. Bullying.
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302
Q

Give 3 perpetuating factors for a child developing depression.

A
  1. Chronic illness.
  2. Malnutrition.
  3. Ongoing neglect.
  4. Ongoing poverty.
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303
Q

Describe the foetal circulation.

A

Placenta -> umbilical vein -> IVC -> RV -> foramen ovale -> LA -> aorta -> umbilical arteries -> placenta.

OR: … RV -> pulmonary artery -> ductus arteriosus -> aorta …

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304
Q

What is the function of the foramen ovale and the ductus arteriosus in the foetal circulation?

A

They are used to bypass the non-functiong lungs.

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305
Q

Name 4 congenital heart problems that can cause a L -> R shunt.

A
  1. VSD.
  2. ASD.
  3. AVSD.
  4. PDA.
306
Q

Most common septal defect?

A

VSD

307
Q

Give 5 signs of a VSD.

A
  1. Poor feeding and FTT.
  2. Tachypnoea.
  3. Thrill.
  4. Pan-systolic murmur.
  5. Increased work of breathing.
308
Q

You request a CXR for a patient with a VSD. What would you expect to see?

A
  1. Cardiomegaly.
  2. Pulmonary oedema.
  3. Enlarged pulmonary arteries.
309
Q

Gold standard diagnosis of septal defect?

A

Echo

310
Q

Murmur for VSD and where to listen?

A
  • VSD = very systolic = pansystolic.
  • Best heard over tricuspid area (lower left sternal border)
311
Q

Management of VSD?

A

Qp/Qs (pulmonary to systemic blood flow ratio) of at least 2.0 requires surgical repair

Aim to close large VSDs within first 2 years of life

75% of small VSDs close spontaneously by age 10

312
Q

Why are ASD’s often asymptomatic?

A

ASD’s are often asymptomatic because the blood flow in the atria is low pressure and so breathlessness etc is uncommon.

313
Q

Most common ASD?

A

Patent foramen ovale.

314
Q

Type of murmur in ASD?

A
  • a split double s2
  • best heard in pulmonary area (upper left sternal edge)
315
Q

RFs for ASD

A
  • maternal smoking in first trimester
  • maternal diabetes
  • maternal rubella
  • maternal drug use eg cocaine/alcohol
316
Q

Give 5 signs of a PDA.

A
  1. Poor feeding, FTT.
  2. Tachypnoea.
  3. Active precordium.
  4. Thrill.
  5. Continuous machinery murmur.
317
Q

Management of ASD?

A

If less than 5mm, spontaneous closure within 12 months
Surgical closure required if bigger than 1cm

318
Q

Describe the management for congenital health defects that cause a L->R shunt.

A
  1. Stabilise the patient.
  2. Increase calorie intake.
  3. NG tube.
  4. Diuretics and ACEi to prevent HF symptoms.
  5. Surgical repair.
319
Q

AVSD is a common defect in people with which chromosomal abnormality?

A

Trisomy 21 (Down’s syndrome).

320
Q

Describe the pathaphysiology of an AVSD?
2 types

A

Failure of endocardial cushions to fuse properly
Complete AVSD: large left to right shunt causes excessive pulmonary blood flow leading to HF and pulmonary vascular resistance
Partial AVSD: left to right shunt and volume overload of RA and RV but the pulmonary artery pressure is only mildly elevated

321
Q

Presentation of AVSD?

A

Tachypnoea, tachycardic, poor feeding, sweating, failure to thrive, all with a complete AVSD are symptomatic by 1 year
Prominent sternal heave, murmur

322
Q

Murmur in AVSD and where to auscultate?

A

Ejection systolic murmur in pulmonary area (left upper sternal border)
Mid-diastolic murmur over tricuspid area (left lower sternal border)
Holosystolic murmur might be heard at apex

323
Q

Management of AVSD?

A

Surgical management as patients would die by age 2-3 without

324
Q

Name 2 congenital heart problems that can cause a R -> L shunt.

A
  1. Tetralogy of fallot.
  2. Transposition of the great arteries.
325
Q

What 4 components make up Tetralogy of fallot?

A
  1. Ventricular septal defect
  2. Pulmonary stenosis
  3. Right ventricular hypertrophy
  4. Overriding aorta

Most common cyanotic congenital heart disease

326
Q

Give 3 signs of Tetralogy of fallot.

A
  1. Cyanosis.
  2. Collapse.
  3. Acidosis.
327
Q

If tetralogy of fallot isn’t managed, what can occur?

A

polycythaemia, cerebral abscess, stroke, infective endocarditis, HF, death

328
Q

Murmur in tetralogy of fallot and where to listen?

A

Pansystolic murmur best heard over mid or upper left sternal edge
Smaller the VSD=louder the murmur

329
Q

What are tet spells?

A

Hypoxic spells in tetralogy of fallot with peak incidence between 2-4 months of life:

Paroxysm of hyperpnoea, irritability, increasing cyanosis

330
Q

Management of tetralogy of fallot?

A

Medical= squatting, prostaglandin infusion, beta blockers, morphine, saline bolus
Surgical management for definitive repair or palliative management

331
Q

Which defects have a higher risk of HF?

A

Significant left to right shunt= VSD, ASD, PDA
Transposition of the great arteries

332
Q

Describe the two types of transposition of the great arteries?

A

dextro-transposition=aorta is anterior and to the right of the pulmonary artery

levo-TGA= aorta anterior and to the left of the pulmonary artery

333
Q

What is coarctation of the aorta?

A

Arterial duct tissue encircles the aorta at the point of insertion of the duct. When the duct closes, the aorta constricts, this causes severe obstruction to LV outflow.

334
Q

When do you get cyanotic heart disease?

A

Absent/not wide enough tricuspid/pulmonary/aortic valve
Coarctation of aorta
Ebstein anomaly
Tetralogy of fallot
TGA
Truncus arteriosus

Anywhere with a right to left shunt

335
Q

What is rheumatic fever?

A

Systemic illness 2-4 weeks after pharyngitis due to cross reactivity to group A beta-haemolytic streptococcus (S. pyogenes)

336
Q

What are the diagnostic criteria for rheumatic fever? Describe

A

Revised Jones diagnostic criteria= positive throat culture or anti-streptolysin O/anti-DNA B titre and 2 major or 1 major+2 minor criteria

337
Q

What are the major criteria in the revised jones criteria?

A

SPECS
Sydenham’s chorea, polyarthritis, erythema marginatum, carditis, subcut nodules

338
Q

What are the minor criteria in the revised jones criteria?

A

CAPE
CRP/ESR raised
Arthralgia
Pyrexia
ECG-prolonged PR

339
Q

Management of acute rheumatic fever?

A

Antibiotics=benzathine benzylpenicillin
Aspirin/NSAIDs
Assess for emergency valve replacement

Secondary prevention with abx every 3-4 weeks

340
Q

What factors lead to subacute bacterial endocariditis?

A

Endothelial damage, platelet adhesion, microbial adherence

341
Q

What organisms cause IE?

A

S. aureus, S. viridans, S. pneumoniae, HACEK organisms

342
Q

Presentation of IE?

A

persistent low grade fever, heart murmur, splenomegaly, petechiae/oslers nodes/janeway lesions/splinter haemorrhages

343
Q

Criteria for diagnosis of IE? describe

A

Modified Dukes criteria

2 major
1 major and 3 minor
5 minor

344
Q

Major criteria in modified dukes?

A

positive blood cultures, evidence of endocardial involvement (echo)

345
Q

Minor criteria in modified dukes?

A

predisposition, fever, vascular phenomena, immunologic phenomena, microbiology evidence, other echo findings

346
Q

Management of IE?

A

IV antibiotics
May require surgical intervention

347
Q

What is coarctation of the aorta?

A

Arterial duct tissue encircles the aorta at the point of insertion of the duct. When the duct closes, the aorta constricts, this causes severe obstruction to LV outflow.

348
Q

Give 3 signs of coarctation of the aorta.

A
  1. Radio-femoral delay.
  2. Weak femoral pulses.
  3. Difference in pre and post-ductal saturations.
349
Q

Give 3 signs of aortic stenosis.

A
  1. Palpable thrill.
  2. Ejection systolic murmur.
  3. LVH.
350
Q

What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L->R shunt?

A

Eisenmenger syndrome: high pressure pulm. blood flow damages pulmonary vasculature -> increased resistance (pulm. hypertension) -> RV pressure increase -> shunt direction reverses -> CYANOSIS!

351
Q

What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L->R shunt?

A

Eisenmenger syndrome: high pressure pulm. blood flow damages pulmonary vasculature -> increased resistance (pulm. hypertension) -> RV pressure increase -> shunt direction reverses -> CYANOSIS!

352
Q

What factors aid reflux in GORD?

A

Low tone of muscular portion of lower oesophagus, short and narrow oesophagus, delayed gastric emptying, shorter lower oesophageal sphincter, liquid diet and high calorie requirement (distends stomach), significant periods recumbent

353
Q

Presentation of GORD?

A

Distressed, unexplained feeding difficulties, hoarseness +/- chronic cough, single episode pneumonia, faltering growth, if able may report pain

354
Q

When does GORD or regurgitation (=posseting) present normally

A

Within 2 weeks of life

355
Q

How to manage a breast fed baby with gord

A

Use alginate eg gaviscon mixed with water after feeds

356
Q

How to manage a formula fed baby with gord

A 
1= do not over feed (less than 150ml/kg/day) 
2= decreased feed volume by increasing frequency (2-3 hourly) 
3= use feed thickener 
4= stop thickener and use alginate added to formula
357
Q

What to use after alginate if no success in GORD?

A

histamine antagonists or ppi

358
Q

Name 5 potential causes of poor feeding

A

Infection, metabolic disorders, genetic disorders, structural abnormalities, neurological disorders, mum and feeding technique

359
Q

Presentation of pyloric stenosis

A

Baby starts bringing up small amounts of milk after feeding, which gets worse over a few days
Then projectile yellow vomit (milk curdles in stomach acid)
Reduced faeces
If untreated: dehydration and not gaining weight

360
Q

When does pyloric stenosis present

A

About 6 weeks after birth

361
Q

You do a blood gas on a neonate with pyloric stenosis. What would it show?

A

Metabolic Alkalosis - low K+ and Cl-.

The baby has vomited up all the HCl and the kidneys go into overdrive - increased K+ secretion.

362
Q

How to manage pyloric stenosis

A
  • IV fluids.
  • Repeat gases to monitor alkalosis.
  • Stop feeding to stop vomiting.
  • Pyloromyotomy once stable
363
Q

You suspect pyloric stenosis in a neonate. What investigations might you do?

A
  1. U+E.
  2. Blood gas.
  3. USS - hypertrophy of pyloric sphincter
364
Q

Potential presentation of IBS

A

Bloating, cramping, chronic or intermittent diarrhoea/constipation, urgency with defecation, incomplete sensation of defecation, passage of mucus in stool
May have dizziness, nausea, loss of appetite

365
Q

How to manage IBS

A

Diet and lifestyle management eg small meals, more often, low fat and high carbs

366
Q

How common is gastroenteritis?

A

Very!
At least one episode per year for most children

367
Q

Common causes of gastroenteritis

A

Rotavirus is most common infantile (NB rotarix oral vaccine at 8 and 12 weeks)
Norovirus commonest acorss all age groups
Campylobacter is most common bacterial cause
Also E.coli and adenovirus

368
Q

Presentation of gastroenteritis

A

Sudden onset diarrhoea +/- vomiting, abdo pain, mild fever

(bloody diarrhoea if campylobacter- generally due to undercooked meat and unpasteurised milk)

369
Q

Management of gastroenteritis

A

Self limiting
Send a stool sample if ?septicaemia/blood or mucus in stool/immunocompromised
Encourage fluids (not carbonated drinks)
IV or oral therapy if dehydrated
Give full strength milk asap
Leave it 48 hours before returning to school

370
Q

How long do sxs of gastroenteritis last?

A

Diarrhoea around 5-7 days
Vomiting around 1-2 days

371
Q

Potential complications of gastroenteritis?

A

Haemolytic uraemic syndrome, reactive complications eg Reiter’s syndrome, toxic megacolon, acquired/secondary lactose intolerance

372
Q

What counts as constipation in children?

A

Less than 3 complete stools/week
Hard/large stool
Rabbit droppings
Overflow soiling in children over 1

373
Q

Management of simple constipation in children?

A

Diet and lifestyle advice- high fibre, increase fluids
Then add Macrogol eg Movicol
Then add stimulant laxative eg Senna
Consider adding in lactulose

In secondary care can do manual evacuation, antegrade colonic enema, polyethylene glycol solutions

374
Q

Describe the pathophysiology of appendicitis

A 
Luminal obstruction (faecolith or lymphoid hyperplasia, impacted stool) 
Decreased venous drainage and localised inflammation by commensal bacteria causes and increased pressure. This causes ischaemia which can lead to necrosis and perforation
375
Q

Presentation of appendicitis

A

Dull peri-umbilical pain transfers to a sharp right iliac fossa pain
May have vomiting, anorexia, nausea, diarrhoea or constipation

MAGNET: migration of pain to RIF, anorexia, guarding, nausea, elevated temp, tenderness in RIF

376
Q

What can be found on examination of appendicitis

A 
Rebound tenderness and percussion pain over McBurneys point and guarding 
Psoas sign (pain when patient lies on left side while right thigh is flexed backward) 
Rovsing's sign (pain in RLQ upon palpation of LLQ)
377
Q

Management of acute appendicitis

A

Antibiotics and laparoscopic appendicectomy

378
Q

Why are children at higher risk of appendix perforation?

A

Tend to have delayed presentation

379
Q

Two types of childhood hernia?

A

Umbilical and epigastric

380
Q

How to manage hernias in children?

A

Umbilical- surgery if still present after 3 years
Epigastric- surgery only if uncomfortable or a nuisance

381
Q

Describe how Crohn’s disease affects the body (microbiology and location)

A

Remitting and relapsing disease, affects anywhere between the mouth and the anus
Transmural inflammation, deep ulcers and fissures with cobblestone mucosa, skip lesions
Non-caseating granulomatous inflammation

382
Q

Presentation of Crohns disease

A

Abdominal pain and bloody/mucus containing diarrhoea
Oral aphthous ulcers, perianal disease (tags, fistulae)
Extra-intestinal features=MSK, eyes, skin, renal, hepatobiliary

383
Q

Investigations for IBD?

A

Faecal calprotectin, routine bloods, stool microscopy and culture, colonoscopy with biopsy (=definitive diagnosis and can distinguish between UC and CD)

384
Q

How to manage Crohn’s disease? Acute attack

A

In acute attack: avoid anti-motility drugs eg loperamide as it can cause toxic megacolon
In acute attack: fluid resus, nutritional support, prophylactic heparin, corticosteroid and immunosuppressant eg mesalazine or azathioprine

385
Q

How to manage Crohn’s disease? Maintenance

A

Azathioprine, smoking cessation
Surgery can be used- ileocaecal resection, stricturoplasty, small/large bowel resections

386
Q

Course and pathophysiology of ulcerative colitis?

A

Relapsing/remitting
Diffuse continual mucosal inflammation of large bowel only. Begins in the rectum and spreads proximally.
Mucosal and submucosal inflammation only, crypt abscesses and goblet cell hypoplasia

387
Q

What is a protective factor against UC?

A

Smoking!

388
Q

Presentation of UC?

A

Bloody diarrhoea, PR bleeding and mucus discharge, increased faecal frequency, urgency and tenesmus

Extra-intestinal: MSK, skin, eyes, hepatobiliary

389
Q

How to induce remission in UC?

A

Corticosteroid and immunosuppressant eg mesalazine or azathioprine

390
Q

Maintenance management of UC?

A

Immunomodulators- mesalazine or sulfasalazine
Next line=infliximab
Surgery= ileostomy and complete resection= curative, but many have sub-total colectomy to begin with

391
Q

Pathophysiology of Coeliac disease

A

T cell mediated autoimmune response.
Response to gliadin and genetic (HLA-DQ2/DQ8)
Anti-gluten CD4 t cell response, with anti-gluten antibodies, anti-tissue transglutaminase, endomysin antibodies and intraepithelial lymphocytes activated

Causes epithelial cell destruction and villous atrophy

392
Q

which HLA molecules is Coeliac Disease often associated with?

A

DQ2 and DQ8.

393
Q

What are the forms of coeliac disease?

A

Classical, atypical, latent, silent, potential

394
Q

What are potential extra-intestinal features of coeliac disease?

A

Dermatitis herpetiformis, osteoporosis, anemia, short, delayed puberty, arthritis

395
Q

How to investigate for coeliac disease?

A

Serology
Must have had gluten in diet for at least 6 weeks prior to testing
IgA and IgAtTG, IgAEMA
Duodenal biopsy

396
Q

Common causes of failure to thrive

A

Down syndrome, cerebral palsy, heart disease, infections, milk allergy, CF, coeliac disease, GORD

397
Q

What is marasmus

A

Protein and energy malnutrition.
Loss of fat mass especially buttocks
Chronic diarrhoea and emaciated body type
There is NO oedema

398
Q

What is marasmus usually a sign of?

A

Poor socioeconomic status, child abuse+neglect, lack of food resources

399
Q

How to manage marasmus?

A

Similarly to kwashiorkor and reintroduce food slowly and correct any electrolyte imbalances

400
Q

What is kwashiorkor

A

Inadequate protein with a reasonable calorie intake

401
Q

Presentation of kwashiorkor

A

Fatigue, irritability, lethargy, failure to thrive, loss of muscle mass, generalised oedema, pot belly, fatty liver, prone to infections, hair and skin changes (erythematous, exfoliation, pigment, dry hair, thin nails)

402
Q

What is seen in bloods for kwashiorkor?

A

low glucose, low protein, high cortisol and GH, low salt, iron deficiency anaemia, metabolic acidosis

403
Q

Management of kwashiorkor?

A

Correct fluids/electrolytes- needs to be done in about 48 hours
Reintroduce foods slowly afterwards using RUTF (ready to use therapeutic foods eg peanut butter, milk powder, sugar, vegetable oil, minerals and vitamins)
Treatment takes around 2-6 weeks

404
Q

Pathophysiology of Hirschprung’s disease

A 
Congenital aganglionic megacolon disease 
Aganglionic segment (short/long/total) remains in tonic state so there is a failure in peristalsis 
Functional obstruction due to accumulated faeces
405
Q

Presentation of Hirschprung’s disease

A

Abdominal distension, bilious vomiting, failure to pass meconium within 48 hours after birth
Males 4 times more likely

406
Q

What is a big complication of Hirschprung’s disease

A

Hirschprung’s enterocolitis: stasis allows bacterial proliferation, which can cause sepsis and death

407
Q

How to investigate for Hirschprungs

A

Initially a plain X ray
Gold standard=rectal suction biopsy

408
Q

Management of Hirschprungs

A

IV antibiotics, NG tube, bowel decompression initially

Definitive= surgery to resect aganglionic section and connect unaffected bowel to dentate line

409
Q

What is intussusception?
Who does it happen to?

A
  • Movement/telescoping of one part of bowel into another > obstruction, inflamm, bloody stools
  • Peak at 5-7 months, rare after 2 years, males twice as likely
410
Q

Presentation of intussusception

A
  1. Colicky pain.
  2. Vomiting.
  3. Abdominal mass.
  4. Redcurrant jelly stool.
411
Q

How to diagnose intussusception?

A

abdominal uss

412
Q

How to manage intussusception?

A
  1. Gas in anal canal to reduce intussusception.
  2. Analgesia e.g. morphine.
  3. IV fluids if shocked.
413
Q

What is meckel diverticulum?

A

Outpouching of lower part of small intestine- a remnant of umbilical cord

414
Q

Presentation of meckel diverticulum?

A

Most asx

  1. Severe rectal bleeding.
  2. Intussusception.
  3. Volvulus.
415
Q

What is malrotation with volvulus?

A

Occurs during embryonic development at about the 10th week
Bowel twists so blood supply is cut off

416
Q

Presentation of malrotation with volvulus?

A
  • Obstruction with bilious vomiting
  • Bouts of crying
  • pull legs into body
  • not passing faeces
417
Q

Give a potential consequence of malrotation.

A

SMA blood supply to the small intestine can be compromised -> infarction.

418
Q

How to manage malrotation with volvulus

A

Dx by Xray
Tx= operation- stop necrosis of bowel and may need bowel resection

419
Q

What is toddler diarrhoea

A

Chronic nonspecific diarrhoea, at least 3 loose watery stools/day
Some alternate with constipation
Self limiting at about age 5-6
NOT due to malabsorption or serious bowel problem

420
Q

What are the 4Fs for diet consideration in toddler diarrhoea

A

Fat, fluid, fruit juices and fibre

421
Q

What is colic

A

Self-limiting repeated episodes of excessive and inconsolable crying for at least 3hrs a day, at least 3 days a week for at least 1 week
Occurs in up to 4 months of age

422
Q

Presentation of colic

A

Often crying in late afternoon or evening, draws knees up and arches back

423
Q

Management of colic?

A

REASSURANCE
Soothing strategies, parental wellbeing aids
Discourage simeticone (infracol) or lactase drops, probiotics, herbal supplements and maternal diet modification
Exclude other possible causes of distress

424
Q

Describe the three types of biliary atresia

A

Type I= common bile duct atresia with patent proximal ducts
Type II= common hepatic duct atresia with cystic structures in porta hepatis
Type III= R and L hepatic duct atresia to level of porta hepatis (most common)

425
Q

What is biliary atresia?

A

Extrahepatic bile ducts obliterated by inflammation and subsequent fibrosis

426
Q

Presentation of biliary atresia?

A

Presentation shortly after birth
Persistent jaundice
Pale stools and dark urine in term infants with normal birth weights

427
Q

What investigations are required in biliary atresia and what results shown?

A

LFTs: increased GGT, TGs normal
USS
Liver histology from percutaneous biopsy

428
Q

Management of biliary atresia?

A

Surgery, liver transplant needed before 8 weeks
Prognosis without treatment is 18 months

429
Q

Complications of biliary atresia?

A

Ascending cholangitis, cirrhosis, portal hypertension, liver failure, hepatocellular carcinoma

430
Q

Pathophysiology of cow’s milk protein allergy

A

Immune-mediated allergic response to casein and whey
IgE mediated type 1 hypersensitivity or non-IgE(T cell activation)

431
Q

Presentation of cow’s milk protein allergy

A

Acute and rapid onset (less than 2 hrs after ingestion)

  • pruritus
  • erythema
  • Urticaria and lip swelling
  • acute colicky abdo pain
  • vomiting
  • diarrhoea
  • may have resp signs
432
Q

Management of cow’s milk protein allergy

A

Specialised formula feeds for at least 6 months/ 9-12 months old, then reevaluate

433
Q

A 6-month old breast fed infant developed widespread urticaria immediately after the first formula feed.

a) What investigation might you do?
b) What is the most likely diagnosis?

A

a) Skin-prick test for cow’s milk.
b) IgE mediated cow’s milk allergy.

434
Q

A 4-month-old infant, formula fed since birth, has loose stools and faltering growth. Skin prick test to cow’s milk is negative but elimination of cow’s milk results in resolution of symptoms which return on trial re-introduction. What is the most likely diagnosis?

A

Non-IgE mediated cow’s milk allergy.

435
Q

Choledochal cysts pathophysiology?

A

Usually in extrahepatic ducts (CBD and HDs), sometimes in intrahepatic ducts
Type I cysts are most common-lower end of CBD joins up with pancreatic duct: pancreatic juice and bile mix leading to a weakening of bile duct wall and ballooning- can lead to pancreatitis and cholangitis

436
Q

Management of choledochal cyst

A

USS to identify
Commonly incidental finding
Surgery for removal of cyst

437
Q

Presentation of choledochal cyst

A

May have jaundice, intermittent abdo pain, cholangitis, peritonitis, lump, pancreatitis

438
Q

Aetiology of neonatal hepatitis syndrome

A

in 20%- viral (cytomegalovirus, rubella, hep a/b/c)
80%- nonspecific virus

439
Q

Presentation of neonatal hepatitis syndrome

A

Usually at 1-2 months
Jaundice, not gaining wt/ht, hepatosplenomegaly

440
Q

Management of neonatal hepatitis syndrome

A

Ix: liver biopsy and bloods
Mx: none specific, may need vitamins, potentially a liver transplant

441
Q

Presentation of liver failure in children

A

Increased AST and ALT with prolonged clotting +/- jaundice +/- encephalopathy

442
Q

Causes of liver failure

A

Viruses eg HSV, EBV, CMV, Hep A/B/E
Inherited metabolic disorders eg Wilsons, mitochondrial
Toxins
Medication eg erythromycin
Autoimmune
Low blood flow eg HF
Chronic: hep C, autoimmune, haemochromatitis, alpha 1 antitrypsin deficiency, CF, biliary atresia, sclerosing cholangitis

443
Q

Give 3 signs of UTI in children.

A
  1. Fever.
  2. Miserable.
  3. Vomiting.
  4. Dysuria.
444
Q

What investigations might you do on a child who you suspect has a UTI?

A
  1. Urine dip.
  2. MCS on clean catch urine. A sample can also be obtained using an in-out catheter.
  3. USS KUB.
  4. DMSA - renal scarring.
  5. MCUG - reflux.
445
Q

What is the treatment for UTI in children?

A

IV cefuroxime.

Switch to PO trimethoprim if stable.

446
Q

How would you treat a UTI that has been caused by ESBL e.coli?

A

You would give meropenem.

ESBL bacteria are resistant to all penicillins and cephalosporins.

447
Q

Presentation of pyelonephritis

A

At least 38 degrees and bacteriuria
or
Less than 38 degrees and loin pain/tenderness and bacteriuria

448
Q

When would you expect children to be dry overnight

A

Age 5

449
Q

Factors contributing to nocturnal enuresis

A

Small bladder, inability to recognise full bladder, hormones, UTI, sleep apnoea, diabetes, chronic constipation, structural problem in urinary tract or nervous system

450
Q

Management of nocturnal enuresis

A

<5= reassurance and advice
Non drug treatment: fluid intake, diet, toilet behaviour, rewards system then enuresis alarm if not responding (still more than 1-2 bed wets in a week)
Drug= desmopressin if over 5 +/- enuresis alarm
Imipramine hydrochloride is last line

451
Q

Causes of acute renal failure

A

Hypoperfusion (blood loss, surgery, shock)
Blockage of urinary tract
Nephrotoxic drugs
Haemolytic uraemic syndrome (e.coli usually)
Glomerulonephritis

452
Q

Symptoms of acute renal failure

A

Haemorrhage, fever, rash, bloody diarrhoea, vomiting, anuria or polyuria, pallor, oedema, eye inflammation, stomach mass

453
Q

Treatment of acute renal failure?

A

Treat underlying cause
U+Es
Dietary changes
Antihypertensives
Iv fluids
Diuretics

454
Q

How long for it to be classed as chronic renal failure?
Potential causes?

A

Over 3 months

Causes: long term blockage, alport syndrome, nephrotic syndrome, PKD, cystinosis, chronic conditions eg diabetes, untreated acute kidney disease

455
Q

What is alport syndrome

A

Inherited condition- X-linked most common, but can be autosomal recessive/dominant
Deafness, renal damage and eye defects

456
Q

Treatment of chronic renal failure

A

Diuretics
Dialysis and transplant
Diet changes (limit protein, potassium, phosphorus, sodium)
Growth promoters/bisphosphonates/iron tablets

457
Q

What defines nephrotic syndrome

A

Generalised oedema, heavy proteinuria (>200mg/mmol) and hypoalbuminaemia (<25g/L)
(Flattened podocytes allow protein leakage)
(Normal protein less than 20mg/mmol, normal albumin 35-45g/L)

458
Q

Causes of nephrotic syndrome
Which is most common?

A

Minimal change disease=most common
Others= congenital nephrotic syndromes, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis

459
Q

Management of nephrotic syndrome

A

High dose steroids- most respond well but some will have relapse (=steroid-resistant nephrotic syndrome)- in which case may need maintenance low dose steroids or immunomodulatory drugs eg cyclophosphamide
Low salt diet, prophylactic abx (as they leak Igs and steroids!), immunisations

Prednisolone for first episode 60mg/m2 for 4-6 weeks then wean down

460
Q

What is minimal change disease

A

Microscopically damage not evident. Idiopathic
Develop nephrotic syndrome more quickly but much more responsive to treatment

461
Q

What is congenital nephrotic syndrome
What is needed in management

A

Genetic condition (parents need 1 faulty and 1 healthy gene each to pass on)
Requires frequent albumin infusions to aid growth and development
Typically leads to renal failure

462
Q

Causes of nephritic syndrome

A

Post-streptococcal glomerulonephritis, henoch schnolein purpura, alport syndrome, sle/lupus nephritis

463
Q

Describe post-strep glomerulonephritis

A

10-14 days after skin/throat infection with group A strep
Haematuria, oedema, decreased urine output, htn, proteinuria, tired
Normally self limiting so needs supportive tx only
If strep infection persisting= penicillin

464
Q

What is henoch schnolein purpura

A

IgA vasculitis
Rash of raised red or purple spots usually on legs and bottom (extensor surfaces)

465
Q

Px of henoch schonlein purpura

A

Rash, arthritis, abdo pain, kidney impairment (self limiting and mild usually), may get PR bleeding and intussusception
Usually all preceded by 2-3 weeks of fever, headache, arthralgia/myalgia, abdo pain

466
Q

Mx of Alport syndrome

A

ACEi/ARB, diuretics, limit sodium
Monitor for potential renal failure

467
Q

Mx of Lupus nephritis

A

Immunosuppressants eg cyclophosphamide or hydroxychloroquine, antihypertensives, may need steroids

468
Q

Types of hypospadias
When does it form

A

Subcoronal (near head of penis)
Midshaft
Penoscrotal (where penis and scrotum meet)

Weeks 8-14 of pregnancy

469
Q

Management of hypospadias

A

Surgery between 3 and 18 months, may need to be done in stages

470
Q

Types of renal malformation?

A

Autosomal recessive polycystic kidney disease
Duplication (single renal unit with more than one collecting system)
Fusion (kidneys joined, but ureters enter bladder on each side)- most common= horseshoe
Malrotation (little clinical significance)
Multicystic dysplastic kidney (nonfunctioning renal unit but contralateral kidney normally fine)
Renal agenesis (bilat=fatal, unilateral)
Renal ectopia=kidney fails to ascend from true pelvis
Renal hypoplasia (underdeveloped)

471
Q

Pathophysiology of vescioureteral reflux

A

Urine flows from bladder back into ureters, potentially causing infection
Shorter than normal attachment between ureter and bladder and incompetent valve

472
Q

Describe the different grades of vesicoureteral reflux

A

I=into ureter
II=into ureter and renal pelvis without distension
III=into ureter and renal pelvis with mild swelling
IV= mod swelling
V=severe swelling and twisting or ureter

473
Q

Management of vesicoureteral reflux

A

Can go away by itself (junction gets longer with age)
Encourage frequent voiding
May need surgery to stop reflux

474
Q

Triad of haemolytic uraemic syndrome

A

Coombs test negative, thrombocytopenia, AKI
Typically a/w e.coli

475
Q

What is haemolytic uraemic syndrome

A

Toxins bind from e.coli causing thrombin and fibrin to be deposited in microvasculature
RBCs damaged while passing through occluded small vessels causing haemolysis

476
Q

Px haemolytic uraemic syndrome

A

Profuse diarrhoea turning bloody 1-3 days later, fever, abdo pain and vomiting

477
Q

Mx of HUS

A

Supportive- fluid and electrolyte mx, dialysis PRN, antihypertensive, keep circulating volume up

478
Q

General management of eczema?

A

Avoid triggers, keep nails short, emollients
Rest of treatment depends on severity: may include corticosteroids, abx, antihistamines, calcineurin inhibitors, phototherapy, immunomodulators or biologic medications

479
Q

What steroid would you give for mild eczema?

A

Hydrocortisone 1%

480
Q

What steroid for moderate eczema?

A

Betamethasone valerate 0.025% or clobetasone butyrate 0.05%

481
Q

What steroid for severe eczema?

A

Betamethasone valerate 0.1%
May need maintenance steroids or topical calcineurin inhibitors eg tacrolimus

482
Q

What is Stevens-Johnson syndrome?

A

Toxic epidermal necrolysis. Rare, acute and potentially fatal skin reaction with sheet-like skin and mucosal loss
Nearly always caused by medications

483
Q

What medications commonly cause Stevens-Johnson syndrome

A

Cotrimoxazole, penicillin, cephalosporins, anti-convulsants, allopurinol, paracetamol, NSAIDs

Usually within the first week of abx therapy

484
Q

Presentation of Stevens-Johnson syndrome

A

Initially a flu-like illness
Then, tender/painful red skin rash on trunk. extending to face and limbs over a course of hours-days

(Skin lesions: macules/diffuse erythema/targetoid/blisters)

The blisters merge to form sheets of skin detachment
At least 2 mucosal surfaces affected eg conjunctivitis/mouth ulcers/cough/diarrhoea

Acute phase lasts 8-12 days

485
Q

Management of Stevens Johnson syndrome

A

Skin biopsy shows keratinocyte necrosis
SCORTEN illness severity score to predict mortality
Cessation of suspected causative drug, admission, analgesia, temp maintenance, nutrition and fluids
General care of affected mucosal surfaces

486
Q

Classification of allergic rhinitis?

A

Seasonal (if due to grass and tree pollens=hay fever)
Perennial (throughout year, due to dust mites and animal dander)
Intermittent (less than 4 days/week or less than 4 consecutive weeks)
Persistent (more than 4 days/week or more than 4 consecutive weeks)
Occupational

487
Q

Management allergic rhinitis? Mild-mod

A

Nasal irrigation with saline
Allergen avoidance
PRN antihistamine- intranasal as first line eg azelastine
Can use 2nd generation non sedating oral eg loratidine or cetirizine for mild-mod symptoms

488
Q

What can be used in mod-severe allergic rhinitis?

A

Intranasal corticosteroid during periods of allergen exposure eg mometasone fluroate or fluticasone propionate

489
Q

For hayfever, what pollens affect you when?

A

Tree=early-late spring
Grass=late spring-early summer
Weed=early spring-late autumn

490
Q

What causes urticaria?

A

Histamine release
Due to: allergens, bee and wasp stings, autoimmune/idiopathic (chronic), physical triggers eg cold/friction, medications

491
Q

Types of antihistamine

A

Non sedating, sedating, short and long acting=H1 antihistamine
H2 antihistamines

492
Q

What is angio-oedema?

A

Swelling deep to the skin, commonly soft areas of skin eg eyelids and lips, more painful than weals/hives and takes longer to clear.

493
Q

Presentation of anaphylaxis

A

Skin features-urticaria, erythema, flushing, angio-oedema
Involves at least 1 of Resp/CV/GI system

494
Q

How to manage anaphylaxis

A

ABCDE, high flow oxygen, IM adrenaline, repeat 3-5 mins if required, treat a drop in BP with fluid bolus, consider salbutamol if wheeze, antihistamines for itch(cetirizine)
Prescribe an epipen

495
Q

What are the most common vascular birth marks?

A

Macular stains or salmon patches: Angel’s kisses (forehead/nose/upper lip/eyelids- disappear with age) or Stork Bites (back of neck, disappear with age)

Hemangioma (grow rapidly 6-9 months then shrink and lose red colour)

Port-wine stain/nervus flammeus (grows with the child, often face/arms/legs, flat pink/red/purple mark)

496
Q

What are the most common pigmented birth marks?

A

Moles/congenital naevi, cafe au lait spots, mongolian spots (blue-grey spots on lower back or buttocks, common on darker skin)

497
Q

Causes of infectious rash in children

A

Meningococcal, steven-johnsons syndrome, impetigo, kawasaki, staphylococcal scalded skin syndrome, eczema herpeticum, erythema nodosum, erythema multiforme, measles, glandular fever, hand foot and mouth disease, erythema infectiosum, chicken pox, nappy rash, scabies, tinea corporis, tinea capitis, mollusucm contagiosum

498
Q

What are the three phases in Kawasaki disease?

A

Acute phase=weeks 1-2
Subacute phase= weeks 2-4
Convalescent phase=weeks 4-6

499
Q

Pathophysiology of kawasaki disease?

A

Idiopathic. Not contagious.
systemic vasculitis
Mainly affects under 5s.

500
Q

What are NICE criteria for diagnosis of kawasaki?

A

Over 38 degrees for at least 5 days and at least 4 key symptoms:
Bilateral conjunctival infection, mouth/throat changes, hand and feet changes, rash, swollen glands

501
Q

How to manage kawasaki disease?

A

IV immunoglobulin and high dose PO aspirin (to prevent thrombosis)

502
Q

What should you be aware of when giving children aspirin?

A

Reyes syndrome: persistent vomiting, lethargy, liver and brain damage

503
Q

Presentation of measles

A

Initially coryzal sxs, fever, small grey-white spots in mouth (Koplik spots) lasting a few days
2-4 days later= maculopapular rash on head/neck and spreads to rest of body

504
Q

Management of measles

A

Self limiting viral illness and usually clears in about 7-10 days
Mx: stay at home for at least 4 days from when rash first appears, antipyretic, fluids, gently clear crust from eyes, hot steam

PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months

505
Q

What are some complications of measles?

A

dehydration, otitis media, conjunctivitis, laryngitis, airway infections, febrile seizures

506
Q

What causes chicken pox?

A

Varicella zoster virus

507
Q

Presentation of chicken pox?

A

Fever, aches and headache for 1 day preceding

rash: small itchy blisters that scab over

508
Q

Management of chickenpox?

A 
AVOID aspirin (reye's syndrome) 
Antipyretic, emollient, sedating antihistamine at night, keep nails short, dress comfortably, fluids 
Stay off school until all blisters crusted over

If an at risk group eg under 1 month, immunocompromised, severe heart/lung disease=aciclovir or vaccination

509
Q

When are you infectious with chickenpox?

A

7-21 day incubation
Infectious from 2 days before spots appear until all blisters crusted over

510
Q

What type of infection is rubella? When is peak incidence?

A

Viral. Mainly in spring and early summer

511
Q

Presentation of rubella?

A

Prodromal phase: low grade fever, headache, mild conjunctivitis, anorexia, rhinorrhoea
Rash occurs 14-17 days after exposure: pink discrete macules that coalesce behind ears and on face, spreads to trunk then extremities
Lymphadenopathy-cervical, suboccipital, post auricular

512
Q

Management of rubella?

A

PCR is gold standard diagnosis
Keep out of school for 4 days after rash appears, antipyretics, ask re contact with pregnant women

PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months

513
Q

What causes diphtheria?

A

Gram positive aerobic bacillus Corynebacterium diphtheriae causing acute upper resp tract infection

514
Q

Presentation of diphtheria?

A

Early sxs=coryzal
Nasal discharge- watery then purulent and bloody

Membranous pharyngitis with fever, enlarged cervical LNs, oedema of soft tissues= bull neck appearance

Cutaneous infection: pustules rupture causing punched out ulcers often on lower legs, feet and hands, with surrounding oedematous pink/purple skin. Takes 2-3 months to heal leaving a depressed scar

May cause difficulty swallowing, paralysis and HF

515
Q

Management of diphtheria?

A

PREVENTION: 6-in-1 vaccination at 2, 3 and 4 months

Antibiotics (eyrtho/azithro/clarithro/penicillin), reinforcing vaccine dose, manage contacts with abx prophylaxis

Booster vaccinations: first between 3.5 and 5 years, second between 13-18

516
Q

Pathophysiology of scalded skin syndrome?

A

Staphylococcal disease. Also known as Ritter disease and Lyell disease
Affects under 5s, especially neonates normally

Exotoxins from S. aureus bind to desmosomes, causing skin cells to become unstuck- causes red blistering skin

517
Q

Presentation of scalded skin syndrome?

A

Fever irritability and widespread erythema, then 24-48 hours later=fluid filled blisters form and rupture easily
Nikolsky sign positive= gentle strokes causes exfoliation

518
Q

Investigations for scalded skin syndrome?

A

Tzanck smear, skin biopsy, bacterial culture

519
Q

Management of scalded skin syndrome?

A

Admit to hospital
IV antibiotics eg flucloxacillin
Supportive -antipyretic, fluids, petroleum jelly

Healing usually complete within 5-7 days of starting treatment

520
Q

Pathophysiology of whooping cough?

A

Bordetella pertussis bacterial infection
Gram negative bacillus. Toxins paralyse cilia and promote inflammation

521
Q

Describe the first phase of whooping cough?

A

1st phase=catarrhal phase for 1-2 weeks
Coryzal sxs: rhinitis, conjunctivitis, irritability, sore throat, low grade fever, dry cough

522
Q

Describe the second phase of whooping cough?

A

2nd phase=paroxysmal for 2-8 weeks
Severe paroxysms of coughing followed by inspiratory gasp (whoop)

NB/ in infants under 3 months the whoop is less common and apnoea is more common. Paroxysms more common at night and often followed by vomiting

523
Q

Describe the third phase of whooping cough?

A

Convalescent phase=up to 3 months

524
Q

Investigations for whooping cough?

A

If cough for less than 2 weeks: nasopharyngeal aspiratr
If cough for more than 2 weeks: anti pertussis toxin IgG serology in under 5s or toxin detection in oral fluid for 5-17year olds
FBC=lymphocytosis

525
Q

Management of whooping cough?

A

Hospital if under 6 months and acutely unwell, sig breathing difficulties, feeding difficulties, complications eg pneumonia
Macrolide antibiotic if cough for less than 21 days= clarithromycin (under 1 month), azithromycin otherwise
Supportive

NB/ abx don’t alter course once disease established

PREVENTION= vaccination at 2,3,4 months and booster at 3 years 4 months

526
Q

Presentation of polio?

A

Fever, tiredness, headache, vomiting, neck stiffness and arm/leg pain
In 1 in 200 cases, affects NS leading to temporary or permanent paralysis

527
Q

Management of polio?

A

No treatment; need physio and OT

PREVENTION= vaccination at 2,3,4 months, 3years 4 months and 13-18 years

528
Q

How to diagnose TB in children?

A

Mantoux test= positive tuberculin skin test
IGRA
CXR
History of contact
Lab cultures very difficult to do in kids

529
Q

TB drugs?

A

RIPE
Rifampicin, isoniazid, pyrazinamide, ethambutol

530
Q

Who gets BCG vaccination?

A

If born in areas of UK where rates are high
If a parent/grandparent born in a country with high TB rates

531
Q

Causes of meningitis in children?

A

Bacterial: Mainly N. meningitidis and S. pneumoniae
In neonates= GBS (group B strep from mum’s vagina)

Viral: HSV, enterovirus and VZV

532
Q

What is the presentation of meningitis in neonates and babies?

A

Non-specific!
Eg Hypotonia, poor feeding, lethargy, hypothermia, bulging fontanelle

533
Q

How to investigate for meningitis?

A

Kernigs and brudzinski’s signs
LP if under 1 month and fever, 1-3 months fever and unwell, under 1 year with unexplained fever and other features of serious illness

534
Q

Management of meningitis?

A

Primary care: benzylpenicillin

Secondary care bacterial:
If under 3 months: cefotaxime and amoxicillin (to cover listeria)
Over 3 months=ceftriaxone
Dexamethasone QDS for 4/7 if LP suggestive bacterial meningitis
PEP for contacts: ciprofloxacin

Viral=aciclovir

535
Q

What CSF findings are suggestive of bacterial meningitis?

A

Cloudy, high protein, low glucose, high neutrophils, cultured for bacteria

536
Q

What CSF findings are suggestive of viral menigitis?

A

Clear, normal protein, normal glucose, lymphocytosis, negative culture

537
Q

Causes of encephalitis?

A

Most common=HSV 1(children) and HSV 2(neonates- from genital warts)

Others= VZV, CMV, EBV, enterovirus, adenovirus, influenza virus

538
Q

Presentation of encephalitis?

A

Altered consciousness, altered cognition, unusual behaviour, acute onset focal seizures and focal neurological symptoms, fever

539
Q

Management of suspected encephalitis?

A

Ix: LP (CSF and PCR) or CT if LP CI. MRI after LP, swabs, HIV testing
Mx: aciclovir for HSV/VZV or ganciclovir (CMV)
Repeat LP to ensure successful tx

540
Q

How does HSV1 affect children?

A

Infectious for 7-12 days
Commonly gingivostomatitis: fever, restless, dribbling, swollen gums which bleed easily, foul breath, ulcers on tongue/throat/palate/inside cheeks, enlarged LNs

541
Q

Management of HSV?

A

Can use antivirals eg aciclovir/valaciclovir but for mild/uncomplicated eruptions require no treatment

542
Q

What is slapped cheek syndrome caused by?

A

Also known as fifth disease/erythema infectiosum

Parvovirus B19

543
Q

When are the peak incidences for slapped cheek syndrome?

A

april and may

544
Q

Presentation of slapped cheek syndrome?

A

Commonly 3-15 years old
Rash=bright red scald-like on 1 or both cheeks, painless
May have mild coryzal symptoms
1 in 4 will be asx
By time rash develops, no longer infectious

545
Q

Management of slapped cheek syndrome?

A

None needed. Caution around pregnant women

546
Q

Types of impetigo and what caused by?

A

Non-bullous (about 70% cases)= S.aureus, S.pyogenes or both
Bullous= S.aureus

547
Q

Presentation of impetigo?

A

Non-bullous= golden/brown crusts from vesicles/pustules, commonly on face, limbs and flexures, may be mildly itchy
Bullous-more common in infants= blisters rupture leaving flat yellow/brown crust, may have systemic features

548
Q

Management of impetigo?

A

Good hygiene, stay away from school until healed or 48 hours after abx
Localised non-bullous=hydrogen peroxide 1% cream (2nd line=fusidic acid 2%)
Widespread non-bullous or bullous=oral flucloxacillin

549
Q

How to manage candida?

A

topical antifungal eg miconazole gel

If vaginal (v. uncommon before puberty)= fluconazole

550
Q

Causes of toxic shock syndrome?

A

Staph or strep- from female barrier contraceptives, tampons, injury on skin, childbirth, nasal packing, ongoing staph or strep infection

551
Q

Presentation of toxic shock syndrome?

A

Fever, flu-like sxs, N+V, diarrhoea, widespread sunburn like rash. lips/tongue/whites of eye turning bright red, dizziness and syncope, dyspnoea, confusion

552
Q

Management of toxic shock syndrome?

A

Antibiotics and fluids

553
Q

What causes scarlet fever?

A

Group A strep- affects people with recent strep throat or impetigo. Children over 3 at risk

554
Q

Symptoms of scarlet fever?

A

Sudden fever a/w sore throat, lymphadenopathy, headache, N+V, loss of appetite, swollen and red strawberry tongue, abdo pain, malaise

12-48 hours after fever=rash: starts below ears/neck/chest/armpit/groin)- scarlet blotches (boiled lobster appearance). Then looks like sunburn with goose pimples, pastia lines (red streaks from ruptured capillaries)

555
Q

Management of scarlet fever?

A

Penicillin
Mostly follows benign course

556
Q

What is Coxsackie’s disease?

A

Hand foot and mouth disease, generally affects under 10s.
Group A coxsackie virus- faecal-oral transmission

557
Q

Presentation of coxsackie’s disease?

A

Prodromal phase then tender oral ulcerative lesions and maculopapular lesions on hands and feet

558
Q

Management of coxsackie’s disease?

A

Supportive mx-fluids, soft diet, antipyretic analgesics, don’t need to isolate the child
Symptoms improve within 3-6 days with full resolution within 7-10 days

559
Q

Red flags for primary immunodeficiency in kids?

A

faltering growth, need IV abx for infections, FH, at least 4 infections a year, at least 2 sinus infections a year, at least 2 pneumonias in past 3 years, frequent deep tissue abscesses, persistent fungal infections (over 6 months), at least 2 deep seated infections over 3 years, at least 2 months on at least 2 abx with little effect

560
Q

Diagnosis of diabetes in children?

A

Random plasma glucose > 11.1 mmol/L if symptomatic

561
Q

Normal fasting blood glucose? post prandial glucose? ketones?

A

Fasting: 3.5-5.6mmol/L
Post prandial: <7.8
Ketones: <0.6

562
Q

DKA management?

A

Fluid, insulin, monitor glucose hourly, monitor electrolytes especially potassium and ketones 2-4 hourly, hourly neuro obs

563
Q

Fluids in DKA?

A

0.9% NaCl 20ml/kg bolus if shocked, 10ml/kg if not shocked
Maintenance and deficit fluids

5% fluid deficit in mild DKA
7% deficit in moderate
10% deficit in severe
Do no subtract boluses given for shock

564
Q

Initial management of type I diabetes mellitus in kids?

A

Aim for glucose 4-7: need to be able to check this
Give insulin (pump or injection): basal bolus regime most common
Carb counting
Advice for hypos
Lifestyle advice

565
Q

Carb counting in DMT1?

A

Insulin:carb ratio= 1 unit for every 15 grams of carb
Correction factor= 1 unit insulin brings blood glucose down by 8
Aim for glucose of 6

566
Q

Presentation of hypoglycaemia?

A

Autonomic fxs: irritable, hungry, nauseous, shaky, anxious, sweaty
Neuroglycopenic fxs: confused, drowsy, headache, coma, seizures

567
Q

Management of hypoglycaemia?

A

Check levels to confirm
Glucose tablets/gel/containing food or drink first
Check glucose in 15 mins
Follow up with longer acting carb (bread or biscuit)

Severe hypo (requiring additional assistance from another person)= IM glucagon

568
Q

Long term management of diabetes?

A

Aim for HbA1c less than 48mmol/mol
Monitoring at least annually: eyes, urine, feet, BP, injection sites, bloods

569
Q

Types of cryptorchidism?

A

True undescended= lies along normal path of descent but never present in scrotum
Ectopic testis= lies outside normal path and outside of scrotum
Ascending testis=previously in scrotum but higher now
Absent/atrophic testis=non palpable testis was in scrotum at birth but later disappears

570
Q

How to manage ?cryptorchidism?

A

6-8 week baby check exam
If not descended, re examine at 4-5 months to see if spontaneous descent
If not surgical treatment (orchodexy) before 12 months of age

571
Q

Pathophysiology of testicular torsion?

A

Testis twists around spermatic cord and cuts off the blood supply to the testicle

572
Q

Presentation of testicular torsion?

A

Sudden severe pain on one side of the scrotum, scrotal skin swells and turns red
Nausea and vomiting potential
May have just abdominal pain

573
Q

Management of testicular torsion?

A

Surgical emergency: blood supply must be restored within 6 hours or the testicle will infarct
If surgery delayed past 12 hours, 75% will need orchidectomy
Newborns with torsion- testes almost always infarcted so need orchidectomy

574
Q

What is a bell clapper deformity?

A

No tissue holding the testes to the scrotum so testes swing inside the scrotum= higher risk of torsion

575
Q

Cut offs for precocious puberty?

A

Girls under 8
Boys under 9

576
Q

2 types of precocious puberty?

A

Gonadotrophin dependent/ central precocious puberty= true precocious puberty with premature activation of the HPG axis

Gonadotrophin independent/precocious pseudopuberty= due to increased production of sex hormones

577
Q

Causes of central precocious puberty?

A

Mainly idiopathic or abnormalities of CNS

578
Q

Causes of precocious pseudopuberty?

A

CAH, tumours (HCG secreting), McCune- Albright syndrome, silver-russell syndrome, testotoxicosis, exogenous androgen exposure, severe hypothyroidism

579
Q

What can you give for true precocious puberty?

A

GnRH agonists

580
Q

Define delayed puberty

A 
Girls= absence of breast development by 13 or primary amenorrhoea with normal breast development by 15 
Boys= absence of testicular development by age 14
581
Q

Causes of delayed puberty

A

Most common= constitutional delay in growth and puberty (CDGP)

Central causes= CDGP, chronic illness, malnutrition, excessive exercise, psychosocial deprivation, steroids, hypothyroid, tumours, congential anomalies, irradiation treatment, trauma, idiopathic hypogonadotrophic hypogonadism

Peripheral causes= bilat testicular damage, syndromes eg Prader-Willi, Klinefelter, rugs. irradation, Turner syndrome, intersex disorders, PCOS

582
Q

How to manage delayed puberty?

A

Most are CDGP so need reassurance and monitoring
Can start course of sex hormones (low dose testosterone or gradual increase of oestrogen then cyclical progestogen)

583
Q

Causes of congenital hypothyroidism

A

Thyroid dysgenesis, thyroid hormone metabolism disorders, hypothalamic/pituitary dysfunction,

584
Q

What happens in congenital hypothyroidism isn’t treated?

A

cretinism: long term irreversible neurological problems and poor growth with intellectual disability

spasticity, gait problems, dysarthria, profound mental disability

585
Q

How to treat congenital hypothyroidism?

A

levothyroxine titrated to TFTs

586
Q

Define gonadotrophin deficiency/hypogonadotrophinism

A

Low serum concentrations of LH and FSH in presence of low circulating concentrations of sex steroids

587
Q

How to manage gonadotrophin deficiency?

A

Induce and maintain secondary sexual characteristics- testosterone and hCG or oestrogen and progestogen

588
Q

What is Kallmann syndrome?

A

Hypothalamic gonadotrophin releasing hormone deficiency (GnRH) and deficient olfactory sense (hyposmia or anosmia)

X linked recessive or autosomal recessive
Greater penetrance in males

589
Q

Presentation of Kallmann syndrome?

A 
Females= primary amenorrhoea 
Males= cryptorchidism, micropenis

Most are normal stature
Lots have associated stigmata- nerve deafness, colour blind, cleft palate, renal abnormalities

590
Q

How to manage Kallmann syndrome?

A

Exogenous sex steroids to induce and maintain secondary sexual characteristics

591
Q

What is congenital adrenal hyperplasia?

A

Group of autosomal recessive disorders of cortisol biosynthesis+/- aldosterone +androgen excess

21-hydroxylase deficiency

592
Q

What is the classic form of CAH?

A

Ambiguous external genitalia in girls
Boys have no signs at birth

593
Q

What is the salt-losing form of CAH?

A

Boys present at 7-14 days with vomiting weight loss, lethargy, hyponatraemia, hyperkalaemia and dehydration
Girls will be identified before a crisis due to genitalia

594
Q

How to manage classic CAH?

A

Glucocorticoid replacement= hydrocortisone
+/- Mineralcorticoids= fludrocortisone
Salt losing form need NaCl
Consider surgery for genitalia

595
Q

Types of androgen insensitivity syndrome? describe

A

Complete=testosterone has no effect so genitals are entirely female
Partial= testosterone has some effect so genitals not as expected eg penis underdeveloped, fully/partial cryptorchidism, some female genitalia.

596
Q

How will females with complete androgen insensitivity syndrome present? and partial?

A

Complete=Develop breasts but amenorrhoea, no pubic or axillary hair, no womb or ovaries

Partial=enlarged clitoris, undescended testicles, hypospadias, often raised as boys

597
Q

Causes of childhood obesity?

A

Diet, exercise lack, sleep deprivation (low leptin and high ghrelin), genetics, socio-economics, medication, endocrine eg PCOS/hypothyroid/Cushings/Prader-Willi

598
Q

Name 2 drugs that can be given as meningitis prophylaxis.

A
  1. Rifampicin.
  2. Ciprofloxacin.
599
Q

2-years-old, 2m history of malaise, pallor and reduced appetite. Occasional febrile episodes associated with a pink rash and soreness in her left thigh. Now reluctant to weight bear despite walking at 13 months. All immunisations are up to date and developmental history shows no concerns.
O/E: low grade fever and cervical lymphadenopathy.

ESR is significantly raised.

Give 3 differentials.

A
  1. JIA.
  2. ALL.
  3. Transient synovitis.
  4. Septic arthritis.
600
Q

3-year-old, 7-day history of high fevers. Now developed red eyes, a rash, sore mouth and throat. Miserable and unwell with a diffuse maculopapular rash on his torso. He has bilateral injected conjunctiva, red cracked lips and a strawberry tongue. 3x2cm cervical swelling and swollen reddened palms.

Give 3 differentials.

A
  1. Kawasaki disease.
  2. Scarlet fever.
  3. Shingles/chickenpox.
  4. Measles - ask about immunisation history.
601
Q

Commonest cancer in children?

A

Leukaemia- ALL and then AML

602
Q

Pathophysiology of acute lymphoblastic leukaemia?

A

Disruption of lymphoid precursor cells in the bone marrow leading to excessive production of immature blast cells: causes drop in functional RBCs/WBCs/platelets

603
Q

Presentation of leukaemia in children?

A

Anaemia (lethargy, pallor)
Thrombocytopenia (easy bruising/bleeding)
Leukopenia (fevers, infections)
May complain of bone pain
Non specific sxs (weight loss, malaise)

604
Q

Investigations for leukaemia?

A

FBC (pancytopenia, thrombocytopenia with significant lymphocytosis), serum biochemistry
Blood films (blast cells)
CXR (exclude mediastinal mass)
Bone marrow aspirate confirms diagnosis
LP

605
Q

Management of leukaemia in children?

A

Chemo, supportive care with blood products, prophylactic anti-fungals
Chemo in 5 phases (induction, consolidation, interim maintenance, delayed intensification, maintenance)
Maintenance= 2 years for girls and 3 years for boys

Stem cell transplants in high risk patients in 1st remission or relapsed patients

606
Q

Name 5 different types of primary brain tumour?

A

Astrocytoma, medulloblastoma, diffuse midline glioma, ependymoma, craniopharyngioma, embryonal tumours, pineoblastoma, brainstem glioma, choroid plexus carcinoma, germ cell tumour

607
Q

What is the most common brain and spinal cord tumour in children (apart from mets)?

A

Astrocytoma

608
Q

Presentation of brain tumour?

A

morning headaches, nausea/vomiting, diplopia, seizure, increased head circumference, bulging fontanelle, signs of raised intracranial pressure

609
Q

4 main types of astrocytoma?

A

Grade 1=pilocytic astrocytoma
Grade 2=diffuse (fibrillary) astrocytoma
Grade 3=anaplastic astrocytoma
Grade 4=glioblastoma multiforme

80% are low grade (1 or 2)

610
Q

What is the most common high grade brain tumour in children?

A

Medulloblastoma (2nd most common overall)
Most commonly found in cerebellum

611
Q

What is Wilm’s tumour? How presents?

A

Most common paediatric renal mass= nephroblastoma
Painless upper quadrant abdominal mass, haematuria in 20%, hypertension in 25%, ,may have acquired von willebrand disease

612
Q

how to manage wilm’s tumour?

A

Nephrectomy and chemo
Cure in about 90% cases

613
Q

How does neuroblastoma present?

A

Presents late- majority of symptoms due to mass effect or mets.

Systemic fxs, bruising, weakness/paralysis/BB dysfunction, bone pain, abdominal distension, htn, horner’s syndrome

Almost all are stage 3 or 4 at diagnosis

614
Q

Where does neuroblastoma arise from?

A

Adrenal or paraspinal sites most common
arises from developing sympathetic nervous system

615
Q

Investigating neuroblastoma?

A

FBC, esr, pt, catecholamine by products in urine, CT, MRI for paraspinal lesions, biopsy

616
Q

Presentation of retinoblastoma?

A

Leukocoria (abnormal appearance of pupil), visual deterioration, red and irritated eye, faltering growth, squint, absent red reflex

617
Q

Management of retinoblastoma?

A

Eye conserving treatments eg cryo/thermotherapy, photocoagulation, chemo
Enucleation for all unilateral tumours filling over half of the eye or invading eye structures and subsequent fitting of prosthesis
V sensitive to radiotherapy

618
Q

Which childhood cancer has the best overall survival?

A

Retinoblastoma (about 99%)

619
Q

What are the 2 main types of primary bone tumour in in children?

A

Osteosarcoma (more common) or ewing’s sarcoma (v rare)

620
Q

Presentation of hepatoblastoma?

A

Abdominal mass +/- poor appetite, weight loss, lethargy, fever, vomiting, jaundice

621
Q

Investigating hepatoblastoma?

A

Raised alpha fetoprotein

622
Q

What immunisations at 8 weeks?

A

6 in 1
rotavirus
men B

623
Q

What is contained in the 6 in 1 vaccination?

A

diphtheria, hepatitis b, Hib, polio, tetanus, whooping cough

624
Q

What immunisations at 12 weeks?

A

6 in 1, PCV (pneumococcal), rotavirus

625
Q

What immunisations at 16 weeks?

A

6 in 1, Men B

626
Q

What immunisations at 1 year?

A

Hib/Men C, MMR, PCV, MenB

627
Q

What immunisations at 3 years 4 months?

A

MMR, 4 in 1 pre school booster (diphtheria, tetanus, whooping cough, polio)

628
Q

What immunisations at 12-13 years?

A

HPV

629
Q

What immunisations at 14 years?

A

3 in 1 teenage booster (tetanus, diphtheria, polio), MenACWY

630
Q

What is klinefelter’s syndrome?

A

Extra X chromosome (commonly 47XXY)

631
Q

How does klinefelter’s syndrome present in kids?

A

Delayed speech or learning difficulties, rapid growth in mid-childhood or truncal obesity, hypogonadism

Small firm testes, decreased facial and pubic hair, loss of libido, impotence, gynaecomastia, history undescened testes

Infertility

632
Q

Diagnosis of klinefelter’s? Management?

A

Low/low-normal testosterone, elevated FSH and LH, chromosomal analysis

Testosterone replacement

633
Q

What is Turner’s syndrome?

A

Loss or abnormality of 2nd X chromosome in phenotypic girls
45X

634
Q

Different age presentations of Turners syndrome?

A

Newborns: borderline small, lymphoedema of hands/feet, excessive skin at nape of neck, cardiac abnormalities

Infants: close to third centile, feeding difficulties, poor weight gain, poor sleeping

Preschool: short stature, recurrent middle ear infections, behavioural difficulties, high activity levels

Adolescence: ovarian failure, impaired pubertal growth spurt, obesity, htn, higher prevalence immune disorders, foot problems, learning disabilities

Can have dysmorphic features eg webbed neck, short fingers, lymphoedema, high palate

Can have renal and heart problems

635
Q

how to diagnose turner’s syndrome?

A

Via amniocentesis
Chromosomal analysis
Measure gondatrophins, TFTs, renal and heart tests, bone age

636
Q

Management of turner’s syndrome?

A

MDT
Short stature- GH therapy
May supplement with oestrogen
Gonadal failure-combined oestrogen and progestogen

637
Q

Neonatal features of down’s syndrome?

A

Hyperflexibility, muscular hypotonia, transient myelodysplasia, bradycephaly, oblique palpebral fissures, epicanthic folds, ring or iris speckles (brushfield spots), low set/folded ears, stenotic meatus, flat nasal bridge, protruding tongue, high arched palate, loose skin on nape of neck, single palmar crease, short little finger, short broad hands, gap between hallux and second toes, congenital heart defects, duodenal atresia

638
Q

What is down’s syndrome? Biggest risk factor?

A

trisomy 21
maternal age

639
Q

Management of down’s syndrome?

A

Annual health checks-hearing, resp (OSA), eyes, heart, neurological, GI, hip, thyroid. Extra support in academia and help with living.

640
Q

What is edward’s syndrome?

A

Trisomy 18

641
Q

Presentation of edward’s syndrome?

A

More likely to be female
Low birth weight, craniofacial abnormalities (low set and malformed ears, micrognathia, prominent occiput, small facial features, microcephaly, cleft lip), skeletal abnormalities, congenital heart defects, GI abnormalities, GU abnormalities (horse shoe kidney), neurological problems, pulmonary hypoplasia

642
Q

Management of edward’s syndrome? What to take into account when making decisions?

A

Feeding difficulties common, 38.5% foetuses die during labour
Chance of survival to 3 months is 20% and to 1 year=8%

May decide to not give life sustaining treatment.

Otherwise manage cardiac failure and resp insufficiency

643
Q

What is patau syndrome?

A

Trisomy 13

644
Q

Presentation of patau syndrome?

A

Many never survive until term and are stillborn or spontaneously abort
Low birth weight, congenital heart defects, holoprosencephaly causing midline facial defects, brain and cns abnormalities, GI and GU malformations

645
Q

Life expectancy of patau syndrome?

A

median is 2.5 days. About 50% live over a week, 5-10% live over a year

646
Q

What is fragile x syndrome?

A

Most common cause of sex linked general learning disability. Delayed milestones and typical features eg high forehead, large testicles, facial asymmetry, large jaw and long ears.

647
Q

Potential diagnoses coming under fragile x syndrome?

A

ADHD, ASD, obsessive compulsive behaviours, emotional lability, aggressive behaviours, specific speech disorders eg echolalia and perseveration

648
Q

Management of fragile x patients?

A

MDT approach and manage each syptom
No cure
Most diagnosed by age 3 usually

649
Q

Name 4 types of muscular dystrophy?

A

duchenne muscular dystrophy ( most common and one of most severe) , myotonic dystrophy, facioscapulohumeral muscular dystrophy, becker muscular dystrophy, limb girdle muscular dystrophy, emery-dreifuss muscular dystrophy

650
Q

Male infant not walking by 18 months- concern? what test?

A

Concerned about duchenne muscular dystrophy
measure creatine kinase

651
Q

Presentation of ?muscular dystrophy?

A

Gower’s maneouvre (stands up by facing floor, wide feet, lift bottom first then hands walked up legs)
Waddling gait, lordosis/scoliosis, large calves and leg muscles compared to other muscles

652
Q

Management of muscular dystrophy?

A

Depends on type and severity eg mobility and breathing assistance
Steroids
Ataluren if has DMD, is over 5 and can still walk
Creatine supplements
Treat swallow and heart complications

653
Q

What features would make you suspect Angleman’s syndrome?

A

Developmental delay apparent by 6 months, but forward progression with no loss of skills once acquired
Sitting late at 12 months, walking late at 3-4 years, 10% can’t walk, flat and turned out feet, lean forward when run, speech impairment, laughter and smiling/happy demeanour, excitable personality. hand-flapping, short attention span, microcephaly, epilepsy, sleep disorders, excess chewing, drooling, fascination with water, hypopigmentation, prominent mandible

654
Q

What is the lifespan of angleman’s syndrome?

A

normal lifespan

655
Q

Presentation prader willi syndrome?

A

Hypotonia, developmental delay, obesity, learning disability, behavioural problems

656
Q

Genetics of angleman’s syndrome?

A

Maternal deletion of chromosome 15 or paternal uniparental disomy

657
Q

Genetics of prader willi syndrome?

A

Paternal deletion of chromosome 15 or maternal uniparental disomy

658
Q

Diagnostic criteria for prader willi?

A

Between birth and 3 years: 5 points including 4 major
3 years-adult=8 points including 5 major

Major (1 point); hypotonia with poor suck, feeding problems, excessive weight gain 1-6 years, characteristic facies, hypogonadism, cryptorchidism, global developmental delay, hyperphagia, chromosomal abnormality

Minor (0.5 points); infantile lethargy, behavioural problems, sleep disturbance, short and failed pubertal growth spurt, hypopigmentation, small hands and feet, narrow hands, eye abnormalities, thick saliva, speech articulation defects, skin picking

659
Q

Management of prader willi?

A

MDT
GH treatment to maintain normal growth
SSRIs trial
No role for appetite suppressants

660
Q

What is noonan’s syndrome (formerly leopard syndrome)?

A

Autosomal dominant condition characterised by: congenital heart disease, short stature, broad and webbed neck, sternal deformity, developmental delay, facial features, cryptorchidism, increased bleeding tendency

661
Q

What is william’s syndrome?

A

Rare autosomal dominant disease characterised by CV disease, distinctive facies, connective tissue abnormalties, intellectual disability, growth and endocrine abnormalities

Specific cognitive profile: good on verbal short term memory and language, but poor visuospatial construction

Overly friendly

Infantile hypercalcaemia

662
Q

Management of william’s syndrome?

A

MDT management- manage infantile hypercalcaemia with diet and corticosteroids, yearly surveillance, monitoring CV complications

663
Q

What is newborn respiratory distress syndrome?

A

Also known as hyaline membrane disease and surfactant deficiency lung disease

Usually affects premature babies who don’t have the necessary surfactant (enough produced by week 34 normally)

664
Q

Presentation of newborn resp distress syndrome?

A

Peripheral and central cyanosis, rapid and shallow breathing, flaring nostrils, grunting

665
Q

Management of newborn resp distress syndrome?

A

Treatment during prem labour= steroid injection before delivery and then a 2nd dose 24 hours later

Treatment after birth= extra oxygen if mild symptoms. More severe= ventilator, dose of artificial surfactant,IV fluids and nutrition

666
Q

What warrants mild chronic lung disease of prematurity (bronchopulmonary dysplasia)?

A

Breathing room air at 36 weeks for babies born before 32 weeks
or
air by 56 days of postnatal age for babies born after 32 weeks

667
Q

What warrants moderate chronic lung disease of prematurity?

A

Needing less than 30% oxygen at 36 weeks if born before 32 weeks
or
needing less than 30% oxygen at 56 days of postnatal age if born after 32 weeks

668
Q

What warrants severe chronic lung disease of prematurity?

A

Need more than 30% oxygen to breathe +/- CPAP at 36weeks/56 days postnatal

669
Q

What can be seen on CXR of a baby with chronic lung disease of prematurity?

A

Diffuse haziness, coarse interstitial pattern=atelectasis, inflammation, +/-pulmonary oedema

670
Q

Management of chronic lung disease of prematurity?

A

Minimise ventilation associated lung injury, continuous oxygen monitoring, nasal CPAP, maintain o2 between 91-95%, send them home on oxygen
Potential: Dexamethasone short term, diuretics, methylxanthines eg caffeine (increases resp drive and decreases apnoea)

671
Q

What is meconium?

A

Dark green faecal material produced during pregnancy, which is passed by the baby

672
Q

Why is meconium aspiration bad?

A

Vasoactive and cytokine substances activate inflammatory pathways and inhibit the effect of surfactant
Causes varying degrees of respiratory distress in the newborn
Potential consequences: airway obstruction, foetal hypoxia, pulmonary inflammation, infection (chemical pneumonia), surfactant inactivation, persistent pulmonary htn

673
Q

Management of meconium aspiration?

A

Depends on severity of resp distress eg obs, routine care, ventilation/oxygen, antibiotics, surfactant, inhaled nitric oxide if pulmonary htn, steroids

674
Q

Prognosis of meconium aspiration?

A

80% have 3-4 day illness then discharged home
Complications= air leak, cerebral palsy, chronic lung disease

675
Q

What is hypoxic ischaemic encephalopathy?

A

also known as intrapartum asphyxia

Brain injury caused by oxygen deprivation leading to death or severe impairment eg epilepsy, developmental delay, motor impairment, neurodevelopmental delay and cognitive impairment, cerebral palsy

Damage is permanent

676
Q

What events could lead to asphyxia?

A

Acute maternal hypotension, cardiac complications, injury from umbilical cord, impaired blood flow to brain during birth, intrapartum haemorrhage, placental abruption, labour and delivery stress, trauma etc

677
Q

What comes under TORCH infection?

A

Toxoplasmosis
Others= syphilis, parvovirus b19, vzv, enteroviruses, lymphocytic choriomeningitis virus, HIV, zika
Rubella
Cytomegalovirus
HSV

678
Q

What do torch infections generally do?

A

Infections during pregnancy that cause congenital defects
Generally greater risk of harm if mum infected in early pregnancy
Can cause miscarriage or spontaneous abortion

679
Q

Congenital toxoplasmosis issues?

A

Chorioretinitis with blindness, anaemia, hepatic and neurological symptoms

680
Q

What can the ‘others’ of Torch infection cause?

A

Syphilis= bowed sabre shins, hutchinson teeth, typical facial appearance
Parvovirus b19= anaemia, hydrops fetalis, myocarditis
VZV= chorioretinitis, cataracts, limb atrophy, cerebral cortical atrophy, neurodisability
Zika= microcephaly, positional/hearing/ocular abnormalities

681
Q

What can rubella do to the neonate?

A

Intrauterine growth restriction, intracranial calcifications, microcephaly, cataracts, cardiac defects, neuro disease
Blueberry muffin appearance
Most occur if exposure during first 16 weeks of pregnancy

682
Q

What can cytomegalovirus do to the neonate?

A

Growth restriction, sensorineural hearing loss, intracranial calcification, microcephaly, hydrocephalus, hepatosplenomegaly, optic atrophy, delayed psychomotor development
More severe in 1st trimester, but more common in 3rd trimester

683
Q

What drug can be used to stop the progression of hearing loss due to CMV?

A

Ganciclovir

684
Q

Describe physiological jaundice in the neonate?

A

Increased rbc breakdown and immature liver function
Present at 2-3 days of age
Jaundice begins to disappear towards end of first week and resolved by 10 days
Bilirubin under 200 micromol/L and baby remains well

685
Q

Causes of early neonatal jaundice aka onset in under 24 hours

A

Haemolytic disease (rhesus, ABO incompatibility, G6PD deficiency, spherocytosis)
Infection (toxoplasmosis/TORCH)
Haematoma
Maternal autoimmune haemolytic anaemia (SLE)
Gilbert’s syndrome
Dubin-Johnson syndrome
Crigler-Najjar syndrome

686
Q

Causes of prolonged neonatal jaundice aka lasting over 14 days (term) or over 21 days (preterm)

A

Infection, hypothyroid, hypopituitarism, galactosaemia, breast milk jaundice, GI eg biliary atresia

687
Q

How common is jaundice in the 1st week of life?

A

60% term and 80% preterm babies develop it
15% of healthy infants get physiological breastmilk jaundice

688
Q

What is kernicterus?

A

A complication of neonatal jaundice- bilirubin encephalopathy (unconjugated)
Occurs mainly in premature babies.

689
Q

Features of kernicterus?

A

Early features= jaundice, hypotonia, poor feeding, absent startle reflex
May then develop: high pitched cry, hypertonia of extensor muscles with arched back and hyperextended neck, bulging fontanelle, seizures

Potential complications= chronic bilirubin encephalopathy, extrapyramidal signs, visual problems, hearing problems, cognitive defects, dental enamel

690
Q

Management of kernicterus?

A

Manage neurological complications, phototherapy and exchange transfusions

691
Q

What is the most common GI emergency in neonates?

A

Necrotising enterocolitis

692
Q

What can be found on examination of a baby with necrotising enterocolitis?

A

Distension, visible intestinal loops, altered stools, bloody mucoid stool and bilious vomiting, decreased bowel sounds, erythema, associated systemic features

693
Q

Management of ?necrotising enterocolitis?

A

Confirm diagnosis by AXR and get baseline biochemistry
Nil by mouth, bowel decompression, Iv fluids and TPN (total parenteral nutrition), IV abx for 10-14 days (gentamicin and metronidazole), surgery if perforated/necrotic bowel suspected

NB/ stages i= suspected, iia=mild, iib=moderate, iiia=advanced, iiib=deteriorating

694
Q

What is gastroschisis?

A

Congenital defect of the abdominal wall where the abdominal contents herniate into the amniotic sack (usually the small intestine, but sometimes stomach, colon and ovaries). There is no covering membrane.
The opening is less than 5cm in length

695
Q

Management of gastroschisis?

A

Scheduled preterm delivery may improve the post op outcome.
Primary closure of the defect

696
Q

Simple v complex gastroschisis?

A 
Simple= intact, uncompromised and continuous bowel 
Complex= bowel perforation, atresia or necrosis
697
Q

Difference between gastroschisis and exomphalos?

A

Exomphalos has bowel contents in a sac, is more variable in size, and has a more central location

Gastroschisis has no covering membrane, is under 5 cm in length and tends to be to the right of umbilical cord insertion

698
Q

What is oesophageal atresia?

A

Congenital abnormality with a blind ending oesophagus in isolation or with at least one fistula(e) communicating between normal oesophagus and trachea (tracheo-oesophageal fistula0

699
Q

Associated anomalies with oesophageal atresia?

A 
VACTERL syndrome (vertebral, anorectal, cardiovascular, tracheo-oesophageal, oseophageal atreisa, renal abnormalities, limb) 
CHARGE association (coloboma, heart, atresia, retarded development, genital hypoplasia, ear) 
Chromosomal abnormalities eg trisomies
700
Q

Postnatal presentation of oesophageal atresia?

A

Respiratory distress, choking, feeding difficulties, frothing in first few hours after birth

701
Q

Management of oesophageal atresia?

A

Surgery

702
Q

Presentation of bowel atresia?

A

Persistent vomiting-often bilious and within hours of birth
on Xray= double bubble sign of duodenal atresia, colonic atresia has normal air levels

703
Q

Most common metabolic problem in neonates?

A

Gestational diabetes and hypoglycaemia

(glucose passes through placenta and elevates glucose levels in foetus, causing increased insulin secretion, this leads to hypoglycaemia)

Plasma glucose under 30 mg/dL

704
Q

Pathophysiology of neonatal hyperthyroidism?

A

Usually caused by maternal graves disease- antibodies cross the placenta and cause the foetus to develop hyperthyroidism

Usually temporary, but can be life threatening.

705
Q

Untreated congenital hyperthyroidism?

A

Risk of craniosynostosis (early skull closing), intellectual disability, growth failure, hyperactivity in later childhood

706
Q

Management of congenital hyperthyroidism?

A

Antithyroids and beta blockers- stopped when antibodies have disappeared (usually fully resolved by 6 months)

NB/ if mum took thyroid drugs during pregnancy, the child may not show symptoms until 3-7 days

707
Q

How many women carry group b strep in genitals?

A

1 in 4

708
Q

Presentation of neonate with GBS infection?

A

Sepsis, pneumonia, meningitis signs, BP changes, seizures

709
Q

Management of GBS infection?

A

IV antibiotics- penicillin G

710
Q

What can listeria do to a neonate?

A

Low birth weight, prematurity, circulatory and/or respiratory insufficiency, meningitis, sepsis

711
Q

Management of neonatal listeria infection?

A

Ampicillin and aminoglycoside eg gentamicin

712
Q

Cause of encephalitis in neonates?

A

HSV2 from vertical transmission

713
Q

Presentation of HSV encephalitis?

A

Non specific: decreased levels of consciousness, seizures, lethargy, fever

714
Q

Management of HSV encephalitis? Prognosis?

A 
IV aciclovir 
Highly lethal (around 50%) 
Complications= deaf, vision loss, cerebral palsy, seizure
715
Q

Management of cleft life/palate?

A

Surgery
Cleft lip at 3-6 months
Palate at 6-12 months

716
Q

What is ophthalmia neonatorum?

A

Conjunctivitis in newborn due to vertical transmission (contact with mum’s birth canal infected by STI)

Commonly chlamydia

Also: haemophilus, strep, staph, pseudomonas, HSV, adenovirus, enterovirus

717
Q

Chance of ophthalmia neonatorum in babies born to untreated chlamydia?

A

30-50%

718
Q

Presentation of ophthalmia neonatorum?

A

Red, discharging, swollen lids, usually bilateral
Lid oedema, conjunctival oedema, mucopurulent conjunctivitis, cornea can be involved

719
Q

Management of ophtalmia neonatorum?

A

Culture conjunctiva and PCR to establish causative organism

Bacterial= systemic penicillin G or cephalosporin for gonorrhoea 
Chlamydia= systemic erythromycin or topical azithromycin 
Herpetic= topical and systemic aciclovir
720
Q

Premature baby still needing breathing support by 36 weeks corrected age = what?

A

Chronic lung disease/ bronchopulmonary dysplasia
Caused by underdeveloped lungs

721
Q

Management of bronchopulmonary dysplasia?

A

Oxygen (can further damage lungs), breathing support (CPAP or mechanical ventilation), may need steroids

722
Q

Pathophysiology of retinopathy of premature baby?

A

Abnormal blood vessel growth are fragile and weak, causing bleeding and leakage
Usually bilateral
Ranges from abnormal blood vessel growth to full retinal detachment (stage i-v)
Can cause blindness

723
Q

Management of retinopathy of premature baby?

A

Laser therapy or cryotherapy: destroys peripheral retina and slows/reverses abnormal vessel growth, but loses some side vision

724
Q

Why are premature babies at risk of osteopenia?

A

May not receive the full amount of calcium and phosphorus needed to form strong bones
Haven’t been as active in the womb and thus have weak bones
Very premature babies lose more phosphorus in urine than term babies
Can be due to low vit D

725
Q

How to treat osteopenia of premature baby?

A

Supplements- calcium and phosphorus added to IV fluids or breast milk
Special formulas if no breast milk
Vit D supplements for babies with liver problems

726
Q

What is periventricular leukomalacia?

A

White matter surrounding ventricles deprived of blood and thus oxygen. This causes softening of the white matter

727
Q

Consequences of periventricular leukomalacia?

A

Most commonly cerebral palsy
May have visual problems and learning disability
Not progressive
Presentation depends on amount of brain tissue damaged

728
Q

What is apnoea in a prem baby?

A

Prem baby pauses breathing for over 15-20 seconds
or
pauses breathing for under 15 seconds but has bradycardia or low sats

Usually self limiting

729
Q

What is transient tachypnoea of the newborn?

A

Caused by leftover fluid in lungs making it harder for the alveoli to stay open
RR over 60
Most improve over 24-48 hours, and most cases self limit

730
Q

Who is more likely to have transient tachypnoea of the newborn?

A

Born before 38 weeks, c-section delivery, born to diabetic or asthmatic mother, twins, male

731
Q

Forms of child abuse

A

Physical, neglect, emotional, sexual

732
Q

What may come under child neglect?

A

Medical eg unimmunised, non attendance
Nutritional eg faltering growth, obesity
Emotional
Educational eg poor school attendance
Physical eg inadequate hygiene, persistent infestation, inappropriate clothing
Failure to supervise eg frequent A+E trips, injury suggesting lack of care such as sun burn

733
Q

Normal presentation/history of toddler’s fracture?

A

Subtle undisplaced spiral fracture of tibia
Usually pre school, sudden twist after an unwitnessed fall
Local tenderness over tibial shaft or on general strain on tibia

734
Q

What counts as mild dehydration and features?

A

Less than 5%
Thirst, dry lips, restlessness, irritability

735
Q

What counts as moderate dehydration and features?

A

5-10%
Sunken eyes, decrease skin turgor, decreased urine output

736
Q

What counts as severe dehydration and features?

A

Over 10%
Cold, mottled, hypotensive, anuria, decreased consciousness

737
Q

What fluids needed for not dehydrated/dehydrated/shocked?

A

Not dehydrated=maintenance
Dehydrated=maintenance+deficit
Shocked=bolus+maintenance+deficit

738
Q

What maintenance fluids do neonates need?

A

10% glucose with increasing rate every day
Monitor U+Es every day
From day 2 if needed= sodium 2-3mmol/kg/day and potassium 1-2mmol/kg/day

739
Q

How to estimate child’s weight?

A

(age + 4) x 2

740
Q

What fluids for maintenance in children? How much?

A

0.9% NaCl (normal saline)

First 10kg=100ml/kg
Next 10kg=50ml/kg
Every other kg=20ml/kg

Calculates how much in 24 hours

741
Q

How to calculate the deficit fluids in children?

A

Deficit x 10 x wt(kg)

Where estimated deficit=
mild= negligible, mod=5%, severe=10%

742
Q

What bolus do you use and which volume?

A

0.9% NaCl, volume=20ml/kg

May use 10ml/kg in some cases eg trauma, DKA
Tends to be 20ml/kg if shocked in DKA, 10ml/kg if not shocked

743
Q

What constitutes an atypical UTI in children and what do you need to do if this is the case?

A 
Atypical= septicaemia/IV abx needed, non e.coli, poor urine flow, abdo/bladder mass, increase creatinine, failure to respond to treatment within 48 hours 
Recurrent= at least 2 episodes with at least 1 with systemic features, or at least 3 episodes without systemic features

Need to investigate for a structural abnormality

744
Q

How to investigate ?structural abnormality in the case of UTI?

A
  1. USS renal tract (looks at size and drainage of kidneys and bladder)
  2. Micturating cystourethrogram/MCUG (looks for vesicoureteric reflux, looks at bladder and posterior urethral valve)
  3. DMSA scan/radionucleide imaging (looks at renal function and any renal scarring)
745
Q

Features of nephritic syndrome?

A

Haematuria, proteinuria, impaired GFR, salt and water retention (htn and oedema)

746
Q

Management of nephritic syndrome?

A

Fluid balance management (input/output, fluid restriction, diuretics, salt restriction)
Correct other imbalances (K, acidosis)
Dialysis may be needed (uncommon)
Penicillin for treatment of an ongoing strep infection

747
Q

Why is sinusitis uncommon in children?

A

Maxillary sinus exists at birth but only grows to full size after 2nd dentition
Ethmomid sinuses only 2-3 cells at birth
No/rudimental frontal sinuses at birth (develop by 7-8 years)

748
Q

Narrowest point in child’s airway vs adult’s?

A

Children=subglottis
Adults=vocal cords

749
Q

What is the most common airway abnormality in children and it’s presentation?

A

Laryngomalacia
Normal voice, stridor worse on feeding/exertion/supine, failure to thrive in 10%, increased work of breathing

750
Q

When do you expect primitive reflexes to disappear?

A

By 4-6 months, lots of babies lose them within 2 months

751
Q

What options for formula for children with cow’s milk protein allergy?

A

Hydrolysed eg althera
Amino acid based eg alfamino
Goat milk formula from birth
Soy formula from 6 months

752
Q

What constitutes faltering growth?

A

Failure to gain adequate weight or height/length during infancy or early childhood

Birth weight <9th centile with a fall across at least 1 wt centiles
Birth weight between 9-91st centile with a fall across at least 2 wt centiles
Birth weight over the 91st centile with a fall across at least 3 wt centiles
Current weight below 2nd centile whatever the birth weight

753
Q

When would you expect a baby to get back to his birth weight?

A

3 weeks

754
Q

Causes of osteoporosis in children?

A

Inherited/congenital eg OI, inborn errors of metabolism, haem problems, idiopathic
Acquired eg drug induced eg steroids, endocrinopathies, malabsorption

755
Q

What is osteogenesis imperfecta?

A

AD inherited condition, v rare
Mostly due to defects in type i collaegen
Characteristed by bone fragility, fractures, deformity, bone pain, poor growth, impaired mobility

756
Q

How can you manage osteogenesis imperfecta?

A

MDT management esp ortho team and metabolic bone specialist
Bisphophonates

757
Q

Describe the types of osteogenesis imperfecta?

A

type 1- most common, mild
2- lethal
3- progressively deforming, severe
4- moderate

758
Q

What may indicate a shaken baby?

A

Hypoxia, subdural haematoma, rib fractures, retinal haemorrhages, may have other fractures, torn frenulum

759
Q

How should a premature baby be fed?

A

IV fluids/parenteral nutrition as suckle and swallow only starts from 32-34 weeks
Start small volumes of expressed breast milk and build up to full feeds slowly (risk of NEC if too quick)

760
Q

Causes of acute scrotum in children?

A

Testicular torsion!!!
Also torsion of appendix testis (self limiting, but if any diagnostic doubt treat as testicular torsion)

761
Q

Vomits and presentation age in pyloric stenosis, malrotation and intussusception

A

Malrotation: bilious green vomit at 24 hours
Pyloric stenosis: milky vomit at 4 weeks
Intussusception: milky then green vomit at 6 months

Phase 3A 2021 (7 decks)

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