Neonatology Flashcards

1
Q

What is the perinatal mortality

A

Stillbirths + deaths within 7 days of birth per 1000 deliveries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

In toxoplasmosis what is the relationship between infection, severity and gestation

A

Lower gestation- reduced risk infection, increased severity
Older gestation- 60% risk of transmission, reduced severity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the signs of congenital toxoplasmosis
What is the treatment

A

Chorioretinitis
Hydrocephalus, microcephaly
Diffuse cerebral calcifications

Hepatospenomegaly
Jaundice
Thrombocytopenia

Pyrimethamine and Sulfadiazine
Leucovorin- helps prevent bone marrow suppression

If during pregnancy in first trimester, Sulfadiazine only
2nd trimester Sulfadiazine and Pyrimethamine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the signs of congenital syphilus

A

“SYPHILIS”
-Sniffles
-Yucky skin
-Periosteal reaction
-Hepatosplenomegaly
-iyes (“eyes”: chorioretinitis, glaucoma
-Lymphadenopathy
-mIscarriage
-Saddle shaped nose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the signs of congenital syphilus

A

“SYPHILIS”
-Sniffles
-Yucky skin
-Periosteal reaction
-Hepatosplenomegaly
-iyes (“eyes”: chorioretinitis, glaucoma
-Lymphadenopathy
-mIscarriage
-Saddle shaped nose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What does VDRL stand for and what does it test

A

Veneral disease related lab test - checks for syphilis antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the symptoms of congenital Rubella

A

RUBELLA
-Retina- cataracts, retinopathy
-U= heart= PDA, PA stenosis, PV stenosis
-Blueberry rash
-Ears- SNHL
-Little- SGA
-Lagging- neurodevelopment delay
-A bit liver and spleen - hepatosplenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the symptoms of congenital CMV
What is the treatment

A

CCMMVV
-Calcifications- periventricular
-Chorioretinitis
-Milestone delay- CP and reduced IQ
-Microcephaly
-Very poor hearing- SNHL
-Very big liver - Hepatosplenomegaly
-Blueberry rash

6 weeks oral Valganciclovir

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the signs of congenital HSV infection
What is the treatment in
-primary infection
-secondary infection
-pregnancy

A

Scarring
microcephaly
Choriretinitis

Primary infection- IV Acyclovir
2nd infection- swab at 24-48 hours- if positive commence IV Acyclovir
Pregnancy: 4 weeks acyclovir before delivery and C-section

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the signs of congenital varicella syndrome
When is the baby most at risk

When is neonatal varicella contracted
What is the treatment
What is the prevention

A

Skin: Scarring
Limbs: hypoplasia, parasthesia
CNS: microcephaly, brain aplasia, hydrocephalus
Eyes: chorioretinitis, cataracts

5 days before to 2 days post birth
Treatment: IV Acyclovir
Prevention: VIG 5 days before - 2 days post delivery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the symptoms of withdrawal
What is the most common sx
What percentage of babies exposed to heroin experience withdrawal vs how many require treatment

A

Wakefullness
Irritability
Tremors
Hypertonia, high pitched cry
Diarrhoea
Rhinnorhea
Autonomic instability- fevers, tachycardia
Weight loss and poor feeding
Apnoea and respiratory distress
Lacrimation

Common - tremors
Least common- seizures - 2%

70% experience withdrawal
only 1/2 of those need treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the normal range of amniotic fluid
What aneuploidy causes oligohydramnios
What aneuploidies cause polyhydramnios

A

5-25cm

T13

T18, T21

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the criteria for fetal hydros
What is an immune causes
What are some non-immune causes

A

Subcutaneous oedema AND 2 of
-pericardial effusion
-pleural effusion
-ascites

Immune- Alloimmune haemolytic disease of the newborn
Non-immune- high cardiac output States
-anaemia e.g. Twin-twin-transfusion syndrome
-Cardiac: SVT, cardiomyopathy
-GI: diaphragmatic hernia
-Chromosomal- T21, Turners
-Infection: parvovirus
-pulmonary lymphangiectasis –> chylothorax

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the difference between IUGR and SGA

A

IUGR= deviation from expected growth pattern - due to unfavourable uterine conditions that cause change in fetal growth pattern
SGA= birth weight <10th centile- can be normal or pathological

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the most accurate way of measure fetal age in first and second trimester

A

Crown-rump length before 12 weeks-most accurate
Biparietal diameter 2nd trimester = after 30 weeks accuracy falls to +/-3 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the 8 cardinal movements of the foetus during delivery

A

1- Head floating not engaged
2- Engagement and flexion
3- Further descent and internal rotation
4- Complete rotation so posterior of head is aligned along the pubic symphisus
5- Complete extension
6- Restitution- external rotation
7- Delivery anterior shoulder
8- Delivery posterior shoulder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the 8 cardinal movements of the foetus during delivery

A

1- Head floating not engaged
2- Engagement and flexion
3- Further descent and internal rotation
4- Complete rotation so posterior of head is aligned along the pubic symphisus
5- Complete extension
6- Restitution- external rotation
7- Delivery anterior shoulder
8- Delivery posterior shoulder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What makes up fetal Hb
What direction is the fetal Hb oxygen saturation curve shifted
What does this mean

At what age do you start making adult Hb
What age is most fetal Hb removed

A

Fetal Hb- 2-alpha and 2-Gamma Hb molecules
Fetal Hb has higher affinity for oxygen, shifting the curve to the left and having a steeper curve
This means that despite reduced overall oxygen content, the Hb is as saturated as adult Hb

Third trimester
Most removed by 3-6 months; All removed by 1 year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What percentage of fetal circulation is delivered to the lungs
What cases pulmonary vasoconstriction

A

10%
Hypoxia, Hypercapnia, Acidosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are a premature infants insensible losses
What are their Na, K and calcium requirements/ day

A

2-3ml/kg/hr

3mmol/kg/day
2mmol/kg/day
1mmol/kg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What does SMOF stand for

A

Soy bean
MCFA
Olive oil
Fish oil

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the most common cause of hyperkalaemia in a premature infant

A

Non-oliguria hyperkalaemia due to immature Na/K ATPase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What causes anaemia in a premature infant

A

reduced iron stores
prematurity suppresses erythropoiesis
rapid expansion of blood volume with growth
frequent sampling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What babies receive supplemental iron

A

<37 weeks
<2500g

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What babies receive probiotics

A

<32 weeks and/or <1500g until 36 weeks gestation
Must be mixed with feeds

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what is the earliest marker of metabolic bone disease
when do you start measuring

A

Serum phosphate (+ ALP)
4-6 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is the NICU survival rate for the following ages
-24
-25
-26-27

A

70%
80%
85%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What are the 3 biggest causes of morbidity in NICU

A

Respiratory distress
Sepsis
IVH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Name bacteria that are considered coagulase negative staph
How does it normally present in the neonate

A

S. epidermis
S. haemolyticus
S. saprophyticus

Present: sepsis/bacteraemia
TX: IV Vancomycin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Where is listeria acquired from
What are maternal sx
What is seen in neonatal infection
What is the treatment

A

Soft cheese, pate, uncooked meats, coleslaw
Maternal flu like illness in pregnancy

Baby
-maculopapular rash
-transplacental transmission –> disseminated abscess –> multiorgan dysfunction
-important cause of purulent meningitis

TX: IV Amoxicillin and Gent
NOT cephalosporins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What are the layers of the scalp

A

Subcutaneous tissue
Connective tissue
Aponeurosis
Loose connective tissue
Periosteum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Where is the default/ blood loss in
-cephalohaematoma
-subgaleal
-caput seccundum

A

-subperiosteal
-sub-galeal-aponeurosis - between the periosteum and overlying galeal aponeurosis
-tissue swelling in the connective tissue layer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What amount of blood loss is required to cause an increase in head circumference by 1cm

What is the transfusion order in a smbgaleal haemorrhage

A

35mls of blood is needed to increased HC by 1cm

1) RBC - FFP - RBC - Cyroprecipitate
2) calcium/glucose infusion
3) RBC- FFP- RBC-PLT-Calcium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

In a subgaleal what do you give if the pH is <7.3

A

Sodium bicarbonate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What is used in the treatment of clavicle fractures

A

Supportive cares only
-PRN paracetamol
-Pin sleeve to clothes to immobilise the arm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What position is the arm held in during and what nerve roots are affected in
-Erbs palsy
-Klumpe palsy
-Facial nerve palsy

A

Erbs = waiters tip position- C5/C6 affected = adducted arm, extended at elbow, pronated, internally rotated

Klumpke palsy = C7=C8=T1 affected = wrist drop, claw hand, Horners syndrome

Facial nerve palsy = compression of lower motor neurons of facial nerve during delivery or with forceps. Ipsilateral facial paralysis including the forehead

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What are the embryology stages of lung development

A

Embryonic stage- 4-7 weeks - lung bud formation from endoderm

Pseudoglanular stage 7-17 weeks- development of airways from bronchi –> terminal bronchioles plus main pulmonary artery

Canullicular phase 17-24 weeks- lengthening of the airways, development of type 1 and type 2 pneumocystis, surfactant production at 24 weeks

Saccular phase- 24-36 weeksdifferentiation of type 1 and type 2 pneumocytes, progressive surfactant production, proliferation of alveolar ducts

Alveolar phase- 36 weeks =40 weeks - expansion of gas exchange area, maturation of nerves and capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What type of alveolar cells produce surfactant

A

Type II pneumocystes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What is the main composition of surfactant

A

Phospholipids and lipids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What is the main constituent of exogenous surfactant preparations

A

Dipalmitoyl-phosphatidylcholine (DPPC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What lipids make up surfactant and what is their role

A

Lipids facilitate in the spread, recycling and adsorption of surfactant. They also have immune function
Surfactant protein A- immune
Surfactant protein B- adsorption and recycling - deficiency is fatal
Surfactant protein C- adsorption and recycling - deficiency results in progressive lung fibrosis
Surfactant protein D- immune

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is the cause of neonatal
hypoxia
hypercapnia

A

V/Q mismatch as alveolar collapse due to reduced compliance and increased chest elastic recoil

Hypercapnia - small tidal volume, reduced minute volume

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What age bracket should be provided antenatal steroids

A

Gestation 23-34+6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

How do antenatal steroids work

A

Increase the differentiation between epithelial cells into type 1 and 2 pneumocysts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

what factors do antenatal steroids reduce

A

RDS
NEC
IVH
Mortality

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

what makes up exogenous surfactant

A

DPPC
Surfactant proteins B + C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Who should receive surfactant

A

FiO2 >30% and clinical diagnosis of RDS i.e. those premature infants with CPAP failure - <32 weeks, FiO2 >30%, on CPAP since birth
OR
Clinical RDS and deteriorating in respiratory status

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

LISA is associated with what 3 better outcomes than INSURE surfactant delivery and in WHAT GESTATION

A

BPD, Pneumothorax, IVH
29-32 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What has LISA been associated increased risk of in extremely preterm infants

A

Spontaneous intestinal perforation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What is the definition of chronic lung disease

A

Ongoing oxygen requirement at 36 weeks (if born <32 weeks) with persistent oxygen for >28 days

OR
If born >32 weeks, persistent oxygen requirement after 28 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is the new vs old causes of CLD

A

OLD: overly aggressive mechanical ventilation and oxygen toxicity –> airway inflammation, injury and parenchymal fibrosis

NEW: Infants born <1500g or <28 weeks have significant disruption in lung development resulting in:
= large abnormal alveoli –> less surface area for gas exchange
= abnormal vasculature with thickening of muscular layer –> pulmonary HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

97% of cases of CLD occur below what age

A

28 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

BPD in relation to mechanical ventilation is primarily due to what type of trauma

A

Volutrauma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What are 5 risk factors for developing CLD

A

-Prematurity (especially <28 weeks) - lungs in the saccular phase of development with few alveoli and poorly organised support structures
-Mechanical ventilation - volutrauma
-Oxygen toxicity
-Infection e.g. chorioretinitis
-PDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What are 5 risk factors for developing CLD

A

-Prematurity (especially <28 weeks) - lungs in the saccular phase of development with few alveoli and poorly organised support structures
-Mechanical ventilation - volutrauma
-Oxygen toxicity
-Infection e.g. chorioretinitis
-PDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

In chronic lung disease why are steroids not used as treatment
When are they considered

A

They do provide benefit of weaning off ventilation
BUT
They increase the risk of neurodevelopment outcome adversity e.g. CP and having increased risk of GI bleed/perforation, hyperglycaemia, poor growth

Considered
-7-21 days requiring mechanical ventilation
-In supplemental oxygen
-High risk of CLD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

What is the difference in length between a low dose course, high dose ‘Cummings course” and tailored course of steroids

A

Low dose course- 10 days
High dose course-= 43 days
Tailored coures = 18 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

What are the treatment of CLD

A

Supportive
-Optimise ventilation to reduce volutrauma and oxygen toxicity
-Fluid restrict to 150ml/kg/day
-Fortify to ensure adequate nutrition and growth

Consider diuretics if concerns regarding pulmonary oedema (PDA, excessive weight gain) OR if fluid restriction has failed to wean off the ventilator

Routine steroids NOT recommended as worse neurodevelopment outcome AND GI perforation

RSV prophylaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

What are babies with CLD at increased risk of

A

-death in first year of life
-reactive airway disease
-pulmonary HTN
-neurodevelopmental impairment
-steroid use –> osteopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Define an apnoea of prematurity

A

Cessation of breathing for >20 seconds plus associated bradycardia (<2/3 baseline) OR desaturation <80% AND infant <37 weeks gestation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

What is the most common type of apnoea in a premature infant

A

Mixed central and obstructive- starts obstructive then becomes central

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Why do premature infants get Apnoea of prematurity

A

Immaturity of the central respiratory drive
-Blunted response to hypercapnia or central and peripheral chemoreceptors
-Biphasic response to hypoxia - initial hyperopnoea then hypopnea and apnoea

PLUS
Immature and floppy airway with impaired protective reflexes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What is an example of a Methyxanthines and when are they used

A

Caffeine
Apnoea of prematurity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

How does caffeine work

A

It reduces the respiratory drive threshold to hypercapnia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What has caffeine been shown to reduce the incidence of

A

BPD
ROP
Cerebral palsy
Post natal steroid use
Cognitive delay at 18-21 months

63
Q

what is the MoA of Doxapram

A

Stimulates peripheral chemoreceptors in the carotid bodies of the carotid arteries

63
Q

what is the MoA of Doxapram

A

Stimulates peripheral chemoreceptors in the carotid bodies of the carotid arteries

64
Q

Why does meconium aspiration cause respiratory distress

A

Causes physical obstruction- ball valve mechanism with hyperinflation
Chemical pneumonitis
Displaces surfactant

65
Q

What do you see on oximetry in a baby with PPHN

A

> 10mmHg difference in saturation between pre and post ductal saturations

Represents ongoing persistence of fetal circulation with R –> L shunting across the PDA due to ongoing high respiratory vascular resistance

66
Q

What are the 3 pathophysiological causes of NEONATAL PPHN

A

-Vasoconstriction- failure to normally respond to reduced hypoxia and improved ventilation
-Obstruction causes increased pulmonary venous pressures
-Abnormal pulmonary vasculature with increased medial muscle thickness- due to fetal hyoxia OR due to pulmonary hypoplasia

67
Q

How is pulmonary vascular resistance calculated

A

PVR = (Pressure Pulmonary Artery - Pressure Pulmonary vein) / Pulmonary blood flow

68
Q

What grades of mean pulmonary arterial pressure are norma, mild, moderate and severe PPHN

A

normal <25
mild 25-40
moderate 40-55
severe >55

69
Q

What pulmonary pressures compared to systemic pressures correlate to normal, mild pphn, moderate pphn and severe pphn

A

o Normal: PA pressure <1/3 systemic pressure
o Mild: PA pressure 1/3-2/3 systemic pressure
o Moderate: PA pressure 2/3 – 1 systemic pressure
o Severe: PA pressure >1 systemic pressure

70
Q

what is the MoA of inhaled NO

A

Diffuses into vascular endothelial cells exclusively and activated cGMP-dependent protein kinases. These protein kinases then phosphorylate various ionic channels in the Endoplasmic reticulum causing calcium sequestration. This prevents calcium mobilization in the cell resulting in relaxation of vascular smooth muscle.

71
Q

what is the MoA of sildenafil

A
  • Potent cGMP specific phosphodiesterase inhibitor
  • As with iNO, cGMP activation results in phosphorylation which reduces calcium mobilisation within a vascular smooth muscle cell
  • Phosphodiesterase breaks down cGMP therefore the inhibitor results in increased circulating levels
72
Q

why is it important to maintain SBP in PPHN

What agents can be used to maintain BP

A

if low blood pressure, will worsening R –> L shunting and therefore hypoxia and acidosis

Dopamine infusion
Dobutamine infusion
Adrenaline infusion

73
Q

What is the difference between venarterial and veno-veno ECMO

A

venoarterial goes from R) atrium –> Ecmo –> Aorta
-have to divide the common carotid artery
-increased risk of stroke
-lung and heart bypass

veno-veno: jugular vein –> ecmo –> RV
-lung bypass only
-nil control over cardiac output
-dont have to divide the aorta

74
Q

What are 4 contra-indications to ECMO

A

< 34 weeks
<2kg
Current bleeding
Irreversible lung damage

75
Q

What stage of lung development do diaphragmatic hernias occur

A

Embryonic and pseudo glandular

76
Q

What are the two types of diaphragmatic hernias
Which is more common

A

o Bochdalek:95% of cases occur posterolaterally. 90% happen on the left side.
o Morgagni: Retrosternal.

77
Q

How do you calculate the oxygenation index for ECMO
What OI is normal
What OI indicated referral for ECMO

A

OI = MAP X FiO2 / Pa O2
- Mean airway pressure
- PaO2 = arterial oxygen content
Index
- OI <5 = normal
- OI >10 = severe oxygenation problem
- OI >20 = extreme oxygenation problem
- OI > 40= ECMO referral

78
Q

In an infant with no microsomal abnormality what other factor determines survival in pulmonary hypoplasia

A

Degree of residual functioning lung
Demonstrated by the lung volume: head size ratio

If the lung volume to head size ratio is < 1 almost no infants survive.
If the lung volume to head size ratio is > 1.4 almost all survive.

79
Q

What are common issues in a baby with congenital diaphragmatic hernia

A

PPHN
Pulmonary hypoplasia
GORD- 50% affected
40% mortality rate

80
Q

What is the pathogenesis of CPAM
What are the types

A

o Abnormal branching morphogenesis of the lung.
o Molecular mechanisms are unknown but may include an imbalance between cell proliferation and apoptosis during organogenesis
o Disorders of HOXB5 have been implicated in the process

Types
based on the LOCATION, plus the size of the cyst and their cellular characteristics
Type 0: trachea and proximal bronchus- small cysts – very rare- fatal

Type 1 (65%)- distal bronchi and proximal bronchioles:
 distinct thin walled cysts- usually single but may be multi-loculated.
 Well differentiated tissue so felt to originate late in embryogenesis.
 Single or multiple large cysts.
 Despite shift effects, there is usually a good prognosis

Type 2 (20%)- terminal bronchioles
 multiple small cysts which blend into adjacent normal tissue.
 Cysts resemble dilated terminal bronchioles.
 Associated with other congenital abnormalities in 60% of cases e.g. TOF, bilateral renal agenesis

Type 3 (5-10%): near alveolus
 very large and can involve entire lobe or several lobes.
 Cysts develop near the alveolus and are so small they look like a solid mass
 Can be a mixture of cystic and solid tissue.
 Due to their size and lack of differentiation, thought to originate early in pregnancy.
 Worst prognosis

Type 4: distal alveolar

81
Q

In pulmonary sequestration when blood supplies the non functional lung

A

Systemic blood normally from a branch of the thoracic aorta

82
Q

What is schmiter syndrome

A

R) lung hypoplasia and TAPV with drainage of the right pulmonary vein into the IVC or R) atrium

83
Q

when does a tension pneumothorax occur

A

Tension pneumothorax occurs if the accumulation of air within the pleural space is sufficient to elevate intra-pleural pressure above atmospheric pressure

84
Q

what cardiac condition is associated with pulmonary haemorrhage

A

PDA

85
Q

In mechanical ventilation what factors affect
-oxygenation
-ventilation

A
  • Oxygenation (PaO2) is determined by the Inspired oxygen fraction (FiO2) and Mean airway pressure (MAP).
  • Ventilation (CO2 clearance) is determined by the Minute Volume (MV= Tidal Volume (VT) x Rate (R)).
86
Q

when setting a volume guarantee what will the machine adjust to ensure a volume is delivered with each breath

A

the PIP

87
Q

when will Volume guarantee not work

A

When there is a large leak around the ETT

88
Q

what is the difference between
PC-AC
SIPPV
PC-SIMV
PC-PSV

A

PC-AC = pressure control- assist control
SIPPV= synchronised intermittent positive pressure ventilation
BOTH the above are the same thing by different names- each breath the baby takes is assisted by the ventilator

PC-SIMV: pressure control-synchronised intermittent mandatory ventilation = Supports a certain number of breaths- if baby breathing above this doesn’t support this

Think-Mandatory = uncompromising = will only assist set breaths

PC-PSV= pressure control pressure support ventilation- similar to PC-AC; all breaths are supported BUT baby also determines the DURATION of the breath

89
Q

In HFOV how do you improve ventilation

A

Increase amplitude
Decrease frequency

90
Q

How do you calculate the ventilation index
What do the numbers mean

A

VI= PCO2 x RR x PIP / 1000
VI > 70 = severe respiratory failure
VI > 90 = very severe failure; consider ECMO

91
Q

what is the insensible fluid loss in premature infants

A

2-3ml/kg/hr

92
Q

What it the step increase in fluid requirements for a day 1 - day 7 baby

How many milli-mols of Na/K/Ca supplementation

A

ml/kg/day
60
75
90
105
120
150
180

Na=3
K= 2
Ca=1

93
Q

What would be 3 reasons for neonatal hyperkalaemia

A

-haemolysis or red cell break down
-Congenital adrenal hyperplasia
-severe metabolic acidosis

94
Q

How many kcal/day does a preterm vs term infant need

A

120 vs 100kcal/day

95
Q

What is the kcal content of glucose, protein and fat

A

Fat= 9
Glucose=4
Protein = 4

96
Q

why is cows milk not recommended in first year of life

A

low iron bioavailability

97
Q

what is the absorbable content of iron in breast milk vs cows miilk

A

breast= 50%
cows=10%

98
Q

what is the dose of prophylactic iron
who is it given to

A

1ml/kg/day
<37 weeks OR <2500g
until.1 year of age

99
Q

what immunoglobulin is found in breast milk

A

IgA

100
Q

what signs in Bells scoring system for NEC
stage
1
IIA
IIB
IIIA
IIIB

A

Stage 1- apnoea, temp instability, feed intolerance, occult blood in stool normal AXR

Stage 2A: apnoea, temp instability feed intolerance, GROSSLY BLOODY STOOL, FOCAL pneumatosis on AXR
Stage 2B: Thrombocytopenia, mild metabolic acidosis, ABDOMINAL WALL OEDEMA, palpable loops and tenderness, WIDESPREAD pneumoatosis

Stage 3A: mixed acidosis, coagulopathic, WORSENING ASCITES, NO free air, worsening wall oedema with erythema and induration

Stage 3B: Pneumoperitoneum- perforated bowel- shock and deterioration

101
Q

what stage of NEC do you get pneumoperitoneum

A

Stage 3B

102
Q

what stage of NEC do you get grossly bloody stool and focal pneumatosis

A

Stage IIA

103
Q

what stage of NEC do you get abdominal wall oedema, widespread pneumoatosis and thrombocytopenia

A

stage IIB

104
Q

What stage of NEC do you get
-thrombocytopenia
-coagulopathy

A

IIB
IIIA

105
Q

What stage of NEC do you get worsened wall oedema with erythema plus no free air in bowel and mixed acidosis

A

IIIA

106
Q

what is pneumatosis

A

presence of gas in the wall of the intestine

107
Q

What % of infants with NEC have bacteraemia

A

20%

108
Q

What haematology sign is associated with impending bowel perforation in NEC

A

DIC

109
Q

What antibiotic regime is used in NEC

A

Amoxicillin- enterococcus
Gentamycin- Gram -ve
Metronidazole - anaerobes

110
Q

Describe the management of NEC

A

-NBM and start TPN for 7-14 days
-IV Abx- Amox/Metronidazole/Gent
-NG gastric decompression- replace gastric losses IV
-Strict fluid balance
-Surgery if perforation OR failure of medical management
-Post surgery do not perform rectal temp

111
Q

What area of gut resection in NEC will cause the most significant malabsorption

A

Jejunum- where most amino acids and disaccharides absorbed

112
Q

what condition is meconium Ileus associated with

A

CF

113
Q

What is the first line treatment in meconium ileus

A

Gastrograffin enema

114
Q

Describe Type A, B, C, D and E TOF

A

Type A- oesophageal atresia but no fistula
Type B- oesophageal atresia with proximal TOF
Type C- oesophageal atresia with distal TOF
Type D- oesophageal atresia with proximal and distal TOF
Type E- nil atresia but a TOF

115
Q

What is the most common type of TOF and describe it
What % have this version

A

Type C- oesophageal atresia with distal TOF
85%

116
Q

How does a Type E TOF frequently present
Describe a Type E TOF

A

Recurrent LRTIs
No atresia but a TOF

117
Q

What syndrome are most TOFs associated with and what does are the signs

A

VACTREL
-Vertebral
-Anal abnormalities
-Cardiac abnormaliteis
-Tracheal-oesophageal anomalies
-Renal anomalies
-Ears
-Limbs

118
Q

What are some complications post atresia/TOF repair

A

stricture
GORD
re-fistulisation
dysphagia

118
Q

What are some complications post atresia/TOF repair

A

stricture
GORD
re-fistulisation
dysphagia

119
Q

What is the cause of duodenal atresia
What syndrome is it associated with

A

Intestinal tract is blocked by endoderm epithelium
Downs syndrome

120
Q

What is the presentation of duodenal atresia

A

Vomitting without abdominal distension in the first day of life

121
Q

At what week does the midgut return to the abdomen in utero

A

10-11

122
Q

What is the centre of intestinal rotation

A

the superior mesenteric artery

123
Q

Where should the duodenal junction and caecum be relative to the spine in a correctly rotated bowel

A

cacum- RLQ
DJ- LUQ

124
Q

What condition is a LADD procedure used for and what does it involve

A

Malrotation
Division of the LADD bands of peritoneum, correction of Volvulus, appendectomy and functional positioning +/- Internal fixation

125
Q

What is a persistent cloaca

A

Failure to divide the cloaca into a separate return and Genitourinary system - rectum, vagina and urinary tract are joint be a single channel

126
Q

What causes an mechkles diverticulum

A

incomplete obliteration of the vitilline duct

126
Q

What causes an mechkles diverticulum

A

incomplete obliteration of the vitilline duct

127
Q

what is the rule of 2s in a meckles diverticulum

A

2% of population
2 inches in length
2 feet from ileocaecal valve
2 types of tissue- gastric and pancreatic
Presents before age of 2

128
Q

what 2 types of tissue are contained in the ileocaecal valve

A

pancreatic
gastric

129
Q

What test is used to diagnose meckles

A

Technitium-99
Taken up by gastric mucosa = stomach and meckles

130
Q

what is the difference in pathogenesis between gastroschisis and omphalmocele

A

gastroschisis- failure of anterior abdominal wall closure
omphalocele- failure of midgut to return to abdominal cavity during development

131
Q

what % of omphalmocele are associated with other congenital abnormalities

A

75%

132
Q

what are the signs of Hirschsprung’s disease

A

bowel obstruction relieved with faecal examination

133
Q

what procedure is contraindicated in hypospadius

A

circumcision as preputial skin often used to reconstruct the urethra

134
Q

what is the difference between
-spina bifida occulta
-meningocele
-myelomengiocele

A

SPO: defect in vertebral closure but no protrusion of meninges or spinal cord

Meningocele: protrusion of meninges with cyst formation but no protrusion the spinal cord

Myelomeningocele: herniation of the meninges, nerve roots and spinal cord though the dorsal vertebral defect

135
Q

In which periventricular area doe premature infants experience IVH

A

subependymal germinal matrix

136
Q

at what gestation is there involution of the germinal matrix

A

34 weeks gestation

137
Q

what are the indications for a cerebral ultrasound

A

<32 weeks gestation OR <1250g
Clinical suspicion
Monitoring of hydrocephalus
MCDA twins

138
Q

Describes the 4 grades of IVH

A

Grade 1- germinal matrix
Grade 2- IVH but no ventricular dilitation
Grade 3- IVH with ventricular dilatation
Grade 4- intra-parenchymal haemorrhage that is localised or extensive (involving 2+ regions)

139
Q

what is the most common adverse outcome associated with IVH

A

Cerebral palsy

140
Q

What is the % affected by cerebral palsy in
PIVH requiring shunt
Grade 4 IVH
Grade 3 IVH

A

75%
89%
50%

141
Q

what type of cerebral palsy does PVL result in

A

spastic diplegia

142
Q

what happens in primary vs secondary neuronal injury in HIE

A

primary injury- hypoxia results in anaerobic respiration- resulting in accumulation of cytotoxic metabolites e.g. lactate and glutamate AND fails to produce enough ATP for Na/K ATPase so sodium accumulates causing cell damage –> cell death and cerebral oedema

secondary injury is a reperfusion injury - hypo perfusion then reperfusion with resuscitation results in neutrophil activation and the release of free radicals and prostaglandin –> apoptosis and death

143
Q

what are signs of severe encephalopathy on a sarnat score

A

absent - only responds to painful stimuli
spontaneous movement - none
flaccid tone
absent suck
absent moro
apnoea

144
Q

what are sarnat signs of moderate encephalopathy

A

reduced responsive to non-painful stimuli
reduced spontaneous activity
hypotonia
incomplete moro
incomplete suck
periodic breathing

145
Q

What weight, gestational age and severity of HIE allows for hypothermia

A

Weight >1800g
Gestation age >35 weeks
Severity: moderate-severe

146
Q

what Apgar score at 10 minutes under which do we consider therapeutic cooling
During what window must it be initiated

A

<5 at 10 minutes
6 hours

147
Q

how long is therapeutic cooling for in HIE

A

72 hours

148
Q

what is the first line seizure treatment in neonatal HIE

A

Phenobarbital

149
Q

what fuses in
-brachiocephaly
-scaphocephaly
-plagiocephaly
-trigonocephaly

A

-brachycephaly= short head = coronal suture
-sachpocephaly = ‘boat head’ = saggital suture
-plagiocephaly = assumetrical lambed or coronal suture
trigonocephaly- metopic suture

150
Q

what are the three zones in the retina
what are the stages of ROP

A

Zone 1: optic nerve - macula center - 2x this as a radius
Zone 2: radius from the optic nerve to the nasal ora serrate
Zone 3: remainder of outer boarder of zone 2 to the temporal serrate

stage 1- demarcation line
stage 2- ridge
stage 3- extra retinal fibrovascular proliferation
stage 4- partial retinal detachment
stage 5- total retinal detachment

151
Q

what is plus disease

A

tortuous retinal vessels
retinal haze
pupil rigidity

152
Q

why does ROP occur

A

oxygen toxic to premature eyes
results in retinal vasoconstriction
if vasoconstriction occurs for long enough will get obliteration
then will get neovascularisation

153
Q

who is screened for ROP

A

<30 weeks
<1250g
plus consider:
Twin-twin transfusion
prolonged NO for PPHN
severe sepsis
Grade 3 or 4 IVH

154
Q

when does zone 1 ROP get treated

A

Zone 1 AND plus
OR
Zone 1 stage 3

155
Q

when is zone 2 ROP treated

A

stage 2-3 AND plus