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Coovadias Paediatrics > Neonatology > Flashcards

Neonatology Flashcards

1
Q

How does the neonate physiologically prepare for breathing?

A
  1. Incr cortisol, TSH, catecholamines -> surfactant production + process to clear lung fluid starts
  2. Maturation of the brain’s respiratory centre
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2
Q

What are the cardiovascular changes from the neonate to the postnate?

A
  1. Decr pulmonary vascular resistance
  2. Ductus venosus
  3. Incr systemic resistance
  4. Shunt reversal
  5. Ductus arteriosus
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3
Q

What does the umbilical artery become in the postnate?

A 
Patent = superior vesicle arteries
Obliterated = medial umbilical ligaments
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4
Q

What are the metabolic changes from the neonate to the postnate?

A

Gluconeogenesis

Glycogenolysis

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5
Q

Name foetal risk factors for poor adaption of the neonate to extrauterine life

A 
Foetal distress
Meconium stained liquor
Premature
Post-term
IUGR
Multiple birth
Abnormal presentation
Shoulder dystocia
Assisted delivery
Infection
Congenital malformation
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6
Q

Name maternal risk factors for poor adaption of the neonate to extrauterine life

A 
Pre-eclampsia
Chronic hypertension
Diabetes
Infection
Drug use
Polyhydramnios
Oligohydramnios
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7
Q

Name placental risk factors for poor adaption of the neonate to extrauterine life

A

Chorioamnionitis
Abruptio placenta
Placenta previa
Cord prolapse

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8
Q

Discuss the APGAR score

A

Activity Pulse Grimace Appearance Resp

  1. Heart rate
    - absent (0)
    - <100bpm (1)
    - >100bpm (2)
  2. Respiration
    - absent (0)
    - slow, irregular (1)
    - regular, cry (2)
  3. Muscle tone
    - limp (0)
    - some flexion of extremities (1)
    - active movement (2)
  4. Response to stimulation
    - no response (0)
    - grimace (1)
    - cough, sneeze, cry (2)
  5. Colour
    - blue/pale (0)
    - body pink extremities blue (1)
    - pink (2)
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9
Q

What are the ABCDs of neonatal resuscitation?

A 
Anticipate high risk pregnancies
Assessment after delivery
Airway management
Breathing
Circulation
Drugs
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10
Q

Name consequences of asphyxia

A 
Acute
CNS
- apnoea
- seizures
Renal
- ATN
Adrenal
- haemorrhage
- failure
Cardiac
- ischaemia
Lung
- PPHN
GIT
- delayed transit
- NEC
Liver
- hepatic dysfunction

Chronic

  • CP
  • epilepsy
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11
Q

Name maternal risk factors for a high risk pregnancy

A 
Age
Diabetes
Hypertension
Anemia
Renal disease
Infection
Drugs
Substance abuse
Unbooked
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12
Q

Name fetal risk factors for a high risk pregnancy

A 
Congenital anomaly
Multiple gestation
Prematurity
Bradycardia
IUGR
Placenta abruptio/praevia
Polyhydramnios
Oligohydramnios
IUGR
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13
Q

Name labour and delivery risk factors for a high risk pregnancy

A 
Abnormal CTG
MSL
PROM
Cord prolapse
Abnormal presentation
Prolonged labour
Emergency C/S
Narcotic drugs
GA
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14
Q

Which 3 vital signs must be assessed?

A

Breathing
Colour
Heart rate

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15
Q

Differentiate primary vs secondary apnoea

A 
Primary = HR >100; recovery spontaneous
Secondary = HR<100; no recovery without resus
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16
Q

What is crucial to assess in the newborn resus algorithm?

A

Term gestation
Breathing
Good tone

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17
Q

What is routine care with the mother?

A

Dry the baby
Place skin to skin
Cover with a dry linen

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18
Q

How do you manage the newborn that is missing any of the 3 crucial assessment objectives?

A

Move to radiant warmer for further management

  1. Stabilise
    - warm
    - position airway ‘sniffing’
    - clear secretions
    - dry
    - stimulate
  2. Ventilate
    - free flow oxygen
    - positive pressure
  3. Chest compressions
  4. Adrenaline w/wo volume expansion
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19
Q

What gestational age should not be dried at birth? What is the alternative?

A

<30 weeks gestation

Wrap preterm baby’s torso in plastic bag

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20
Q

What is ‘the golden minute’

A

60s to stabilise, re-evaluate and start ventilation

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21
Q

What is assessed in the breathing step of the newborn resus algorithm?

A

Assess breathing/crying and/or heart rate

Gasping, apnoeic, persistent central cyanosis despite 100% oxygen or HR<100 = next step!

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22
Q

How do you maintain normothermia in the newborn?

A 
Environment must be 24-26 degrees
Put on radiant warmer before dilvery
Close doors and windows
Dry baby
Remove wet linen
Thermal mattress
Head cap
Plastic bag
Humidified resus gases
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23
Q

Neonates below what values are at high risk of hypothermia?

A

<1500g

<32 weeks

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24
Q

What is newborn hypothermia associated with?

A

Respiratory distress
Hypoglycemia
Late-onset sepsis

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25
Q

When do you perform suctioning?

A

Obvious obstruction to spontaneous breathing

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26
Q

When do you perform suction in MSL newborns?

A

If infant is non-vigorous

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27
Q

Discuss how you would approach suctioning the infant with a catheter

A

Mouth BEFORE nose

Rule of 5

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28
Q

What is the rule of 5?

A

Suction catheter passed to the depth of no more than 5cm for 5 seconds

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29
Q

What can overzealous suctioning cause?

A

Laryngospasm
Bradycardia
Minor abrasions -> infection

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30
Q

How can you stimulate the newborn?

A

Dry
Rub the back
Flick the soles of the feet

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31
Q

What are the methods of free flow oxygen delivery to the newborn?

A

Hand cupped
Funnel
Mask

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32
Q

Name 2 assisted ventilation devices and which is best?

A

Ambubag

T-piece resuscitator = best (can control amount)

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33
Q

Compare rim masks with round masks

A

Rim mask

  • conforms to shape of face (better seal)
  • less pressure required (safer for eyes)

Round mask

  • don’t need to inflate rim
  • danger of pressure on the eyes if large
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34
Q

What technique must you use to apply the mask to the face?

A

C E

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35
Q

How do you know your ventilation is effective?

A

HR improvement
Effective chest expansion
Colour improvement

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36
Q

Name common causes of ineffective ventilation

A

Poor head position
Airway obstruction
Poor mask application
Faulty bag

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37
Q

What is the acronym to ensure adequate ventilation?

A 
MR SOPA
Adjust face MASK to assure good seal
REPOSITION airway
SUCTION mouth and nose
OPEN mouth slightly and move jaw forward
PRESSURE increase to achieve chest rise
AIRWAY alternative (ETT/laryngeal mask)
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38
Q

What is the best indicator of successful oxygenation?

A

Heart rate

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39
Q

How can you assess heart rate?

A

3 lead ecg (gold standard)
Saturation monitor
Auscultation

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40
Q

What oxygen saturation do you start infants at?

A

Term
- 21% (room air)
Preterm
- 21%-30%

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41
Q

How do you measure pre-ductal sats?

A

Right hand/ear

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42
Q

What are normal pre-ductal sats after birth?

A 
1min >60%
2min >65%
3min >70%
4min >75%
5min>80%
>10min 90-95%
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43
Q

What are indications for neonatal intubation?

A

Ineffective bagmask
Prolonged ventilation
Need for compressions

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44
Q

Should you use an ETT or laryngeal mask for neonatal intubation?

A

ETT first

If unsuccesful/unfeasible and neonate >34w -> laryngeal mask

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45
Q

Which blade should you use on your laryngoscope in neonates?

A

Straight blade

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46
Q

Do neonatal ETT have inflatable cuff?

A

No

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47
Q

How do you verify correct tracheal tube placement?

A

Symmetrical chest movement
Auscultation (breathing sounds all lung fields)
Condensation
Improvement in colour, HR and activity

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48
Q

What is the formula for tube depth?

A

Weight (kg) + 6 = _cm at the lip

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49
Q

Discuss tube sizes required for infants of varying weight

A 
<1000g = 2.5 
1000g-2000g = 3.0
2000g-3000g = 3.5
3000g-4000g = 4.0
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50
Q

How often should you assess your infant’s breathing, HR, colour and sats?

A

Every 30-60s

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51
Q

What do you do if HR<100?

A

Start PPV

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52
Q

What do you do if HR<60?

A

3:1 compressions over 2 seconds repeated

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53
Q

What are the techniques for chest compressions in infants and which is superior?

A

Hand encircling
Two finger

Hand encircling is better as it generates higher BP and results in less rescuer fatigue

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54
Q

What is the rule of 3?

A

Compressions at lower 1/3 baby sternum
Compress to 1/3 baby chest
3 compression:1 breath

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55
Q

What is the preferred route of IV access in neonates?

A

Umbilical vein

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56
Q

What do you do if HR <60 despite adequate ventilation and chest compressions?

A

Give adrenaline

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57
Q

What is the dose for normal saline?

A

10ml/kg IV over 5-10min

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58
Q

What is the dose for dextrose?

A

2-3ml/kg IV 10% dextrose

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59
Q

When should you not start resus?

A

Futile

  • dead baby
  • lethal anomaly

Poor prognosis

  • BW<500g
  • trisomy 13
  • trisomy 18
  • severe hydocephalus
  • single ventricle
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60
Q

When do you stop resus?

A

After 10min continuous and adequate resus if HR undetectable

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61
Q

When do you stop resus?

A

After 10min continuous and adequate resus if HR undetectable

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62
Q

What should you do as part of routine post-delivery care?

A

Vitamin K
Antibacterial eye ointment
Umbilical cord care

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63
Q

What should you do at the postnatal follow up?

A

Surveillance of growth and development
Immunisation
Hearing screen

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64
Q

What should you do at the postnatal follow up?

A

Surveillance of growth and development
Immunisation
Hearing screen

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65
Q

Define a birth injury

A

Traumatic event at birth causing structural destruction or functional deterioration of the neonate’s body

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66
Q

Name risk factors for birth injuries

A 
Macrosomia
Prematurity
Instruments (forceps, vacuum)
Abnormal presentation
Precipitous delivery
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67
Q

Name kinds of birth injuries

A 
Soft tissue
Cranial
Nerve
Fractures
Intra-abdominal
Subconjunctival haemorrhage
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68
Q

Name soft tissue birth injuries

A 
Erythema
Abrasions
Petechiae
Ecchymoses
Bruising
Subcutaneous fat necrosis
Lacerations
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69
Q

Compare localised vs generalised petechiae in infants

A

Localised

  • due to sudden increase in venous pressure -> pinpoint capillary rupture
  • resolves spontaneously

Generalised
- investigate

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70
Q

Name risk factors for subcutaneous fat necrosis

A

Perinatal asphyxia
Cold exposure
Trauma

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71
Q

How long does subcutaneous fat necrosis take to resolve?

A

6-8 weeks

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72
Q

What is a child with subcutaneous fat necrosis at risk of developing?

A

Hypercalcemia up to 6months

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73
Q

Discuss management of laceration birth injuries

A 
Superficial = adhesive tape, keep clean
Deep = suture
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74
Q

Name cranial injuries

A

Extracranial

  • skull moulding
  • caput succedaneum
  • cephalhaematoma
  • subgaleal haemorrhage

Intracranial

  • subdural haemorrhage
  • epidural haemorrhage
  • subarachnoid haemorrhage
  • intraventricular haemorrhage
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75
Q

How long does moulding take to resolve?

A

Days

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76
Q

What are the layers of the scalp?

A 
SCALP
Skin and dense Connective tissue
Aponeurosis (epicranial)
Loose areolar connective tissue
Periosteum
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77
Q

Discuss management of caput succedaneum

A

Improves 48-72 hours and resolves in 4-6days
No treatment
Not resolving -> investigate

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78
Q

What is cephalhaematoma associated with?

A

Vacuum delivery

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79
Q

How long does cephalhaematoma take to resolve?

A

3-4 weeks

Calcifies up to 4 months

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80
Q

Compare the different extracranial injuries

A

Caput = soft oedema
Cephalhaematoma = subperiosteal bleeding
Subgaleal bleed = subaponeurotic bleeding

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81
Q

What limits the bleeding of a subgaleal haematoma?

A

Orbital ridges anteriorly
Temporal fascia laterally
Nape of neck posteriorly

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82
Q

What are infants with subgaleal haematoma at risk of developing?

A

Hypovolemic shock (can lose substantial blood volume)

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83
Q

How do you observe an infant with subaponeurotic haemorrhage?

A

Serial head circumference
Hb
Vital signs

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84
Q

What are all infants with a bleeding injury at risk for?

A

Jaundice

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85
Q

Discuss management of a subaponeurotic haemorrhage

A

Pain relief
Volume resus
RBC/FFP transfusion

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86
Q

Name signs of intracranial birth injury

A

Bulging fontanelle
Hypotonia
Seizures
Decr LOC

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87
Q

Which nerve injuries are common birth injuries?

A

Facial nn
Brachial plexus
Phrenic nn

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88
Q

Does facial nerve injury resolve spontaneously or require treatment?

A

Resolves within 2-3 weeks

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89
Q

Name signs of facial nerve injury

A

Loss of nasolabial fold
Ptosis
Corner of mouth drooping
Mouth drawn to unaffected side

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90
Q

Name risk factors for brachial plexus injury

A

Shoulder dystocia
Macrosomia
Breech presentation
Instrument assisted deliveries

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91
Q

What is the most common type of brachial plexus injury?

A

Erb-Duchenne palsy (C5-6)

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92
Q

How does a child with Erb-Duchenne palsy appear?

A

Arm in adduction, internal rotation, fully extended at elbow, pronated forearm and flexion of wrist
Intact grasp reflex
Assymetrical Moro

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93
Q

Name the types of brachial plexus injury

A

Erb-Duchenne (C5-6)
Klumpke (C8-T1)
Total arm paralysis
Horner syndrome

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94
Q

How does a phrenic nerve injury occur?

A

Damage C3-C5
Impaired diaphragm
Ineffective respiration
Assoc arm weakness

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95
Q

How do you identify a phrenic nn injury on CXR?

A

Elevated diaphragm

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96
Q

Name fracture birth injuries

A

Clavicle
Humerus
Femur
Skull

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97
Q

Name risk factors for clavicle fracture

A

Shoulder dystocia
Macrosomia
Instrument assisted delivery

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98
Q

What is the prognosis for clavicle fracture at birth?

A

Excellent

Heals 7-10 days

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99
Q

How will a child with a humerus fracture at birth appear?

A 
Deformity
Crepitus
Overlying petechiae
Absent arm movement
Absent Moro
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100
Q

How do you treat a humerus fracture?

A

Immobilise arm in adduction 2-4w

Pain management

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101
Q

What is femur fracture associated with?

A

Breech delivery

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102
Q

Define neonatal encephalopathy

A

Disturbed neurological function in the first 28 days in infants born ≥ 35 weeks gestation

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103
Q

Name signs of neonatal encephalopathy

A 
Abnormal state of consciousness
Decreased spontaneous movements
Resp difficulty
Feeding difficulty
Poor tone
Abnormal posture
Absent/partial primitive reflexes
Seizures
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104
Q

Name causes of neonatal encephalopathy

A 
Hypoxic ischaemic encephalopathy
Perinatal stroke
- maternal cocaine abuse
- pre-eclampsia
- prothrombotic disorders
- congenital heart disease
IVH
Metabolic disorders
- hyperammonemia
- hypoglycemia
- organic acidemia
- amino acidemia
- mitochondrial d/o
- fatty acid metabolism d/o
Withdrawal from maternal drugs
- narcotics
- alcohol
- SSRIs
- TADs
Genetic
- Prader Willi
- chromosomal
Structural brain anomalies
Meningitis
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105
Q

Discuss how you determine the cause of neonatal encephalopathy

A 
Clinical evaluation of neonate
Maternal medical history
Obstetric history
Intrapartum factors
Blood gas within 1hr of birth
Multi-organ dysfunction
Neuroimaging
EEG
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106
Q

How do you diagnose HIE?

A
  1. Abnormal clinical signs
  2. Contributing events in close temporal proximity to labour and delivery (sentinel event)
  3. Developmental outcome (long term) – spastic
    quadriplegia or dyskinetic CP
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107
Q

Name pre-conceptual and conceptual risk factors for HIE

A

Maternal age >35yo
Family hx of seizures/neurological disease
Infertility treatment
Prev neonatal death

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108
Q

Name antepartum risk factors for HIE

A 
Maternal prothrombotic/proinflammatory disorder
Maternal thyroid disease
Severe preeclampsia
Chorioamnionitis
Multiple gestation
Genetic abnormality
Congenital malformations
IUGR
Trauma
Breech
APH
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109
Q

Name intrapartum risk factors for HIE

A 
Abnormal CTG
Thick meconium
Assisted vaginal delivery
GA
Emergency C/S
Abruptio placenta
Cord prolapse
Uterine rupture
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110
Q

Name postnatal risk factors for HIE

A

Pulmonary disease
Neurological disease
Cardiovascular disease

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111
Q

Name labour and delivery risk factors for HIE

A 
Maternal
- cardiac arrest
- asphyxiation
- hypovolemic shock
- status epilepticus
Uteroplacental
- abruptio placenta
- cord prolapse
- uterine rupture
- hyperstimulation of uterus with oxytocics
Foetal
- fetomaternal haemorrhage
- twin to twin transfusion syndrome
- cardiac arrythmias in utero
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112
Q

What are signs that the encephelopathic event was acute peripartum or intrapartum?

A

APGAR <5 at 5 and 10min
Fetal umbilical aa acidemia (pH <7 or base deficit >12)
Multisystem involvement
Neuroimaging evidence of acute brain injury

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113
Q

Which organs can be involved in HIE?

A 
Renal
- acute kidney injury
-Hepatic
- elevated liver enzymes
Cardiac 
- decr CO -> low BP
Haematological
- low platelets
- coagulopathy
- DIC
GIT
- NEC
Metabolic
- hyponatremia (SIADH)
- hypocalcemia
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114
Q

What is the long term outcome of hypoxic insult according to gestational age?

A

26-36 weeks
Ischaemia
- periventricular white matter
- spastic diplegia

> 36 weeks
Partial asphyxia
- parasagittal watershed areas w/wo cortex
- behavioural problems, language delays, cognitive deficits, possible epilepsy, severe motor impairment (rare)
Acute total asphyxia
- deep gray matter and peri-rolandic cortex
- spastic quad CP
- dystonic CP
- cognitive

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115
Q

What is the long term outcome of partial asphyxia >36weeks?

A 
Area: parasagittal watershed areas w/wo cortex
Result
- behavioural problems
- language delays
- cognitive deficits
- possible epilepsy
- severe motor impairment (rare)
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116
Q

What is the long term outcome of acute total asphyxia >36w?

A 
Area:deep gray matter and peri-rolandic cortex
Result
- spastic quad CP
- dystonic CP
- cognitive
- possible epilepsy
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117
Q

What is the long term outcome of ischaemia before 36 weeks?

A 
Area: Periventricular white matter
Result
- spastic diplegia
Why?
Periventricular malacia increases risk of IVH which leads to spastic diplegia
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118
Q

Name deep grey matter

A

Basal ganglia

Thalamus

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119
Q

Name the constituents of the peri-rolandic cortex

A

Precentral gyrus
Postcentral gyrus
Paracentral lobule

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120
Q

What is the gold standard for identifing a hypoxic injury?

A

MRI

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121
Q

Discuss your management of HIE

A 
Therapeutic hypothermia
Supportive
Neuroprotective 
- antiepileptics
- EPO
- melatonin
- xenon
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122
Q

How soon must you start therapeutic hypothermia?

A

Best outcome - within 3 hours

Must be within 6 hours

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123
Q

What is the core temperature and duration of therapeutic hypothermia?

A

Core temp 33.5 - 34.5

72 hours

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124
Q

Discuss how you would determine the child’s eligibility for cooling

A 
A. Gestation age >36w AND <6hrs of age
B. Physiological criteria
- pH <7 or BD >16
- <5 APGAR 5 and 10min
- ongoing resus from birth >10min
C. Neurological criteria
- moderate to severe encephalopathy 
- abnormal background/seizure activity on aEEG
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125
Q

Discuss the modified sarnat score for encephalopathy

A 
Moderate encephalopathy
LOC = lethargic
Spontaneous activity = decr
Posture = distal flexion, complete extension
Tone = hypotonia
Primitive reflexes
- suck = weak
- moro = incomplete
Autonomic system
- pupils = constricted
- HR = bradycardia
- resp = periodic
Severe encephalopathy
LOC = stupor/coma
Spontaneous activity = none
Posture = decerebrate
Tone = flaccid
Primitive reflexes
- suck = absent
- moro = absent
Autonomic system
- pupils = deviated/dilated/non-reactive
- HR = variable
- resp = apnoea
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126
Q

Why do we not cool deeper or for longer?

A 
More side effects with no benefit (found in study)
120 hours
- incr arrythmia risk
- incr anuria risk
- longer hospital stay
32 degrees
- incr bradycardia risk
- incr iNO use
- incr ECMO need
- more days on O2
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127
Q

Name complications of therapeutic hypothermia

A 
Sinus bradycardia
Prolonged QT interval
Systemic hypothermia
Subcutaneous fat necrosis
Thrombocytopenia
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128
Q

Discuss supportive management of encephalopathy

A

Control seizures
- Phenobarbital, lorazepam, phenytoin, levetiracetam
Exclude meningitis
- LP

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129
Q

Which seizures can’t an aEEG detect?

A

Short seizures
Low amplitude seizures
Focal discharges

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130
Q

When do you perform the MRI for HIE?

A

T1/T2/diffusion weighted

24-96hrs post delivery and repeat >day 9

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131
Q

When do you perform a Thomson score and what does it predict?

A

Perform at birth
Predicts outcome at 12months
Good prognosis = <10
Poor prognosis = >15

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132
Q

Name minor abnormalities in the first few days of life

A 
Peripheral cyanosis
Subconjunctival haemorrhage
Ebstein pearls
Umbilical hernia
Breast enlargement
Neonatal menarche
Vaginal discharge
Hymen tags
Positional talipes
Nevus simplex
Nevus flammeus
Milia
Miliaria
Erythema toxicum neonatorum
Transient pustular melanosis
Dermal melanosis
Transient vascular phenomena
Sucking blisters
Neonatal teeth
Gingival cysts
Polydactyly
Preauricular skin tag
Preauricular pit
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133
Q

What are Ebstein pearls?

A

Entrapped epithelium during palatal development -> 1-3mm at midline of palate

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134
Q

Which part of the palate are Ebstein pearls typically found?

A

Hard palate

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135
Q

Why do newborns experience breast enlargement?

A

Declining maternal oestrogen -> prolactin stimulation of newborn’s pituitary gland

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136
Q

Why do female neonates sometimes experience white vaginal discharge?

A

Maternal hormone withdrawal

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137
Q

How do you differentiate positional talipes from talipes equinovarus?

A

Passively move the foot into neutral position
Possible = positional
Impossible = equinovarus

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138
Q

How is a club foot managed?

A 
Non surgical
- stretching
- casting
Surgical
- tendons
- ligaments
- joints
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139
Q

What is another name for a club foot?

A

Talipes equinovarus

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140
Q

Do nevus simplex blanche when compressed?

A

Yes

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141
Q

What is nevus simplex?

A

Dilated superficial capillaries on eyelids, forehead, scalp and nape of neck

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142
Q

What do you need to exclude in a patient with nevus flammeus in opthalmic distribution of CNV?

A

Sturge Weber syndrome

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143
Q

What are milia?

A

Retention of keratin and sebaceous material in follicles -> pin sized on nose, cheeks and forehead

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144
Q

What are miliaria?

A

Retained sweat in obstructed eccrine glands -> pin sized vesicle on face, neck and chest

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145
Q

How can miliaria be prevented?

A

Avoid overheating

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146
Q

What is erythema toxicum neonatorum?

A

Small, white/yellow pustules on erythmatous base

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147
Q

When does erythema toxicum neonatorum appear?

A

Appears 1-3 days of life but can be present at birth

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148
Q

Where does erythema toxicum neonatorum appear?

A

Trunk, face, perineum, extremities

NEVER palms or soles!

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149
Q

What is transient pustular melanosis?

A

Superficial vesicopustular lesions with no erythmatous base

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150
Q

When does transient pustular melanosis appear?

A

Birth - weeks later

Ruptures within 48hrs and leaves hyperpigmented macules

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151
Q

What is another name for mongolian blue spots?

A

Dermal melanosis

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152
Q

Which population is dermal melanosis more common in?

A

African population

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153
Q

What is harlequin colour change and how does it appear?

A

Immature hypothalamic center -> lacks control of dilation of peripheral blood vessels
Appears as erythema on dependent side of body with blanching of contralateral side

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154
Q

What is cutis marmorata and how does it appear?

A

Vascular response to the cold -> symmetrical reticulated mottling

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155
Q

How are neonatal teeth managed?

A

Remove if loose to prevent aspiration

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156
Q

What other anomalies should you look for in polydactly?

A

Trisomy 13

Beckwith-Weideman syndrome

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157
Q

What abnormalities are associated with preauricular skin tags/pits?

A

Renal abnormalities -> perform renal sonar

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158
Q

Differentiate vomiting from regurgitation

A 
Regurgitation = passive, effortless
Vomiting = involuntary, active, forceful
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159
Q

What are you looking for on antenatal history in the vomiting child?

A

Pre-eclampsic toxemia (IUGR)
Maternal infections
Polyhydramnios
Oligohydramnios

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160
Q

What is polyhydramnios in an infant that vomits an indication of?

A

Upper GI obstruction

  • atresia
  • stenosis
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161
Q

What is oligohydramnios in an infant that vomits an indication of?

A

Congenital renal abnormalities

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162
Q

What are you looking for on postnatal history in the vomiting child?

A 
Birth hx
- premature
- HIE
Onset
Feeds
Growth, weight, FTT
Neurological signs
- CP w/ bulbar weakness
- raised ICP
Respiratory signs
- apnoea
- strider
- cystic fibrosis
- recurrent aspiration
- tracheosopheagel fistula
Gastrointestinal
- abdominal distension
- stool characteristics (passage, colour)
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163
Q

What do children with CP with bulbar weakness often have?

A

Associated reflux

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164
Q

What are symptoms of oesophagitis?

A

Irritability
Arching back
Feed refusal
Haematemesis

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165
Q

What are symptoms suggestive of cow’s milk protein allergy?

A

Eczema

Diarrhoea

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166
Q

What is golden standard for GER?

A

pH manometry

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167
Q

GER vs GERD

A

GER is normal

GERD is a disease

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168
Q

Why can pH manometry test negative in infants?

A

Alkali reflux (not acidic)

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169
Q

Discuss management of GER

A

Non-pharmacological

  • position
  • smaller volumes more frequently
  • splitting feeds
  • feeds thickener

Pharmacological
- PPI (efficacy not established)

Surgical
- Nissan fundoplication

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170
Q

How can you categorise vomiting?

A

Bilious

Non-bilious

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171
Q

What are obstructive causes of non-bilious vomiting?

A

Obstruction above ampulla of vater

  • pyloric stenosis
  • upper duodenal stenosis
  • annular pancreas
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172
Q

What are non-obstructive causes of non-bilious vomiting?

A 
Physiological
- overfeeding
- NGT placed incorrectly
Infection
- gastro
- NEC
- septicemia
- UTI
- meningitis
CNS
- incr ICP
- intracranial bleed
Endocrine
- congenital adrenal hyperplasia
Drugs
- aminophylline
- caffeine
- neonatal abstinence syndrome
Inborn error of metabolism
Cow's milk protein allergy
Decreased motility
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173
Q

What are causes of bilious vomiting?

A 
Anatomical obstruction
- small bowel stenosis/atresia
- large bowel stenosis/atresia
- imperforated anus
- annular pancreas
- volvulus 
Functional obstruction
- Hirschprung's disease
- meconium ileus
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174
Q

How do you approach the newborn with abdominal distension?

A
  1. Intestinal obsutrction
    - functional vs mechanical
  2. Ileus
  3. Pneumoperitoneum
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175
Q

Which neonates usually have ileus?

A

Neonate with sepsis

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176
Q

What is the issue with hypokalemia in ileus?

A

Causes decreased peristalsis -> worsens the situation!

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177
Q

How do you manage ileus?

A

Rest the bowel
NPO
NGT decompression on bowel
Identify and treat underlying cause

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178
Q

What do you look for on AXR to diagnose pneumoperitoneum?

A

Air under the diaphragm

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179
Q

How do you manage pneumoperitoneum?

A

NPO
NGT decompression on bowel
ABCs
Surgery

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180
Q

How does UGIT obstruction present?

A

Polyhydramnios
Vomiting (early)
Abdominal distension (late)
W/wo meconium passage

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181
Q

How does LGIT obstruction present?

A

Abdominal distension
Delayed/absent meconium passage (early)
Vomiting (late)

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182
Q

What is oesphaeal atresia usually associated with?

A

Trachea-oesophageal fistula

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183
Q

Name clinical features of oeophageal atresia

A

Polyhydramnios
Incr frothy secretions
Choking
Cyanotic spells

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184
Q

How do you diagnose oesophageal atresia?

A

Place NGT and take CXR

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185
Q

Which condition is duodenal atresia associated with?

A

Trisomy 21

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186
Q

How do you diagnose duodenal atresia using AXR?

A

‘Double bubble’

187
Q

Name clinical features of pyloric stenosis

A

Visible peristalsis
Olive-like mass in RUQ
Hypochloremic, hypokalemic metabolic alkalosis

188
Q

How do you diagnose pyloric stenosis?

A

U/S

189
Q

When will a patient with pyloric stenosis present?

A

4-8weeks after birth

190
Q

Which birth defect is more common: gastroschisis or omphalocele?

A

Omphalocele

191
Q

Differentiate gastroschisis from omphalocele

A

Gastroschisis

  • right paraumbilical
  • no covering sac
  • free intestinal loops
  • NEC common

Omphalocele

  • central
  • covering sac
  • firm mass
  • NEC uncommon
192
Q

What anomalies is gastroschisis commonly associated with?

A

Intestinal atresia
Malrotation
Cryptorchidism

193
Q

What anomalies is omphalocele commonly associated with?

A

Trisomy
Cardiac defects
BW syndrome
Bladder extrophy

194
Q

Which birth defect has a better prognosis: gastroschisis or omphalocele?

A

Gastroschisis

195
Q

How do you manage gastroschisis clincally?

A

Silo bag to reduce gut into abdominal cavity

196
Q

When is nephrogenesis complete?

A

34-36w

197
Q

What is the renal function in the neonate’s purpose?

A

Optimising retention > excretion

Low GFR which increases over the 1st year of life

198
Q

What is CAKUT?

A

Congenital abnormalities of the kidney and urinary tract?

199
Q

What is the differential diagnosis for unilateral hydronephrosis?

A

Pelviureteric junction obstruction

Vesicoureteric junction obstructioon

200
Q

What is the differential diagnosis for bilateral hydronephrosis?

A

Bladder neck obstruction

Posterior urethral valves

201
Q

What is the most common urinary tract abnormality in the neonate?

A

Unilateral hydronephrosis

202
Q

Discuss polycystic kidney disease

A 
Autosomal dominant
- asx in childhood
- presents in late adulthood w/ renal failure
Autosomal recessive
- neonatal period
203
Q

What is the most common electrolyte abnormality encountered in newborns?

A

Dysnatremias

204
Q

What are dysnatremias in the newborn assoc with?

A

Significant morbidity

Poor long-term neurological outcome

205
Q

Define hyponatremia

A

Na <130mmol/l

206
Q

Differentiate between the two forms of hypoatremia

A

Too much water

  • BP stable
  • normal skin turgor
  • weight stable

Too little salt

  • BP decrease
  • poor skin turgor
  • weight decreased
207
Q

What are causes of too much water in hyponatremia?

A

Excess fluid administration
Cardiac failure
Renal failure
SIADH

208
Q

What are causes of too little salt in hyponatremia?

A

Extra-renal salt loss

Congenital adrenal hyperplasia

209
Q

What is the most common reason for hypernatremia?

A

Water deficit

Other: excess salt intake

210
Q

What is the major cause of water deficit in hypernatremia in newborns?

A

Transepidermal fluid losses

Other causes: poor feeding

211
Q

Why would a newborn have excess salt intake?

A

Formula feeding

212
Q

Discuss the management of hypernatremia in the newborn

A

Calculate total body water deficit and replace over next 24-48hrs -> reduction of Na by 0.5-1mmol/l/hr

213
Q

What risk is there if the sodium level is reduced too quickly in hypernatremia?

A

Cerebral oedema

214
Q

Define hyperkalemia

A

K+ > 6.5mmol/l

215
Q

What is the major risk of hyperkalemia?

A

Cardiac arrythmias

216
Q

Name causes of hyperkalemia in the newborn

A

Renal impairment
Excess potassium administration
Congenital adrenal hyperplasia
Incorrect test (haemolysed specimen)

217
Q

What is a clinical feature of hypocalcemia in the newborn?

A

May present with neonatal seizures

218
Q

Name causes of hypocalcemia

A

Maternal

  • hypothyroidism
  • vitamin D deficiency
  • diabetic

Infant

  • HIE
  • DiGeorge syndrome
219
Q

Other than hypocalcemia, what other electrolyte abnormality is often found in DiGeorge syndrome?

A

Hypomagnesemia

220
Q

What is the most common bacterial infection in the neonatal period?

A

UTI

Boys > girls

221
Q

Name risk factors for UTI in the newborn

A

Prem
Caucasian
CAKUT

222
Q

Name common causative organisms of neonatal UTIs

A

Ecoli
Enterobacter spp
Klebsiella pneumonia

223
Q

How do neonates with UTI present clinically?

A 
Poor feeding
Poor weight gain
Pyrexia
Vomiting
Diarrhoea
Lethargy
Irritability
Jaundice
224
Q

What should you always look for in a neonate with sepsis?

A

UTI

225
Q

What should you always look for in a patient with neonatal jaundice?

A

UTI

226
Q

How do you diagnose UTI in the newborn?

A
  1. Blood investigations
    - CRP
    - FBC
  2. Urine specimen
  3. Urine culture
227
Q

Discuss the management of UTI in newborn

A

Empirical therapy until culture results come back
IV broad spectrum antibiotic
Avoid nephrotoxic ABs
Renal U/S

228
Q

Define acute kidney injury in the newborn

A

Abrupt decline in ability to clear waste products and to maintain fluid and electrolyte homeostasis

229
Q

What is the KIDGO definition of AKI?

A

Incr in serum creatinine by 0.3g/dl
Incr in serum creatinine by 1.5x baseline
Urine output <0.5ml/kg/hr for 6hrs

230
Q

What is the RIFLE criteria?

A

Used to classify kidney injury

Risk of renal dysfunction (creatinine incr by 150%)
Injury to the kidney (creatinine incr by 200%)
Failure of kidney function (creatinine incr by 300%)
Loss of kidney function (loss of function >4 weeks)
End stage kidney disease (loss of function >3months)

231
Q

Name causes of acute kidney injury in the newborn

A

Prerenal = decrease in renal blood flow to glomeruli

  • hypovolemia
  • decr CO
  • blood loos
  • HIE

Renal

  • uncorrected prerenal -> ATN
  • congenital renal abnormality
  • renal vv thrombosis
  • nephrotoxins
  • infection

Post renal = obstructive

  • congenital obstructive uropathy
  • neurogenic bladder
232
Q

How should you evaluate a newborn you suspect has AKI?

A 
History
Examination
Lab
- UCE 
- CMP
- urine analysis
Imaging
- U/S 
- doppler
233
Q

How would you manage a newborn with AKI?

A

Supportive

  • maintain fluid balance
  • correct electrolytes
  • correct acidosis
  • treat hypertension
  • nutritional (avoid potassium, phosphates)
234
Q

Which type of undescended testis is more common: unilateral or bilateral?

A

Unilateral (2/3) > bilateral (1/3)

235
Q

What is an assoc abnormality of bilateral undescended testes?

A

Hypospadia

236
Q

When should you refer undescended testes for surgery?

A

If undescended by 6months (gonadotropin surge at 3months)

Before 2 years

237
Q

What are complications of an inguinal hernia in the newborn?

A

Strangulation
Incarceration

Need to correct surgically!

238
Q

Discuss treatment of a hydrocele

A

No intervention

Resolves spontaneously

239
Q

Give the components of a hypospadia

A

Ventral urethral meatus
Hooded foreskin
Chordee
Ventral curvature

240
Q

Provide the most common reason for congenital adrenal hyperplasia and discuss the features

A

21-hydroxylase deficiency -> 17-hydroxyprogesterone increase

  • decreased aldosterone
  • hyponatremia
  • hyperkalemia
  • decr cortisol in adrenal crisis with hypoglycemia
  • incr androgen production (female virilisation)
241
Q

Discuss the presentation of a newborn in a salt-losing adrenal crisis

A 
1-3w of age
Vomiting
Weight loss
Circulatory collapse
Hypoglycemia
Hyponatremia
Hyperkalemia
242
Q

What are the effects of poor maternal glycemic control during pregnancy?

A 
1st trimester 
- major birth defects
- spontaneous abortions
2nd + 3rd trimester
- hyperinsulinemia
- macrosomia
243
Q

Name congenital abnormalities of diabetic embryopathy in maternal type I diabetes

A 
CNS
- neural tube defects
- holoprosencephaly
Cardiac
- hypertrophic interventricular septum
- ASD
- VSD
- TGA
- coarctation
Renal
- agenesis
- dysplasia
- hydronephrosis
GIT
- small left colon
- bowel atresia
- anorectal malformation
Skeletal
- caudal regression syndrome 
Facial
- midline cleft
- microsomia
- microtia/anotia
244
Q

Name delivery complications of infants to diabetic mothers

A 
Shoulder dystocia
Brachial plexus injury
Humerus fracture
Clavicle fracture
Visceral trauma
245
Q

Name postnatal complications of infants to diabetic mothers

A

Prem birth -> poor feeding
Hyperinsulinism -> hypoglycemia
Hyperinsulinism -> low cortisol -> decr surfactant -> RDS
Macrosomia -> C/S -> transient tachypnoea of the newborn
Polycythaemia -> stroke, renal vv thrombosis, seizures, jaundice
Transient hypoparathyroidism -> hypocalcemia, hypomagnesemia
Excess RBC precursors in bone marrow -> thrombocytopenia

246
Q

Discuss the management of infants to diabetic mothers

A

Prevent hypoglycemia
Identify complications
Screen for congenital abnormalities

247
Q

What is normal physiological weight loss after birth and when will it be regained?

A

7-10%

Regained by 10-14 days postdelivery

248
Q

Name causes of poor feeding

A 
Inadequate breastfeeding
Hypoglycemia
Infection
Electrolyte abnormalities
Inborn error of metabolism
249
Q

What is a normoglycemia in a neonate?

A

≥ 2.6mmol/l on 2 occasions

250
Q

Name risk factors for hypoglycemia in the neonate

A

SGA
IUGR
LGA
IDM

251
Q

What is a differential diagnosis of delayed passing of meconium in the neonate?

A

Imperforated anus
Meconium ileus
Intestinal atresia

252
Q

What is a differential diagnosis for delayed passing of urine in the neonate?

A

Dehydration
Posterior urethral valves
Renal dysgenesis
Renal agenesis

253
Q

Name causes of apnoea attacks in the neonate

A 
Seizure
Hypoglycemia
Sepsis
IVH
Airway obstruction
Premature resp centre
254
Q

Is it normal for neonates to become cyanosed around the mouth in the first few days of life?

A 
Yes esp during feeds
BUT must exclude
- congenital CHD
- polycythaemia
- infection
255
Q

Why do you need to manage nasal discharge in the neonate ASAP?

A

Obligatory nose breather

Use saline drops and suction!

256
Q

Name causes of regurgitation in neonates

A 
Excessive volume feeds
Not burped
Feeding horizontally
Lying down immediately after feed
Poor LES tone
257
Q

Name causes of pallor in the neonate

A

Twin-to-twin transfusion
Foetomaterno haemorrhage
Abruptio placenta

258
Q

How do you manage a neonatal conjunctivitis that is only sticky?

A

Clean with sterile water

259
Q

How do you manage a neonatal conjunctivitis that appears on day 3-5?

A

Gonococcal infection

3rd generation cephalosporin IVI if severe

260
Q

How do you manage a neonatal conjunctivitis that appears on day 5 - 14?

A

Chlamydia infection

Oral erythromycin

261
Q

What is the common cause of omphalitis?

A

Staph/strep infection

262
Q

How do you treat an umbilical granuloma?

A

Silver nitrate sticks to the granuloma for 3 days

263
Q

How do you differentiate fungal from contact dermatitis?

A

Contact - does not involve the skin folds

FUngal - red papules, involves skin folds, does not heal after conventional management

264
Q

How do you prevent nappy dermatitis?

A

Change immediately after wet/soiled

Clean skin with warm water

265
Q

How do you manage nappy dermatitis?

A

Keep skin dry and clean
Leave bottom exposed to air
Wash skin with warm water
Use mild soap only to clean after stools

If fungal - apply antifungal 4x day OR after each bottom cleaning for stools

266
Q

Is oral thrush common in neonates or do you suspect something more?

A

Common in neonates

NOT indicative of secondary immunodeficiency

267
Q

When do you administer VZIG to the newborn?

A

Mother develops vesicle 5 days before delivery - 2 days after delivery

268
Q

How do you treat neonatal chicken pox?

A

IV acyclovir

269
Q

What types of neonatal HSV are there?

A

Disseminated
Encephalitis
Localised

270
Q

How do you treat neonatal HSV?

A

IV acyclovir

271
Q

How do you treat SSS syndrome?

A

IV ABs

272
Q

What is the cause of epidermolysis bullosa?

A

Genetic in areas of trauma/friction

273
Q

How do you treat epidermolysis bullosa?

A

Prevent dehydration
Prevent infection
Analgesia

274
Q

How do you differentiate jittery movements from a seizure in the newborn?

A

Jittery movements -> stop when holding limb

Seizure -> continues when holding limb

275
Q

What is the gold standard of diagnosing seizure in newborn?

A

Video EEG

276
Q

Name causes of seizures in the newborn

A 
Hypoglycemia
Electrolyte abnormality
Meningitis
Drug withdrawal
Hypoxic brain injury
IVH
ICH
277
Q

Discuss treatment of seizures in the newborn

A

Phenobarbitone
Levetiracetam
BZDs

278
Q

Name symptoms and signs of neonatal hypoglycemia

A 
Jittery
Irritable
High pitched cry
Lethargy
Decr LOC
COma
Hypotonia
Apnoea
Seizures
279
Q

Name risk factors for neonatal hypoglycemia

A

Antenatal

  • maternal diabetes
  • maternal obesity
  • maternal beta blockers

Neonatal

  • IUGR
  • prem
  • LGA
  • sepsis
  • iatrogenic (no feeds/fluids)
  • polycythaemia
  • HIE
  • hypothermia
  • rhesus disease
280
Q

Name causes of persistent hypoglycemia in the neonate

A 
Hyperinsulinism
- IDM
- BW sydrome
- insulinoma
Endocrine deficiency
- panhypopituitarism
- GH deficiency
- adrenal deficiency
Inborn error of metabolism
281
Q

What investigations will you order in a neonate with persistent hypoglycemia?

A 
Serum glucose
Serum insulin
Serum cortisol
Serum GH
Adrenal gland sonar
Brain imaging
IEM investigations
282
Q

How do you manage neonatal hypoglycemia?

A

Early feeding to prevent
Asx/glucose betw 1.4-2.5mmol/l = enteral feeds
Sx/glucose <1.4mmol/l = IV 10% dextrose bolus followed by continuous infection
Treat the underlying cause

283
Q

What are the WHO recommendations for optimal infant feeding?

A
  1. Exclusive breastfeeding for 6months -> complementary feeding from 6 months up to 2yo
284
Q

What are exceptions that are allowed in exclusive breastfeeding?

A

Oral rehydration solution
Vitamin/minerals
Medication

285
Q

Give reasons for exclusively breastfeeding for 6 months

A
  1. Provides all nutritional needs
  2. Benefits increase with duration
  3. Risk of death from diarrhoea reduced
  4. Risk of resp illness reduced
  5. PMTCT
286
Q

What does breastmilk contain?

A 
Water
Fat
Carbohdydrates
Vitamins
Minerals
Oligosaccharides
Whey proteins
WBC
Immunoglobulins
Epidermal GFs
287
Q

What is the role of whey proteins in breastmilk?

A

Kills bacteria, viruses and fungi

288
Q

What are the benefits of breastfeeding for the mother?

A 
Short term
Decr risk of PPH
Accelerate prepregnancy weight recovery
Bonding
Free, convenient and readily available

Long term
Delays return of fertility
Decr breast and ovarian Ca risk
Decr risk of DM II, HT, CV disease and hyperlipidemia

289
Q

What are the benefits of breastfeeding for the infant?

A 
Short term
Ideal nutrition
Healthy weight gain
Easily digested -> less constipation
Maintain GIT integrity
Infection protection
Decr SIDS risk
Long term
Decr immunological diseses
Better cognitive function
Decr obesity
Decr adulthood CV risk
Decr childhood leukemia risk
290
Q

Name infant conditions where breastmilk substitutes are required

A

Galactosemia
Maple syrup urine disease
Phenylketonuria
Hypoglycemia

291
Q

Name maternal conditions where breastmilk substitutes are required

A 
Severe illness
Medication
Substance abuse
HIV infection w/ high viral load on 2nd line ART
Hepatitis C
292
Q

What medications contraindicate breastfeeding?

A

Radioactive iodine
Cytotoxic chemotherapy
Sedatives

293
Q

How many calories does a preterm infant require and what is the target feed volume?

A

120-150kcal/kg/day

160-180ml/kg/day

294
Q

How many calories does a term infant require and what is the target feed volume?

A

100kcal/kg/day

150ml/kg/day

295
Q

Name an example of breastmilk fortifier

A

FM85

296
Q

What is the name of the multivitamin given to preterm infants?

A

Vidaylin

297
Q

Why is phosphate supplementation necessary in the preterm infant?

A

Need large amount of phosphate to grow -> don’t have -> osteopenia -> metabolic bone disease of prematurity

298
Q

What factors promote lactation?

A

Frequent feeding
Empty breasts
Calm environment
Medication (metaclopromide, sulpiride)

299
Q

What factors inhibit lactation?

A

Breasts engorged
Stressful environment
Pain, illness
Medication (oestrogen, bromocriptine)

300
Q

When is prolactin production highest?

A

At night

301
Q

Give examples of breastfeeding positions

A 
Cradle
Cross cradle
Back lying
Football
Australian hold
Inverted side lying
Side lying cradle
Side lying
302
Q

Give signs of adequate milk intake

A

Good weight gain
Good urine output
Change from meconium to transitional stools by day 4

303
Q

Name causes of inadequate milk intake

A 
Delayed initiation of breastfeeding
Poor attachment
Infrequent/short feeds
Bottle/dummy use
Giving other liquids/soft foods
Not feeding at night
304
Q

How should HIV mothers breastfeed when experiencing breast pain?

A

Should only feed using the unaffected breast

If both breasts - consider pasteurizing if no alternative feeding available

305
Q

Name signs of breast candida infection

A 
Mother
- sore nipples
- red/flaky rash on areola
Infant
- oral thrush
- may refuse feeds
306
Q

How do you treat a breast candida infection?

A

Nystatin suspension QID x7d after breastfeeds

Nystatin cream QID x7d after breastfeeds

307
Q

Explain the process of bilirubin synthesis

A

Hemolysis of RBC -> heme + globin
Free heme binds to haptoglobin -> low s haptoglobin
Heme is oxidated via heme oxygenase -> biliverdin
Biliverdin catalysed by biliverdin reductase -> bilirubin
UC, fat soluble bilirubin binds to albumin -> transported to liver -> dissociates with albumin to enter liver
UCB binds to intracellular proteins -> transported to endoplasmic reticulum -> conjugated to glucoronic acid via UGT -> water soluble
Water soluble CB -> excreted into bile caniculus -> intestine -> hydrolysed via beta glucoronidase to UCB -> goes back to liver

308
Q

What happens to the bilirubin excreted into the intestine?

A

25% reabsorbed as UCB
10% excreted unchanged as CB
65% -> urobilinoids -> stercobilinogen (majority) + urobilinogen

309
Q

What colour is urobilinogen?

A

Colourless!

310
Q

Categorise the causes of unconjugated jaundice

A

Increased production
Decreased hepatic uptake/conjugation
Increased enterohepatic circulation

311
Q

Categorise the causes of conjugated jaundice

A

Hepatocellular dysfunction

Biliary obstruction

312
Q

What is the cause of physiological jaundice

A

High hb due to hypoxic environment no longer needed and RBC lifespan shorter -> increased RBC breakdown -> intravascular haemolysis

313
Q

Name intravascular causes of haemolysis in the neonate

A 
Physiological
Allo-immune
- ABO incompatibility
- Rh incompatibility
- minor antigen incompatibilities
Non-immune
- RBC membrane defects
- RBC Hb defects
- RBC enzyme defects
314
Q

Name extravascular causes of haemolysis in the neonate

A

Cephalhaematoma
Bruising
Subaponeurotic bleed
IVH

315
Q

How do we establish whether the haemolysis is allo-immune or non-immune?

A

Direct Coombs test

316
Q

What is Coombs reagent?

A

Antihuman antibodies

317
Q

How does a Coombs test test positive?

A

Foetal blood with maternal antibodies (Rh, ABO) + Coombs reagent -> agglutination -> positive

318
Q

Which blood group has IgG antibodies?

A

O

319
Q

Why does ABO incompatibility not occur in AB babies?

A

Mother has to be O blood group -> cannot have an AB baby

320
Q

Why does ABO incompatibility not occur in A,B or AB mothers?

A

The antibodies are IgM

321
Q

Name RBC membrane defects in the neonate

A

Hereditary spherocytosis

Hereditary elliptocytosis

322
Q

Name RBC haemoglobin defects in the neonate

A

A-thalassemia

323
Q

Name RBC enzyme defects in the neonate

A

Glucose-6-phosphate dehydrogenate deficiency

Pyruvate kinase deficiency

324
Q

How can you diagnose an RBC membrane defect?

A

Assess the osmotic fragility of the RBC

325
Q

Why do newborns not present with beta thalassemia or sickle cell disease?

A

Newborns have haemoglobin f not haemoglobin a

326
Q

What investigations should you do to diagnose intravascular haemolysis and what results do you expect?

A 
Hb -> decreasing/low
Reticulocytes -> high
LDH -> high
Haptoglobin -> low
RBC fragments on smear
327
Q

Name causes of decreased hepatic uptake and conjugation in the neonate

A

Physiological:
Decreased net hepatic uptake
Immature liver enzymes

Pathological:
Breast milk jaundice
Hypothyroidism
Gilbert's disease
Crigler-Najjar syndrome
328
Q

Name causes of increased enterohepatic circulation in the neonate

A

Breastfeeding jaundice
Meconium ileus
Hirschprung disease
Intestinal atresia

329
Q

Why is breastfeeding jaundice a misnomer?

A

They are NOT feeding well -> poor intake -> delayed stool passage -> incr enterohepatic circulation

330
Q

What investigations are required in unconjugated neonatal jaundice?

A

TSB
Direct bilirubin

If indirect bilirubin high

  • Hb
  • reticulocyte
  • LDH
  • haptoglobin
  • RBC smear

If intravascular haemolysis
- direct Coombs test

+ Coombs

  • ABO
  • Rh
  • Coombs
  • RBC membrane defects
  • RBC Hb defects
  • RBC enzyme defects
331
Q

What does a biliruin rising >17umol/l/hr indicate?

A

Severe haemolysis -> pathological jaundice

332
Q

Compare the difference between Rh and ABO incomptaibility

A 
Rh:
Mom Rh -
Dad Rh +
Baby Rh +
Sensitization yes
ABO:
Mom O
Dad A,B or AB
Baby A or B
Sensitization no
333
Q

Discuss the management of unconjugated jaundice

A

Phototherapy
Exchange transfusion
IVIG

334
Q

Name dangers of unconjugated jaundice?

A

Bilirubin neurotoxicity

335
Q

How can bilirubin neurotoxicity occur?

A

UCB -> water soluble -> crosses BBB

CB -> BBB disrupted -> crosses BBB

336
Q

Name signs of acute bilirubin encephalopathy

A

Early

  • hypotonia
  • lethargy
  • poor suck
  • high pitched cry

Intermediate

  • irritability
  • fever
  • convulsions
  • w/wo opisthotonus

Advanced

  • severe opisthotonus
  • apnoea
  • convulsions
  • coma
  • death
337
Q

Name signs of chronic bilirubin encephalopathy

A 
Athetosis
Sensorineural deafness
Dental dysplasia
Teeth discolouration
Intellectual deficits
338
Q

What would MRI of the brain show in chronic bilirubin encephalopathy?

A

Increased signal intensity

  • basal ganglia
  • cranial nn nuclei
  • cerebellar nuclei
339
Q

What would autopsy of the brain show in chronic bilirubin encephalopathy?

A

Kernicterus

340
Q

Name indications for phototherapy

A 
Absolute
- phototherapy chart
Prophylactic
- ELBW
- extravascular blood collections
341
Q

What is a contraindication for phototherapy?

A

Conjugated jaundice -> bronze baby syndrome (iireversible!)

342
Q

Explain how phototherapy works

A

Uses blue green light to convert UCB into water soluble isomers
Structural isomers excreted into bile + urine
Configurational isomers excreted into bile

343
Q

What should the blue light spectrum be in phototherapy?

A

425-475nm

344
Q

What should the irradiance be in phototherapy?

A

10-30uW/cm2/nm

345
Q

How far should the light be from the infant?

A

20cm

346
Q

Give examples of types of phototherapy lights

A

Fluorescent tubes
LED lights
Halogen bulbs

347
Q

Name complications of phototherapy

A 
Impaired bonding
Incr water loss
Watery stools
Maculopapular skin rash
Lethargy
Potential for retinal damage
348
Q

What do you do using the phototherapy chart if the time is after 120h?

A

Plateus at 120h -> use the threshold measurement

349
Q

Name indications for exchange transfusion

A
  1. TSB >85umol/L above threshold at presentation
  2. TSB remains above EL despite 6hrs of intensive phototherapy
  3. Any signs of acute bilirubin encephalopathy
  4. TSB rising >17umol/l/hr despite intensive phototherapy
350
Q

Why do we perform exchange transfusion when TSB>85umol/L

A

We do not expect the TSB to fall below the threshold within 6hours

351
Q

How does exchange transfusion aid in elevated bilirubin?

A

Removes maternal antibodies
Replaces 87% of blood with new blood
Removes bilirubin from plasma

352
Q

What blood is used for exchange transfusion?

A

Fresh, irradiated, reconstituted whole blood from packed RBC and FFP
Contains no platelets

353
Q

How do you perform an exchange transfusion?

A 
Use umbilical venous catheter
Exchange double the BV 
- term 160ml/kg
- preterm 180mk/g
Aliquots of 5ml/kg max 20ml every 2min
354
Q

Name complications of exchange transfusions

A 
Hypocalcemia
Hypoglycemia
Hyperkalemia
Vasospasm
Arrythmias
Bleeding
Infections
Graft-vs-host disease
Hypothermia
Volume overload
355
Q

Why does hypocalcemia occur in exchange transfusion?

A

Citrate in the blood bag to prevent clotting

356
Q

Why does hypoglycemia occur in exchange transfusion?

A

RBC use glucose

Usually feeds stopped during transfusion

357
Q

Why does hyperkalemia occur in exchange transfusion?

A

RBC lyse due to mechanical stressors

358
Q

Why do vasospasms occur in exchange transfusion?

A

Catheter related

359
Q

Why do arrythmias occur in exchange transfusion?

A

Catheter related

360
Q

Why does bleeding occur in exchange transfusions?

A

Low platelets

361
Q

Why do infections occur in exchange transfusion?

A

Blood not properly screened

362
Q

Why does graft-vs-host disease occur in exchange transfusion?

A

Immune reaction if blood not irradiated

363
Q

Why does hypothermia occur in exchange transfusion?

A

Blood not warmed prior to use

364
Q

Why does volume overload occur in exchange transfusion?

A

More blood transfused than removed

365
Q

Explain how IVIG works to reduce the rate of haemolysis in infants with alloimmune haemolysis

A

Binds to RE receptors and blocks them -> RE cell unable to bind to antibody on RBC -> RBC does not lyse

366
Q

What is conjugated jaundice also known as?

A

Cholestatic jaundice

367
Q

When do you diagnose conjugated hyperbilirubinaemia?

A

CB > 20% TSB

368
Q

What colour is conjugated bilirubin in the urine?

A

Dark urine

369
Q

What lab investigations indicate hepatocellular dysfunction?

A

Incr AST/ALT

370
Q

Name causes of hepatocellular dysfunction in neonates

A

Infections:

  • TORCHES
  • HIV
  • parvovirus B19
  • UTI
  • sepsis

Metabolic/genetic:

  • galactosemia
  • tyrosenemia
  • Niemann Pick disease type C
  • alpha 1 antitrypsin deficiency
  • Gauchers
  • CF

Endocrine:

  • hypothyroidism
  • hypopituitarism

Allo-immune:
- gestational alloimmune liver disease (GALD)

Toxins:

  • drugs eg INH
  • parenteral nutrition

Misc:
- idiopathic neonatal hepatitis

371
Q

What lab investigations indicate biliary obstruction?

A

Incr ALP/GGT

372
Q

Name causes of biliary obstruction

A

Extrahepatic:

  • biliary atresia
  • choledocal cyst
  • choledocal stones
  • tumour/mass
  • neonatal sclerosing cholangitis
  • inspissated bile syndrome

Intrahepatic:

  • alagille syndrome
  • intrahepatic biliary atresia
373
Q

Name complications of jaundice found on examination

A
  1. Encephalopathy
    - raised ammonia (LF)
    - hypoglycemia (LF)
  2. Cataracts
    - metabolic disease
    - congenital infection
  3. FTT
    - advanced liver disease
    - sepsis
    - metabolic disease
  4. Bleeding
    - coagulopathy (LF)
  5. Firm hepatomegaly
    - biliary atresia
  6. Splenomegaly
    - storage disease
  7. Portal hypertension
    - HSM
    - ascites
    - caput medusa
  8. Acholic stool/dark urine
    - obstructive jaundice
374
Q

Discuss management of conjugated jaundice

A 
Give ADEK vitamins
MCT oil
Adequate calories
Ursodeoxycholic acid 
Avoid lactose in galactosemia
Surgical
375
Q

Why do we give ursodeoxycholic acid?

A

Promotes bile excretion

376
Q

What investigations are required in conjugated neonatal jaundice?

A

TSB and direct bilirubin
Direct >20% TSB = conjugated
Test liver enzymes
Raised ALP/GGT = obstructive = abdominal U/S + urine dipstix
Raised AST/ALT = hepatocellular = multiple investigations
Last resort -> liver biopsy

377
Q

What investigations should you do for hepatocellular causes of conjugated hyperbilirubinaemia?

A 
Infections
GALT
A1AT
Thyroid function
Serum ferritin
Sweat test
Fecal elastase
378
Q

Which investigations are used to assess liver function?

A 
INR
PTT
Albumin
Ammonia
Glucose
379
Q

When can NVP be stopped in the infant during PMTCT and under what condition?

A

Stop at 12 weeks if mother’s VL <1000c/ml

380
Q

What do we give as low risk PMTCT prophylaxis?

A

NVP daily x 6w

381
Q

What do we give as high risk PMTCT prophylaxis?

A

Breastfeeding: NVP 12w and AZT 6w

Formula fed: NVP 6w and AZT 6w

382
Q

What do you put the infant on in PMTCT after stopping the ARVs and when do you stop this medication?

A

Cotrimoxazole

Stop when
- PCR - >6w after breastfeeding cessation
AND
- infant clinically HIV negative

383
Q

What is the prophylactic dose of zidovudine according to weight?

A 
<2kg = 4mg/kg/dose
2.0 - 2.49kg = 10mg
2.5 - 2.99kg = 15mg
3.0-5.9kg = 60mg
6 - 7.9kg = 90mg
8-13.9kg = 120mg
384
Q

What is the prophylactic dose of nevirapine according to weight

A 
2.0-2.49kg =10mg
>2.5kg = 15mg
>6w - 6m =20mg
6-9m = 30mg
>9m = 40mg
385
Q

What is the prophylactic dose of cotrimoxazole syrup according to weight

A

2.5-5kg = 2.5ml

5 - 13.9kg = 5ml

386
Q

Which infants are at higher risk of developing anemia on AZT?

A

Premature

Malnourished

387
Q

When do we test the infant in PMTCT?

A 
Birth PCR
10w PCR
6m PCR (HIV exposed)
18m RAPID/ELISA for ALL children
6w postcessation of BF
Any time symptomatic
388
Q

How common is cleft lip/palate?

A

1/1000 live births

389
Q

What is the cause of cleft lip/palate?

A

In the 8th week of gestation, failure of fusion of medial and lateral nasal processes (lip) and maxillary processes (palate)

390
Q

Discuss management of cleft lip/palate

A

Surgical repair of lip at 3 months

Surgical repair of palate at 6-12m

391
Q

Name complications of cleft lip/palate

A 
Short term
- feeding difficulties
Long term
- OME 
- hearing impairment
- speech difficulties
- facial growth issues
- orthodontic issues
392
Q

How can you assist with feeding difficulties in cleft lip/palate?

A

Special teats

Dental plate

393
Q

What is Pierre Robin sequence?

A

Autosomal recessive
Mandibular hypoplasia between 7th and 11th week of gestation -> tongue high in oral cavity -> inverted U shaped cleft

Triad:

  1. Micrognathia
  2. Retroglossoptosis
  3. Posterior palatal defect
394
Q

Name complications of Pierre Robin sequence

A

Resp obstruction
Hypoxia
Pulmonary hypertension
Cor pulmonale

395
Q

What other genetic form of Pierre Robin sequence has been reported?

A

X linked variant assoc with cardiac malformations and club feet

396
Q

Discuss management of Pierre Robin sequence

A

Avoid tongue obstruction
- nurse prone
- CPAP vs NPT
Surgical repair of posterior palate at 1yo

397
Q

Discuss management of choanal atresia

A

Oral airway/intubate

Surgical correction asap

398
Q

Name causes of respiratory distress in newborns

A 
Birth asphyxia
Sepsis
Transient tachypnoea of the newborn
Meconium aspiration syndrome
Respiratory distress syndrome
Congenital diaphragmatic hernia
Pneumonia
Pneumothorax
399
Q

Explain the mechanism of transient tachypnoea of the newborn

A

Lower [ ] of circulating catecholamines -> reduced reabsorption of alveolar fluid via sodium channels in the lung epithelium -> TTN

400
Q

When is TTN most common?

A

C/S

401
Q

Discuss management of TTN

A

Oxygen
Nasogastric feeding
CPAP

402
Q

Name constituents of meconium

A

Intestinal epithelial cells
Lanugo
Mucus
Intestinal secretions (bile)

403
Q

Why does meconium aspiration mainly affect infants born at term and post term?

A

Meconium is rarely found in amniotic fluid <34-36w GA

404
Q

Name causes of meconium aspiration

A 
Any cause of intrauterine stress
Placental insufficiency
Hypoxia
Maternal hypertension
Preeclampsia
Oligohydramnios
Maternal drug abuse
405
Q

Name complications of meconium aspiration

A 
Airway obstruction
Surfactant dysfunction
Chemical pneumonitis
Pneumothorax
PPHN
406
Q

Discuss management of meconium aspiration

A 
Mechanical ventilation
Systemic vasoconstrictors (inotropes)
Sildenafil
Nitric oxide
NO CPAP
407
Q

Name risk factors for congenital pneumonia

A 
PROM
Maternal fever
Maternal tachycardia
Chorioamnionitis
Maternal UTI
Maternal avginitis
Spontaneous preterm labour
408
Q

What is the most common cause of congenital pneumonia?

A

GBS

409
Q

Discuss treatment of congenital pneumonia

A

Penicillin

Ventilation

410
Q

How should all infants with respiratory distress be managed?

A

Start on broad spectrum ABs until results known

  • blood culture
  • CRP
  • FBC
  • lumbar puncture
411
Q

What is a Bochdalek diaphragmatic hernia?

A

Hernia of bowel through the posterolateral foramen of the diaphragm

412
Q

What is a Morgagni diaphragmatic hernia?

A

Hernia of bowel through central anterior of diaphragm

413
Q

Name signs of diaphragmatic hernia

A

Resp distress
Chest assymetry
Apex beat displaced
Scaphoid abdomen

414
Q

Discuss management of a congenital diaphragmatic hernia

A 
Intubate and ventilate from birth 
Pass NGT and suction
Stabilise
Early PN
Nitric oxide/sildenafil for PPHN
Surgical repair
NO CPAP
415
Q

Describe the ventilation required in the management of a congenital diaphragmatic hernia

A

Gentle ventilation, allowing permissive hypercapnia, i.e. PaCO2 > 60 mmHg (8kPa) but maintaining
pH >7.25

416
Q

Name signs of pneumothorax

A

Chest assymetry
Unilateral decr breath sounds
Unilateral decr chest movements
Transillumination of chest

417
Q

What are the complications of oxygen therapy in the infant

A 
Retinopathy of prematurity
NEC
IVH
Bronchopulmonary dysplasia
Chronic lung disease
418
Q

Discuss management of PPHN

A 
Oxygen
Mechanical ventilation
Surfactant therapy
Pulmonary vasodilator
High frequency oscillatory ventilation
ECMO
419
Q

Categorise sepsis and differentiate between these categories

A 
Early onset sepsis
- transplacental or genital tract
- 48-72hrs
Late onset sepsis
- nosocomial, community and environment
- >72hrs
420
Q

Name examples of organisms involved in early onset sepsis

A

GBS
Gram -
Listeria
Staph aureus

421
Q

Name examples of organisms involved in late onset sepsis

A 
GBS
Gram -
Staph aureus
CoNS
Enterococcus
Fungal
422
Q

Define neonatal sepsis

A

Presence of infections involving bloodstream, urine, cerebrospinal, peritoneal structures and/or sterile tissues during first 28 days of life

423
Q

The earlier the presentation of sepsis post delivery, the ____ the clinical presentation and outcome?

A

Worse

424
Q

What is the most common source of postnatal infections in infants admitted in hospitals?

A

Hand contamination

425
Q

Name maternal risk factors for early onset neonatal sepsis

A 
Premature labour 
PROM>18hrs
Traumatic delivery
Chorioamnionitis
Maternal fever >38
MSL
Low SES
GBS bacteruria
426
Q

Name neonatal risk factors for early onset neonatal sepsis

A 
Prematurity
Male
Low apgar
Hypothermia
Foetal distress
427
Q

Name neonatal risk factors for late onset neonatal sepsis

A 
GA:weight inverted
Central catheters
Ventilation
ABs
PN
Gastric acid suppression therapy
Skin damage (tape, probe)
428
Q

Name clinical signs and symptoms of infection in the neonate

A

Skin

  • omphalitis
  • petechiae
  • poor circulation
  • jaundice

GIT

  • intolerance
  • vomiting
  • diarrhoea
  • abdominal distension
  • jaundice

Cardiopulmonary

  • resp distress
  • poor capillary refill
  • tachy/bradycardia

Metabolic

  • hypoglycemia
  • hyperglycemia
  • metabolic aidosis
429
Q

What is the gold standard for diagnosing neonatal infection?

A

Blood/urine/CSF culture using strict aseptic technique

430
Q

What should CSF be sent for?

A

Chemistry
Microscopy
Culture
Latex agglutination

431
Q

What are signs of NEC on AXR?

A

Dilated loops of bowel
Pneumatosis intestinalis
No air in rectum

432
Q

What I/T ratio suggests infection?

A

> 0.2

433
Q

Why is there a delayed rise in CRP for newborns?

A

Time needed for release by liver in response to IL6

434
Q

What is initial empiric treatment for early onset sepsis?

A

Pen G
Ampicillin
Amikacin

435
Q

What is initial empiric treatment for late onset sepsis?

A

Cephallosporins
Vancomycin
Carbapenems
Antifungals

436
Q

Which drugs are reserved for gram - meningitis?

A

3rd/4th gen cephalosporin

437
Q

What is specific anti-RSV prophylaxis?

A

Palivizumab

438
Q

Name viruses that cause congenital infections

A 
CMV
Rubella
HSV
VZV
PB19
HBV
HCV
HIV
Enterovirus
HPV
439
Q

Name bacteria that cause congenital infections

A

Toxoplasma gondii
Treponema pallidum
Mycobacterium tuberculosis
Plasmodium

440
Q

Differentiate between a primary and secondary maternal infection

A

Primary - acquired in pregnancy + most likely to cause disease
Secondary - reactivation of prev infection + less likely to cause disease

441
Q

Name clinical signs of CMV

A 
Prematurity
Rash
IUGR
Jaundice
HSM
Microcephaly
Seizures
Hypotonia
Lethargy
Chorioretinitis
Intracranial calcifications
Sensorineural hearing loss
442
Q

How do you diagnose CMV?

A

Mother

  • IgG
  • IgM
  • CMV DNA PCR

Infant

  • IgM
  • CMV viral DNA
443
Q

How do you treat congenital CMV?

A

Oral valganciclovir for 6 months

IV ganciclovir for 6w at 6mg/kg/dose

444
Q

How do infants with congenital rubella infection present?

A

Petechiae
Jaundice
HSM

445
Q

What kind of virus is rubella?

A

Ss + sense RNA virus

446
Q

How do you diagnose congenital rubella?

A

DNA PCR <1yo
IgM <3mo
IgG 6-12mo

447
Q

Name sources of toxoplasmosis infection

A 
Undercooked meat/meat products
Raw fruits
Raw vegetables
Poor hygiene
Contaminated surface water
Cat faeces
448
Q

When is the risk of toxoplasmosis infection transmission from the mother to the child highest?

A

3rd trimester

449
Q

When is the risk of toxoplasmosis infection causing harm to the baby highest?

A

1st-2nd trimester

450
Q

What is the classic tetrad of toxoplasmosis?

A

Hydrocephalus
Epilepsy
Cerebral calcification
Chorioretinitis

451
Q

Discuss treatment of neonatal varicella

A

Immunoglobulin

Acyclovir

452
Q

Discuss immunoprophylaxis of hepatitis B

A

Single antigen HBV cavvine

HBIG 0.5ml

453
Q

Discuss the stages of syphilis in the mother

A
  1. 3-6w: painless, spontaneously resolving paple
  2. 6-8w: diffuse inflammation and disseminated rash
  3. Latent stage
  4. Granulomas affecting bones, joints, CV and neurological systems
454
Q

Name the clinical manifestations of early congenital syphilis

A 
Prem
Growth restriction
HSM
Nasal chondritis
Skin rash
Osteochondritis
Hydrocephalus
455
Q

Name the clinical manifestations of late congenital syphilis

A 
Craniofacial abnormalities
Dental abnormalities
Interstitial keratitis
Deafness
Neurosyphilis
456
Q

What investigations would you do in a case where you suspect congenital syphilis?

A 
Dark field microscopy of placenta
VDRL
RPR
Enzyme immunoassay
FTA-ABS
457
Q

Discuss the treatment of congenital syphilis

A

IV penicillin

458
Q

What is the new FDC?

A

Tenofovir (TDF)
Lamivudine (3TC)
Dolutegravir (TLD)

459
Q

What anomaly is dolutegravir use associated with?

A

Neural tube defects

Avoid in periconception and first 6w of pregnancy

460
Q

Define VL suppression in HIV

A

VL <50c/ml

461
Q

Classify prematurity

A 
<37w
Late preterm = 34-36w6d
Moderate preterm = 32-34w
Very preterm = 28-32w
Extreme preterm = <28w
462
Q

Classify low birth weight

A

<2500g
LBW = 1500-2499g
VLWB = 1000-1499g
ELBW = <1000g

463
Q

Give benefits of delayed cord clamping

A 
Improves BP
Reduces IVH
Reduces NEC
Reduces need for vasopresssors
Reduces need for blood transfusion

Coovadias Paediatrics (10 decks)

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