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Flashcards in Neonatology Deck (65):
1

A baby presenting with hypotonia, facial weakness and respiratory distress is suggestive of which condition ?

Congenial myotonic dystrophy

2

What is the genetic mutation which causes congenital myotonic dystrophy?

Triplet repeat in DMPK gene

3

What part of the brain is most affected by hypoglycaemia?

Occipital lobe

4

In sick preterm infants what is the most important variable affecting insensible fluid losses?

Gestation

5

What are 4 causes of hydrops?

Alpha thalassaemia
Blackman diamond anaemia
Foetal parvovirus
Rhesus disease of the newborn

6

What are three key features of Blackfan Diamond Anaemia?

1. Normochromic macrocytic anaemia
2. Congenital malformations (microcephaly, cleft palate, thumb anomalies, cardiac renal anomalies)
3. Increased risk malignancies (AML, myelodysplastic syndrome, osteosarcoma)

7

Term baby presents at two weeks of life with persistent jaundice. Clinical findings include decreased tone, umbilical hernia, large anterior fontanelle. What is the most likely cause?

Congenital hypothyroidism

8

What are the clinical features of congenital hypothyroidism?

Microcephaly
Large anterior fontanelle
Jaundice
Sluggishness, minimal cry
Poor appetite/feeding difficulties
Somnolence/lack of interest
Protuberant abdomen, umbilical hernia
Macroglossia
Cold & mottled skin
Hypotonia

Later diagnosis 3-6 months
Coarse facial features:
-hypertelorism
-narrow palpabral fissures, swollen eyelids
-delayed dentition
-short thick neck
-broad hands, short fingers
-dry scaly skin
-coarse brittle hair

9

What are the risk factors for NEC in term infants?

Polycythaemia
Cardiac surgery
Abdominal surgery (gastroschisis)
Endocrine abnormalities

10

What are the clinical features of NEC?

Abdominal distension
Bilious vomiting
Bloody stools
Temperature instability
Apnoeas
Bradycardia

11

What are the radiographic findings of NEC?

Ileus
Portal venous gas
Pneumatosis intestinalis
Pneumoperitoneum

12

What is the treatment for NEC?

NBM
Blood cultures
IV antibiotics (vancomycin, gentamicin, metronidazole)

13

What are some protective factors for NEC?

Antenatal corticosteroids
Breast milk
Early NBM if suspect NEC

14

What are the clinical features of Hirschsprung’s disease?

Looks well
Abdominal distension
Bilious vomiting
Failure to pass meconium <48hrs
Squirt sign - explosive passage of gas/stool with palpation

15

At what gestational week does intestinal malrotation occur?

Week 10

16

What is the most severe outcome of 2nd trimester oligohydramnios?

Pulmonary hypoplasia

17

10 day old baby presenting with bilateral supportive conjunctivitis is suggestive of which organism ? What is the treatment ?

Chlamydia trachomatis
Azithromycin

18

2 day old neonate presenting with bilateral suppurative conjunctivitis is suggestive of which organism ?

Neisseria gonorrhoea

19

How to differentiate traumatic tap from SAH?

SAH
High opening pressure
RBC don’t clear with subsequent tubes

20

What are the risk factors for neonatal subarachnoid haemorrhage?

Instrumental vaginal deliveries

21

What is the mechanism of action of phototherapy?

Blue light causes geometric photo-isomerisation of unconjugated bilirubin into a more water soluble molecule

22

What are the predominant cells found in erythema toxicum?

Eosinophils

23

What is the rate of congenital malformation for sodium valproate?

1-2%
(10-20x general population)

24

What are the differentiating features between deformational plagiocephaly and craniosynostosis?

Plagiocephaly:
Parallelogram shaped head
Ipsilareral ear displaced anteriorly
No palpable bony ridges

Craniosynostosis:
Trapezoid shaped head
Ispilateral ear displaced posteriorly
Palpable bony ridges

25

Which parts of the brain are most affected by bilirubin in kernicterus encephalopathy?

Nasal ganglia
Acoustic nerve

26

Fortification of human donor milk increases risk of NEC - true or false

False as per Cochrane review

27

Antenatal steroids reduces risk of NEC - true or false

True

28

Probiotics reduce the incidence of NEC and mortality - true or false

True

29

What are the indications for surgery for NEC?

Perforation
Abdominal mass
Failure to respond to medical management

30

What provides the strongest stimulus for postnatal closure of the ductus in a term infant ?

Circulating systemic oxygen

31

What percentage of RV output goes through the lungs in foetal circulation?

13%

32

Which parts of the body do the left and right ventricles supply in foetal circulation ?

LV upper 1/3 of body
RV lower 2/3

33

What should you suspect as the cause of jaundice in a 2 week baby with poor weight gain, umbilical hernia, large fontanelle

Congenital hypothyroidism

34

What is the mechanism of macrosomia?

Insulin doesn’t cross placenta
Baby receives low glucose levels from mum which stimulates baby’s own insulin levels to go up - stimulates growth factor — big baby

35

What are the benefits of caffeine in preterm neonates?

Decreases incidence of chronic lung disease
Increases disability free survival at 20 months of age
Reduces weight gain
Decreases incidence of severe ROP

36

What are the outcomes for late preterm babies compared to term?

Worse neurodevelopmental outcomes
Increased risk of cerebral palsy

37

Giving magnesium sulphate to <32 weekers has what benefit?

Reduces incidence of CP by 1/3

38

What is the survival rate of babies born at 24 weeks in Australasia? And 23 weeks?

70%
50%

39

Caffeine therapy reduces the incidence of chronic lung disease - true or false

True

40

At what spinal level are the
Renal arteries
Superior mesenteric artery

L1-2
T12 - L1
Therefor aim UAC not below T10

41

Baby presents with projectile non bilious vomiting
X-ray shows double bubble sign
What is the diagnosis and what syndrome is associated ?

Duodenal atresia
Down syndrome 30%

42

What is the pattern of inheritance for retinoblastoma ?

Autosomal dominant with incomplete penetrance

43

What syndrome is associated with omphalocoele and Low glucose

Beckwith Wiederman

44

Gastroschisis is associated with
IUGR and intestinal atresia - true or false

True
Intestinal atresia in 10-15%

45

In regards to congenital diaphragmatic hernia - which side has higher mortality ?

Right

46

Congenital diaphragmatic hernias are usually associated with additional congenital anomalies - true or false

False

47

Penicillin is an appropriate antibiotic to cover listeria - true or false

False - 30% are resistant to penicillin

48

Ingestion by the mother of which drugs gives babies a smaller head?

Methadone
Methamphetamine

49

What is the immune function of human milk oligosaccharides?

Anti adhesive function
Specific actions against GBS, enteropathogenic E. coli & Campylobacter

50

What is the most likely neirodevelopmental outcome for a 26 weeker with a relatively uncomplicated course or RDS and feed intolerance

Spastic diplegia (leg area of vortex closest to ventricle - most susceptible to any small insult)

51

What are the risk factors for cerebral palsy ?

Chorioamnionitis
Low birth weight
Maternal thyroid disease & thrombophilia
Elevated cord levels of inflammatory cytokines
Multiple births
Other congenital abnormalities

52

What are the contraindications to BCG vaccination?

HIV infection/immune compromise
Generalised skin disease eg eczema
Maternal anti TNF therapy during pregnancy
- delay BCG
Positive mantoux and under 8 months old

NB effective against generalised/miliary TB, TB meningitis but not pulmonary TB

53

In regards to HIV vertical transmission - what is the most predictive factor?

Viral load
(Not CD4 count)

54

What are the usual patterns of brain damage in HIE for preterm vs term babies?

Preterm usually white matter - periventricular
Term usually Grey matter - subcortical white matter and parasagittal critical injury

55

What is the mortality of HIE

20-30%

56

What is the the therapeutic window for HIE ?

2-6 hours

57

What is the estimated protein requirement for a stable preterm infant > 1 week of life ?
What are the energy requirements ?

3-4g/kg/day
ELBW energy requirements = 75-85 kcal/kg/day
VLBW = 60-70kcal/kg/day

58

What congenital infection is associated with salt and pepper retinopathy and cataracts ?
(Clue sensorineural deafness, cardiac disease, blueberry muffin rash)

Rubella

59

What congenital infection is associated with dermatomal rash, hypoplastic limbs?
Chorioretinitis & cataracts
Cutaneous/cicatricial scars
Seizures

Varicella

60

What congenital infection is associated with microcephaly, intracranial calcifications, sensorineural hearing loss, hepatosplenomegaly, thrombocytopenia ?

CMV

Sensorineural hearing loss is classic

61

Which congenital infection is associated with irritability, rhinitis, periostitis and jaundice ?

Syphilis
(Treponema pallidum)

Later disease > 2 yrs
Bulldog facies
Mulberry molars
Hutchison teeth

62

What congenital infection is associated with microcephaly, limb contractures, eye and neurological abnormalities and sensorineural hearing loss ?

Zika virus

63

What are the characteristic features of prune belly syndrome?

95% male
Bowel malrotation
Cardiac abnormalities 10%
Patent urachus
Cryptorchidism

64

How do you define the grade of IVH?

Grade I - bleed into germinal layer
Grade II - bleed into ventricle without dilation
Grade III - bleed into ventricle with dilatation
Grade IV - intraparenchymal bleed

65

Without intervention what is the vertical transmission rate of HIV?

What are the recommendations for mode of delivery and breastfeeding ?

15-45%
Caesar only indicated for obstetric reasons
Breastfeeding recommended for developing world but not first world
Formula fed in first world with viral suppression therapy risk comes down to <2%