Paediatrics Flashcards

1
Q

Gross Motor Development - 6w, 6m, 9m, 12m and 18m milestones

A

6 weeks -
- Good head control (raises head to 45 degrees when prone on tummy)
- Head stabilised when raised to sitting position

6 months -
- Unsupported sitting w/ rounded back
- Rolls prone to back (vice versa later)

9 months -
- Stands holding on
- Straight back sitting (7 1/2 months)

12 months - Walks alone (9-18months); 18m is threshold for worry eg Duchenne’s MD, Hip problems, CP etc

18 months -
- Runs (16m)
- Jumps (18m)

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2
Q

Gross Motor Development - 2y, 2.5y, 3y and 4y

A

2 years -
- Tiptoe running
- Ball throw at shoulders
- Upstairs walking with both feet on each step

2.5 years - Kicks ball

3 years -
- Hops on 1 foot x3 (each)
- Upstairs walking 1 foot per step
- Downstairs 2 feet per step

4 years - Stair walking both ways in adult manner

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3
Q

Fine Motor/Vision Development - 6w, 6m, 9m, 12m and 18m milestones

A

6 weeks - Tracks object/face

6 months -
- Palmar grasp (5m)
- Transfers objects hand to hand

9 months -
- Inferior pincer grip (pads of fingers in pincer)
- Object permanence (understanding that objects continue to exist when they cannot be seen, heard, or touched - eg peek-a-boo)

12 months -
- 2 brick tower
- Neat pincer grip (10m)
- Casting bricks like fishing (should disappear by 18m otherwise abnormal)

18 months -
- Drawing to and fro
- 4 brick tower (should be smooth wooden blocks to force balance)

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4
Q

Fine Motor/Vision Development - 2y, 2.5y, 3y, 3.5y and 4y

A

2 years -
- Draw; vertical line
- Bricks; 8 block tower
- Turns several pages of book at a time
- Puzzles; shape matching >2y

2.5 years - Draw; horizontal line

3 years -
- Draw; Circle
- Bricks; Bridge or train
- Cuts; Single
- Griffiths Beads

3.5 years - Cuts pieces

4 years -
- Draw; Cross, Sqaure (4.5y), Triangle (5y)
- Bricks; 12 block tower, 6 brick steps, 10 brick steps (5y)
- Cuts; paper in half
- Beads; small beads

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5
Q

Speech/Language Development - 6w, 6m, 9m, 12m and 18m milestones

A

6 weeks - Stills/startles at loud noises

6 months -
- Turns head to loud sounds
- Understands bye-bye/no (7m)
- Monosyllabic babbles

9 months -
- Responds to own name
- Imitates adult sounds (polysyllabic)

12 months -
- Shows understanding of nouns eg where’s mummy
- 3 words (50% 13m)
- Points to own body parts (15m), points to body parts on doll (18m)

18 months -
- Shows understanding of nouns eg show me the x
- 1-6 different words

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6
Q

Speech/Language Development - 2y, 2.5y, 3y, 3.5y and 4y milestones

A

2 years -
- Shows understanding of verbs; eg what do you draw with, what do you eat with
- 2 words joined together (50+ word vocabulary)

2.5 years -
- Shows understanding of prepositions in/on (eg put the cat on the bowl)
- 3-4 words joined together

3 years -
- Understands negatives; which of these is NOT an animal?
- Understands adjectives; which of these is red?

3.5 years - Understands comparitives; which boy is bigger than this one (point to middle sized)?

4 years -
- Understands complex instructions; before you put x in y, give z to mummy
- Uses complex narrative/sequences to describe events

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7
Q

Social Development - 6w, 6m, 9m, 12m and 18m milestones

A

6 weeks - Social smile (visual problem if not)

6 months -
- Puts object to mouth (stops at 1y)
- Shakes rattle
- Reaches for bottle/breast

9 months -
- Stranger fear (6-9m to 2y)
- Holds and bites food; eg digestive on table

12 months -
- Waves bye-bye
- Hand clapping
- Plays alone if familiar person nearby
- Drinks from beaker with lid

18 months - Imitates every day activities

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8
Q

Social Development - 2.5y, 3y and 4y milestones

A

2.5 years - Eats skillfully with a spoon (no/little spillage)

3 years -
- Begins to share toys with friends
- Plays alone without parents
- Eats with fork + spoon
- Bowel control

4 years -
- Concern/sympathy for others if hurt
- Has best friend
- Bladder control (4.5y)
- Engages in imaginative play/observing rules; eg pretending to be an animal (4.5-5y)
- Eats skillfully with little help
- Handles knife (5y)
- Dressing and undressing

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9
Q

Paediatric Respiratory Conditions - Causative agents (Croup, Acute Epiglottitis, Bronchiolitis, Whooping Cough)

A

Croup - Parainfluenza virus

Acute Epiglottitis - Haemophilus influenzae type B

Bronchiolitis - RSV

Whooping Cough - Bordetella pertussis

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10
Q

Downs Syndrome - aetiology and percentage of cases with each (3)

A

Meiotic Chromosomal Non-Disjunction (Maternal Chromosome 21) - 95%

Robertsonian Translocation of Unbalanced Chromosomal Material (often t(14,21) translocation) - 4%

Mosaicism - 1%

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11
Q

Downs Syndrome - strongest risk factor

A

Increased maternal age at time of delivery
- 20 y/o = 1/1,530
- 45 y/o = 1/37

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12
Q

Downs Syndrome - dysmorphic features (General, head, neck, hands, feet)

A

General -
- Hypotonia
- Hyperflexibility

Head -
- Oblique palpabral fissures (edge of eyes)
- Epicanthic folds (upper eyelid skin fold covers the inner corner of the eye)
- Flat nasal bridge
- Brachycephaly (flat occiput)
- Dysplastic, low set ears
- Open mouth with protruding or furrowed tongue
- High arched palate
- Brushfield spots in eyes (small, often white spots arranged in a ring)

Neck - Short neck with excessive skin at nape

Hands -
- Transverse palmar crease (Simian)
- Short and incurved little finger

Feet - Sandal toe deformity

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13
Q

Downs syndrome - congenital heart defects; 3 most common

A

~ 40-50% of neonates with T21 will have congenital heart disease

  • AVSD; 37%
  • VSD; 31%
  • ASD; 15%
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14
Q

Downs Syndrome - neurological complications

A

Developmental delay + intellectual disability (varies significantly)

Autism, ADHD, aggressive behaviour more prevalent

Alzheimer’s - 75% develop by 6th decade of life

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15
Q

Downs Syndrome - cause of deafness

A

Eustachian tube abnormalities lead to glue ear -> conductive hearing loss (so need for regular audiometry)

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16
Q

Downs Syndrome - respiratory complications

A

Asthma and OSA

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17
Q

Downs Syndrome - GI tract abnormalities

A

12% require surgery

Most characteristic is duodenal atresia (failure of intestine to recanalize during development)

Others include:
- Imperforate anus
- Tracheo-oesophageal fistula
- Increased risk of Hirschsprung’s disease

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18
Q

Down’s Syndrome - endocrine complications

A

Hypothyroidism - 10-20%

T1DM

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19
Q

Down’s Syndrome - haematological complications

A

ALL
Immunodeficiency
Transient myeloproliferative disorder
Polycythaemia
Acute megakaryoblastic leukaemia (AMKL)

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20
Q

Down’s Syndrome - ophthalmological complications

A

Myopia, strabismus, cataracts

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21
Q

Down’s Syndrome - Screening; Combined Test (timing, components, results)

A

10-14 weeks

Serum Screening
- Beta-hCG; increased in T21
- PAPPA-A; decreased in T21

Nuchal Translucency Screening - increased nuchal translucency (thickness >6mm)
- Less reliable later in pregnancy

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22
Q

Down’s Syndrome Screening - comparison between triple and quadruple test

A

Less reliable than the combined test

Both performed at 14-20 weeks

Both maternal blood tests only

Triple - beta-hCG (raised) + AFP (low) + Serum uE3 (low)

Quadruple - beta-hCG (raised) + AFP (low) + Serum uE3 (low) + Inhibin A (raised)

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23
Q

Down’s Syndrome - what is a positive screening result (ratio score)

A

Risk >1/150 - then offered diagnostic testing

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24
Q

Down’s Syndrome - comparison of antenatal testing methods

A

Methods collect foetal cells to karyotype them for definitive answer

Chorionic Villus Sampling (9-12 weeks)
- US Guided biopsy of placental tissue
- Done when testing is done earlier in pregnancy
- A higher risk of pregnancy loss with chorionic villus

Amniocentesis (15-19 weeks)
- US Guided fluid aspiration of amniotic fluid using needle and syringe
- Later in pregnancy once enough amniotic fluid to make it safer to sample

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25
Q

Down’s Syndrome - what does NIPT test?

A

Cell-free DNA shed from placenta into maternal blood stream

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26
Q

Down’s Syndrome - post-natal investigations

A

Echocardiogram - congenital heart defects

Red reflex testing - congenital cataracts

TFTs - congenital thyroid disease

FBC - myeloproliferative disorders/polycythaemia

Hearing assessment - congenital hearing issues

Radiographic swallowing assessment - performed if feeding difficulties present to screen for GI abnormalities eg duodenal atresia

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27
Q

Down’s Syndrome - prognosis

A

60 y/o
- Varies depedning on severity of associated complications

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28
Q

Kawasaki Disease - definition

A

An acute, self-limiting, systemic, medium-vessel vasculitis of unknown cause that mainly affects children <5 y/o and commonly presents with a high fever (above 39ºC) lasting 5 or more days

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29
Q

Kawasaki Disease - risk factors

A

Asian ethnicity - especially Japanese

Aged <5 y/o - 80% of cases

Male - 1.5:1 (M:F)

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30
Q

Kawasaki Disease - clinical features

A

Consider in any child with persistent high fever (above 39ºC) lasting 5 or more days

CREAM:
- Conjunctivitis; bilateral, non-exudative
- Rash; any non-bullous rash, often maculopapular and desquamation of hands and feet occurs in sub-acute phase
- Edema/Erythema; of hands of feet
- Adenopathy; cervical, commonly unilateral and non-tender
- Mucosal involvement; strawberry tongue, oral fissures etc

AHA Diagnostic criteria is similar

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31
Q

Kawasaki Disease - complications

A

Most Common - Coronary Artery Aneurysms - 15-25% untreated children develop aneurysms but risk much lower with early treatment (giant aneurysms can rupture -> cardiac tamponade)

Coronary Artery Thrombosis/MI

Myocarditis/Pericarditis

Valvular Disease - eg MR

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32
Q

Kawasaki Disease - management

A

Hospital admission - early treatment to prevent coronary artery aneurysms

Oral aspirin (reduce thrombosis risk) -
- High dose until fever resolves for 48 hours
- Low dose for ~6 weeks after

IVIG (reduce CA aneurysm risk)- infusions within first 10 days can reduce incidence of coronary artery aneurysms (clinical improvement within 36 hours or 2nd dose may be given)
- Corticosteroids or infliximab (anti-TNF) if ineffective/resistant

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33
Q

Kawasaki Disease - natural history

A

Acute Phase (1-2 weeks from fever onset) -
* High fever
* Irritability
* Rash
* Lymphadenopathy
* Mucositis
* Peripheral erythema and oedema

Subacute Phase (2-4 weeks from fever onset)
* Afebrile
* Most clinical features begin to resolve, desquamation of the hands and feet
* Arthralgia
- The highest risk period for developing cardiac complications

Convalescent Phase (4-8 weeks from fever onset)
* Asymptomatic period
* Clinical features have resolved
- Coronary artery aneurysms often improve but may get worse

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34
Q

Kawasaki Disease - investigations

A

Bedside -
- ECG; may show arrhythmias or ST-T changes if myocarditis/MI occurs
- Urinalysis; may show sterile pyuria (raised WCC w/o infection)

Lab:
Baseline Bloods -
- FBC - may be anaemia, raised WCC, thrombocytosis
- U&Es
- LFTs - may be deranged if hepatitis occurs, hypoalbuminaemia can occur

ESR + CRP; Often significantly raised

Anti-streptolysin O Titre (ASOT); Exclude GAS infection

Consider PIMS-TS blood tests if suspected as DDx - Troponin
- D-dimer
- Coagulation w/ fibrinogen
- Ferritin

Imaging:
- Echocardiography essential
* Findings include:
○ Coronary artery aneurysms
○ Valvular disease (e.g. mitral regurgitation)
○ Coronary artery thrombosis
○ Poor ventricular function/evidence of myocarditis
- Pericardial effusion/evidence of pericarditis

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35
Q

Kawasaki Disease - complication of treatment

A

Aspirin use in <16 y/o associated w/ Reye’s Syndrome; rare acute encephalopathy associated with liver failure

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36
Q

Difference between CMPA, CMPI and Lactose Intolerance

A

CMPA - hypersensitivity to cow’s milk protein; allergic process (IgE mediated or non-IgE mediated)

CMPI - non-allergic process (no immune system response) to cow’s milk protein, just GI symptoms and no systemic allergic symtpoms like wheeze/sneezing etc

Lactose Intolerance - lactose is a sugar not a protein and no allergic response

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37
Q

CMPA - types and differences between

A

IgE Mediated/Immediate - Rapid reaction to cow’s milk, occurring within 2 hours of ingestion

Non-IgE Mediated/Delayed - reactions occur slowly over several days

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38
Q

CMPA - epidemiology

A
  • Typically affects infants and young children <3 y/o
  • Typically presents within first 3 months of life in formula-fed infants
    • Rarely seen in exclusively breastfed infants
  • 3-6% of all children
  • More common in:
    • Formula fed babies and those with a personal or family history of atopic conditions
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39
Q

CMPA - presentation (onset and symptoms)

A

Presents before 1 y/o typically <3 months; often apparent when weaning from breast milk to formula milk/food containing milk
* Breastfed babies when mother is consuming milk products
* In severe cases - failure to thrive (needs paediatric referral)

GI Symptoms
- Bloating and wind
- Colic symptoms/Abdominal pain - irritability and crying
- Diarrhoea
- Vomiting

Systemic Allergic Symptoms
- Urticarial rash (hives)
- (Rare) Angio-oedema (facial swelling)
- Cough or wheeze
- Sneezing
- Watery eyes
- Atopic eczema
*Rarely, in severe cases, anaphylaxis

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40
Q

CMPA - diagnosis/investigations

A

Often clinical diagnosis and trial of eliminating cow’s milk (with symptom resolution) should support diagnosis

Others:
- Skin prick/patch testing
- Total IgE and specific IgE (RAST) for cow’s milk protein

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41
Q

CMPA - management

A

Formula Fed:
- Mild/Moderate symptoms; 1st line is extensive hydrolyed formula (eHF)
- Severe/No response from eHF; Amino acid-based formula (AAF) eg neocate

Breastfed:
- Continue breastfeeding
- Mother eliminate all dairy from diet (consider Ca2+ supplements to prevent deficiency)
- Use eHF when breastfeeding stops until 12 months old for at least 6 months

Milk Ladder:
- Every 6 months or so try on 1st step
- Slowly progress until they develop symptoms
- Over time should be able to progress towards normal diet containing milk

42
Q

CMPA - prognosis

A

Usually resolves:
- IgE mediated intolerance - ~55% will be milk tolerant by the age of 5 years
- Non-IgE mediated intolerance - most children will be milk tolerant by the age of 3 years

43
Q

Reflux - GORD vs physiological

A

GORD - causes symptoms or complications eg FTT

Physiological - Up to 40% regurgitate feeds to some extent, usually begins before 8 weeks anfd resolves by 1 y/o (90%). Due to LOS natural weakness at this stage

44
Q

When should physiological reflux resolve by?

A

1 y/o in 90%

45
Q

GORD - aetiology

A

Anatomical causes:
- Shorter/narrower oesophagus
- LOS slightly above diaphragm

Physiological causes:
- Natural weakness of LOS (should resolve by 1 y/o)

46
Q

GORD - risk factors

A
  • Prematurity - the incidence of GORD is even higher in preterm infants, particularly if the infant has a feeding tube
  • History of congenital diaphragmatic hernia or oesophageal atresia
  • Hiatus hernia
  • Neuro-disability - e.g. cerebral palsy
  • Parental history of heartburn or acid regurgitation
47
Q

GORD - history features suggestive in <1 y/o

A
  • Time taken to feed >30 minutes
  • Distressed behaviour during meal times (e.g. crying while feeding or refusing to feed)
  • Hoarseness and/or chronic cough
  • Faltering growth
  • Vomiting - milky after feeds, may occur after being laid flat
  • Arching of the back
  • Drawing up of the knees into the chest - can be mistaken for seizures
  • Episode of pneumonia secondary to aspiration
48
Q

GORD - history features suggestive in >1 y/o

A

Similar to adults;
* Retrosternal pain
* Epigastric pain
* Bloating
* Nocturnal cough

49
Q

GORD - important features of examination

A

Observations; Temperature - exclude acute differentials like infections

Head Circumference; Measure + plot to exclude other worrying patterns eg faltering growth

Resp Exam; May have a single episode of pneumonia with GORD but recurrent episodes should prompt further investigations

Abdo Exam; Usually normal - positive findings should make you consider DDx

50
Q

Red flags of vomiting

A

General - Persistent symptoms despite management of GORD >1 y/o

Pyloric Stenosis/Intestinal Obstruction
* Not keeping down any feed
* Projectile or forceful vomiting (non-bilious in pyloric stenosis)

Intestinal Obstruction
* Bile stained vomit (green)
* Abdominal distension

Peptic Ulcer/Oesophagitis/Varices
* Haematemesis
* Melaena

Meningitis/Raised ICP
* Reduced consciousness
* Bulging fontanelle
* Neurological signs

Aspiration and Infection - Respiratory symptoms

Gastroenteritis/CMPA - Blood in stools

Infections
* Pneumonia
* UTI
* Tonsilitis
* Otitis
* Meningitis

CMPA
* Rash
* Angioedema
* Other signs of allergy

Apnoeas- Concerning feature and may indicate serious underlying pathology and need urgent assessment

51
Q

GORD - diagnosis/investigations

A

Clinical Diagnosis

  • If breastfed - consider feeding assessment by health visitor
  • Feeding history - to exclude red flags
  • Growth chart - document on chart to exclude other causes of faltering growth
52
Q

Bronchiolitis - definition

A

Infection and inflammation of bronchioles (LRT) leading to narrowing of the small airways

53
Q

Bronchiolitis - epidemiology

A
  • Most common 3-6 months
  • 1/3 develop <1 y/o
  • Occurs mostly over Winter with episodes lasting 7-10 days
54
Q

Bronchiolitis - aetiology

A

RSV - 80%

Adenovirus
Mycoplasma
Parainfluenza virus
Rhinovirus
Influenza
Human metapneumovirus

55
Q

Bronchiolitis - risk factors for more severe disease

A

Prematurity - bronchopulmonary dysplasia
Chronic lung disease
Congenital heart disease
CF
<3 months old
Down’s Syndrome
Neuromuscular disease

56
Q

Bronchiolitis - symptoms

A
  • SOB
  • Wheezing + persistent cough (often wet sounding)
  • Runny nose (coryza)
  • Mild temp
  • Often worsens on 2nd/3rd night
  • Poor feeding and dehydration if more severe
57
Q

Bronchiolitis - concerning feature at presentation in <6 weeks old

A

Apnoea

58
Q

Bronchiolitis - signs

A
  • Bilateral polyphonic wheeze
  • Tachypnoea
  • Tachycardia
  • Low grade fever
  • Irritability
  • Increased WOB (recessions, nasal flaring, accessory muscles, additional sounds, tracheal tug, head bobbing etc)
59
Q

Bronchiolitis - DDx

A

Pneumonia:
- Fever >39o
- Focal crackles

Viral-induced wheeze:
- Persistent wheeze without crackles
- Recurrent wheeze associated with a viral illness
- Personal or family history of atopy
- >1-year-old
- Responsive to salbutamol treatment

Early-onset asthma
- Persistent wheeze without crackles
- Recurrent wheeze associated with triggers
- Personal or family history of atopy
- >1-year-old
- Responsive to salbutamol treatment

Bordetella pertussis or whooping cough
- Coryza
- Characteristic hacking cough followed by an inspiratory ‘whoop’
- Unvaccinated

Gastro-oesophageal reflux
- Chronic cough
- Poor weight gain

Foreign body aspiration
- May have a history of choking
- Monophonic wheeze

Chronic heart disease or failure
- Cyanosis
- Shortness of breath
- Hepatomegaly
- Murmurs

60
Q

Bronchiolitis - diagnostic criteria

A

Present with coryzal symptoms lasting up to 3 days, followed by:

1) Persistent cough and
2) Tachypnoea or chest recession and
3) Wheeze or crackles on chest auscultation

61
Q

Bronchiolitis - supportive management

A

O2 - if SpO2 <92%

Feeding supplementation (Oral, NG or IV), stepwise approach may be considered:
1) Small and frequent oral feeds,
2) NG tube placement with small and frequent NG bolus feeds,
3) Continuous NG feeds,
4) IV fluids

Saline nasal drops/nasal suctioning - clears nasal secretions especially before feeding (as obligate nasal breathers)

Severe resp distress - resp support:
1) High flow O2 (eg Airvo to add PEEP) or CPAP
2) Intubation/ventilation
*Need to assess with capillary blood gases

62
Q

Bronchiolitis - other factors in managemet (especially if discharging)

A

Parental smoking cessation

Safety netting

63
Q

Bronchiolitis - treatment given to high risk babies

A

Palivizumab - monoclonal antibody against RSV
- Monthly injection
- Passive protection
- High risk; eg ex-prem, congenital heart disease

64
Q

Bronchiolitis - admission criteria

A
  • Apnoea
  • SpO2 <92%
  • Reduced oral intake <50-75% normal
  • Persistent respiratory distress - significant chest recessions, grunting
  • Presence of risk factors for severe disease
  • Difficult social factors
    ○ Living far away
    *Lack of parental confidence
65
Q

Bronchiolitis - why are bronchodilators and antibiotics not given

A

Not effective:
- Respiratory tract narrowing due to increased secretions, not bronchoconstriction
- Viral aetiology

66
Q

Bronchiolitis - complications

A
  • Clinical dehydration
  • SIADH and subsequent hyponatraemia
  • Apnoea and respiratory failure
67
Q

GORD - what is Sandifer’s syndrome

A

Rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants:

  • Torticollis; forceful neck muscle contractions causing twisting
  • Dystonia; abnormal muscle contractions cauisng twisting movements, arching of back or unusal postures

Referral to specialist needed as DDx is West syndrome/infantile spasms and seizures

68
Q

Scarlet Fever - definition

A

Reaction to erythrogenic toxins produced by Group A haemolytic streptococci (often Strep pyogenes)

69
Q

Scarlet Fever - epidemiology and transmission

A

Children 2-6 y/o
Peak incidence 4 y/o

Respiratory droplets

70
Q

Scarlet Fever - incubation period

A

2-4 days

71
Q

Scarlet Fever - Symptoms and signs

A

Symptoms:
- Fever (typically 48hrs)
- Abdo pain and N+V
- Sore throat
- Strawberry tongue

Rash:
- Sandpaper, rough
- Fine punctuate erythema (pinhead) generally appearing on torso
- Palms and soles spared
- Flushed appearance with circumoral pallor

72
Q

Scarlet Fever - diagnosis

A

Largely clinical but throat swab should be taken, start treatment whilst sample processed (don’t wait)

73
Q

Scarlet Fever - management

A

1st Line - Oral penicillin V for 10 days; if well no need to admit

Penicillin allergy - Macrolides; Clarithromycin/Azithromycin

PHE need to be notified

74
Q

Scarlet Fever - isolation period

A

Can return to school 24hrs after commencing Abx as no longer infectious

75
Q

Scarlet Fever - complications

A

Otitis Media - most common

Rheumatic Fever - typically 20 days after infection

Acute Glomerulonephritis - typically occurs 10 days after infection (Post-strep glomerulonephritis)

(Rare) Invasive complications - eg bacteraemia, meningitis, necrotising fasciitis

76
Q

Red flag fever readings

A

<3 months old - >38C

> 3 months - >39.5C (higher fevers may indicate serious bacterial infection)

77
Q

Barlow vs Ortolani

A

Barlow - attempts to dislocate an articulated femoral head

Ortolani - attempts to relocate a dislocated femoral head

78
Q

DDx for wheeze (expiratory)

A

Bronchiolitis - acute, coryza, <1 y/o (3-6m), RSV +ve

Viral-induced wheeze - acute, <3 y/o, no atopic hx, only occurs during viral infections

Asthma - chronic difficult in <5 y/o, family hx, atopic features, episodic, diurnal variation

CF - chronic, recurrent chest infections

Foreign body inhalation - typically unilateral, in toddlers

Allergy/Anaphylaxis - sudden onset, associated symptoms like urticarial rash, swelling of lips/face/tongue

Reflux - worse when lying down, associated with feeds

Chronic lung disease - ex pre-term, genetic malformation, chromosomal deletion

Pneumonia

79
Q

HSP - definition

A

A small vessel IgA vasculitis occurring in children
* Characteristic non-blanching purpuric/petechial rash typically distributed on lower limbs and buttocks
* Affects: Skin, Kidneys, GI Tract
* Often triggered by URTI or gastroenteritis
* Degree of overlap with IgA nephropathy (Berger’s disease)

80
Q

HSP - epidemiology

A

Most common in <10 y/o

81
Q

HSP - pathophysiology (incl. rash)

A

IgA Deposits - in small blood vessels causes inflammation in the affected organs

Rash - non-blanching purpuric
* Inflammation and leaking of blood from capillaries under skin forming purpura
* Hence non-blanching as has bled underneath/into skin

82
Q

HSP - key presentation

A
  • Child <10 y/o
  • Presenting following infection (URTI/gastroenteritis)
  • 100% - Non-blanching purpuric rash across lower limbs/arms/buttocks + extensor surfaces w/ localised oedema
  • 75% - Joint pain; mostly knees and ankles, can be swollen/painful, reduced ROM
  • 50% - Abdo pain
  • 50% - IgA nephritis (proteinuria, potentially hypertension)
  • May be pyrexial
83
Q

HSP - GI complications

A

Abdominal pain

Can lead to:
* Intussusception
* GI haemorrhage
* Bowel infarction

84
Q

HSP - IgA nephritis features

A

Proteinuria - if ≥2+ = Nephrotic syndrome; will have a degree of oedema

Micro or Macroscopic haematuria

85
Q

HSP - DDx

A
  • Meningococcal septicaemia
  • ITP
  • Leukaemia
  • Haemolytic Uraemic Syndrome
  • Viral illnesses
  • Mechanical causes (eg abuse)
  • Petechiae around face can be from coughing/vomiting
86
Q

HSP - investigations

A

Need to exclude serious pathology first

Bloods:
* FBC - ITP (Thrombocytopaenia), ?Sepsis (Neutrophilia)
* Blood Film - Leukaemia (presence of blast cells)
* Renal Profile
* Serum albumin - Nephrotic syndrome
* CRP - Sepsis

Blood Cultures - For meningococcal sepsis

Urine
* Dipstick - Proteinuria (≥2+ = Nephrotic syndrome)
* Urine Protein:Creatinine Ratio - Quantify proteinuria

Blood Pressure - Hypertension (corrected for height)

87
Q

HSP - EULAR/PRINTO/PRES diagnostic criteria

A

Palpable purpura (not petechiae) + 1 of:
1) Diffuse abdominal pain
2) Arthritis or arthralgia
3) IgA deposits on histology (biopsy)
4) Proteinuria or haematuria

88
Q

HSP - management + monitoring

A

Supportive - Simple analgesia, rest + hydration

Other -
* Anti-hypertensives may be considered if hypertensive
* Steroids (inconsistent evidence) - potential if severe abdo pain/renal involvement

Monitoring - BP and urinalysis for 12 months

89
Q

HSP - prognosis

A
  • Majority recover completely within 4-6 weeks
  • 1/3 relapse within 6 months
  • 1/5 have long term renal impairment with significant proteinuria (very small proportion develop ESRD)
90
Q

HSP - Gell and Coombs hypersensitivity category

A

Type 3 - Immune complex mediated + IgG

91
Q

Cerebral Palsy - definition

A

A group of permanent, non-progressive movement/posture disorders occurring as a result of a lesion of the motor pathways in the developing CNS
* Nature of symptoms may change over time during growth and development
* Huge variation from completely wheelchair bound/dependent to paralympic athletes with subtle co-ordination/mobility problems

92
Q

Cerebral Palsy - epidemiology

A

2-3/1,000 children

93
Q

Cerebral Palsy - aetiology

A

Antenatal - 80%
* Congenital malformation
* Congenital infection - eg rubella, toxoplasmosis, CMV
* Trauma during pregnancy

Perinatal/Intrapartum - 10%
* Birth asphyxia/trauma (hypoxic-ischaemic encephalopathy)
* Pre-term birth

Postnatal - 10%
* Intraventricular haemorrhage
* Meningitis
* Head-trauma
* Medication toxicity
* Kernicterus/Severe neonatal jaundice - brain damage from hyperbilirubinaemia

Idiopathic

94
Q

Cerebral Palsy - types

A

(Manifestations dependent on site + extent of lesion, categories may overlap and have wide variety)

Spastic/Pyramidal - 70-90%
* Hypertonia and reduced function resulting from damage to upper motor neurones (pyramidal pathways)
* General features: increased tone and reflexes, clasp knife, flexed hip and elbow
* Scissor gait
* May be monoplegic, diplegic, hemiplegic

Dyskinetic/Athetoid/Extrapyramidal -
* Damage to basal ganglia (may exhibit choreiform movement or signs of Parkinsonism)
* Problems controlling muscle tone, with hypertonia and hypotonia
* Athetoid movements and oromotor problems

Ataxic -
* Cerebellar damage - DANISH and lack of co-ordination

Mixed

95
Q

Cerebral Palsy - distribution patterns

A

Monoplegia - One limb

Hemiplegia - One side of body affected

Diplegia - Four limbs affected but mostly legs

Quadriplegia - Four limbs affected more severely, often with:
* Seizures
* Speech disturbance
* Other impairments

96
Q

Cerebral Palsy - possible manifestations + signs and symptoms

A
  • Hand preference below 18 months is a key sign to remember for exams
  • Abnormal tone early infancy - hyper or hypo, general or in specific limbs
  • Delayed motor milestones
  • Abnormal gait
  • Feeding/swallowing difficulties
  • Problems with coordination, speech or walking
  • Learning difficulties
97
Q

Cerebral Palsy - non-motor problems

A
  • Learning difficulties (60%)
  • Epilepsy (30%)
  • Squints (30%)
  • Hearing impairment (20%)
98
Q

Cerebral Palsy - examination

A

Gait
* Hemiplegic or diplegic; caused by increased muscle tone and spasticity in the legs
* Legs extended with plantar flexion of feet and toes; so have to swing leg around in a large semicircle when moving leg forward (not enough space to swing extended leg below them)

UMN Signs
* Good muscle bulk
* Increased tone
* Brisk reflexes
* Slightly reduced/normal power

Athetoid Movements - indicate extrapyramidal (basal ganglia involvement)

Co-ordination Tests - for cerebellar involvement

99
Q

Cerebral Palsy - DDx

A
  • Acquired brain injury
  • Brain tumour
100
Q

Cerebral Palsy - management

A

MDT Approach:

*Physio - stretch/strengthen muscles to maximise function + prevent painful contractures

*Occupational Therapy - used to help patients manage their everyday activities eg techniques or home adaptations

  • SALT - help with speech and swallowing (including safe swallow)
  • Dieticians - ensure meeting nutritional requirements
  • Surgical - Ortho (MSK deformities, tenotomies, injuries), GenSurg (PEG tube fitting), Neurosurg (selective dorsal rhizotomy)
  • Paeds - Regular involvement + optimising meds
  • Social workers - help with benefits and support
  • Charities/Support groups - opportunities to connect with others affected and share info
101
Q

Cerebral Palsy - pharmacological management options

A

Muscle relaxants for spasticity -
* Oral and intrathecal baclofen for spasms
* Botox injections (botulinum toxin type A) for contractures
* Oral diazepam

Anti-epileptic drugs

Glycopyrronium bromide - for excessive drooling

Analgesia

102
Q

Cerebral Palsy - complications

A
  • Learning disability
  • Epilepsy
  • Injuries from impaired balance/co-ordination
  • Aspiration pneumonias from impaired swallowing
  • Kyphoscoliosis and other MSK deformities resulting from impaired posture and muscle control
  • Muscle contractures and wasting
  • Hearing and visual impairment
  • Gastro-oesophageal reflux