Neonates Flashcards
1
Q
NEC Clinical features Radiological features
A
General early clinical signs
- temp instability
- apnoea
- bradycardia
- lethargy
Abdo signs
- bile-stained aspirates
- abdominal distension, tenderness
- feed intolerance
- positive faecal occult blood/fresh PR blood
Radiological
- pneumatosis (gas within bowel wall)
- pneumoperitoneum (if perf)
- portal venous gas or bubbles in portal vein on ultrasound
2
Q
Risk factors or causative factors for NEC
A
Prematurity
- Premature immunen system
- Impaired intestinal barrier function
- VLBW (poor blood supply to gut)
Exogenous RF
- Formula feeding (carb and protein excess leads to bacterial overgrowth) –> Mx: Breast milk
- infx -> inflammation
- hypoxia -> incr ROS
- anaemia -> incr ROS
Microbial dysbiosis
- Decr beneficial microflora
- Incr pathogenic bacteria
–> Mx: probiotics
Other RF
- PDA (treating PDA doesn’t reduce NEC however)
- Incr gut pH (H2 blockers are assoc w NEC however ?cause/effect)
- Fortification in PT infants
3
Q
Routine prophylaxis against NEC
A
Probiotics Breast milk (mother’s own or donor)
Early (day 2) commencement of enteral feeds
Antenatal steroids in premature infants reduce risk of NEC
4
Q
Microbiome in term babies
A
Pick up the flora of mother’s vaginal canal as they are born: Term babies ‘good’ bacteria - Lactobacilli and bifidobacteria Formula-fed Coliforms, enterococci, bacteroids
5
Q
Microbiome in preterm babies
A
Often from nursery environment rather than from mother’s vaginal canal and skin surface Use of abx can reduce the bacterial diversity (prolonged duration of initial abx course in preterm babies is assoc w risk of NEC and death) Hence give probiotics to give them ‘good bacteria’ normally found in the gut of healthy term bugs (lactobacillus etc) - shown to reduce risk of NEC
6
Q
Indications for surgical mx of NEC
A
Perforation Abdo mass Not responding to medical management Via Formation of ileostomy then closure after 4-6 weeks (contrast study pre closure to ensure no stricture)
7
Q
What is the strongest stimulant for the closure of ductus arteriosus
A
Increased systemic oxygen supply is most important (from initial breaths Also - Decr circ Prostaglandin - Decr pulm vasc res - incr pulm blood flow (due o foramen ovale closure and reduced shunting across DA)
8
Q
Ix for prolonged jaundice (and what is considered ‘prolonged’)
A
2 weeks or 3 weeks in preterm babies FBC Direct/conjugated bilirubin TFTs
9
Q
Congenital hypothyroid features
A
Early signs - prolonged jaundice - poor suck/feeding - bradycardia - constipation - poor tone - FTT - umbi hernia - large anterior fontanelle
10
Q
Definition of hypoglycaemia in newborn
A
<2.6
11
Q
Causes of transient hypoglycaemia in newborn
A
ketotic (low substrate availability ) - IUGR - prematurity - asphyxia - hypothermia - sepsis - malformation Hyperinsulinism (non-ketotic) - diabetic mother - GDM - rhesus isoimmunisation
12
Q
Clinical features of hypoglycemia
A
Apnoea Jitteriness Seizure lethargy Hypotonia
13
Q
Causes of persistent hypoglycaemia in newborn
A
Hyperinsulinism: - Persistent hyperinsulinaemic hypoglycaemia of infancy - Beckwith-Wiedemann syndrome - Insulinoma Metabolic: Carbohydrte metabolism disorder - Galactosaemia - Hereditary fructose intolerance Organic academia - Maple syrup urine disease
14
Q
Mx of hypoglycaemia in nweboern
A
If can feed - Oral glucose (BM or formula then hourly feeds with monitoring if blood glucose levels If cannot feed - IV 10% dextrose (5mg/kg bolus then infusion with TFI 60-90ml/kg/d) - If remains low, may need to escalate to 15-20% glucose infusion
15
Q
What part of brain is most vulnerable to effects of hypoglycaemia?
A
Occipital lobe
16
Q
Effect of caffeine when used to treat AOP
A
Decr ventilation Decr CLD decr need for PDA ligation decr incidence of severe ROP Incr disability free survival Decr cerbral palsy Decr cognitive delay
17
Q
What are the target sats in a preterm baby and why?
A
aim spO2 91-95% reduce risk of ROP reduce need for ongoing O2 at 36 weeks
18
Q
What is late preterm?
A
34 to 36 weeks gestation Incr morbidity and mortality Worse neurodev outcomes at 2 years of age Incr risk of CP
19
Q
Risks of mid trimester oligohydramnios
A
Pulmonary hypoplasia
20
Q
Mx of neonatal abstinence syndrome
A
Morphine -weaning regime
21
Q
Mx of congenital diaphragmatic hernia
A
Intubate at birth then use conventional mechanical ventilation (over oscillation)
22
Q
What does this ultrasound series show?
A
grade IV bleed (arrow in a), which developed into a porencephalic cyst (arrow in b).
23
Q
Maternal antenatal screening ix (not including imaging)
A
Blood group and Ab (Rh)
RBC
Rubella, syphilis and hep B serology
HIV status
Sickle/thal haemoglobinopathy screening
Rhesus Ab if negative
OGTT
Urine dips
24
Q
Maternal antenatal screening ultrasounds
A
1) 8-12 week dating scan
2) 11-14 week nuchal scan
- > nuchal fold thickness measured, is increased in down syndrome (77% sensitivity, 5% risk of false +)
- > presence/absence of nasal bone can give up to 97% risk of down syndrome (when combined with nuchal fold thickness)
- > gives risk for down, trisomy 13 and 18
- > can detect early anomalies and diagnosis of twins and onset twin-twin transfusion syndrome
3) 20-24 week anomaly scan
- > more detailed look at getal growth and looking for major congenital abnormalities and neural tube defects
4) +/- >20 weeks - growth scans (only done if concern for fetal growth or wellbeing)
25
Invasive tests for downs syndrome
How early can they be done and what is the risk of miscarriage with each?
1) Chorionic villous sampling
- Can be done as early as 11 weeks GA.
- overall 3-4% risk miscarriage
2) Amniocentesis
- Can be done as early as 16 weeks GA
- overall 1.5-2% risk of miscarriage
26
What is 'the triple test' ?
It is 3 maternal serum tests used to predict risk for down and other trisomies at 14-22 weeks GA if nuchal scanning is not available
Tests for:
- free beta-hcg - incr in downs
- unconjugated oestradiol - decr
- alpha fetoprotein (AFP) - decr
62% sensitivitity w 5% risk of false positives
27
Indications for the following things in pregnancy:
- folic acid
- oily fish/omega 3
FOlic acid
- reduces incidence of neural tube defects
Oily fish
- neonatal brain development
- reduce risk of preterm labour
28
Definition of twin-twin transfusion syndrome
what sort of twins does it affect?
Which twin is at higher risk?
Monochorionic twins
Share the same placenta
Difference in Hb \>5 between the twins due to one twin hogging the majority of placental blood flow and nutrients -\> becomes large and plethoric vs the othertwin becoming small and anaemic
Larger twin is at higher risk due to reduced
29
Do antenatal steroids reduce rate of IVH?
Yes
30
egg on a string xray = ?
TGA
31
boot shaped heart xray = ?
TOF
32
What is this image of?
What is the pathophys and clinical significance of this condition?
**Periventricular leukomalacia**
Pathophys:
It likely occurs as a result of hypoxic-ischaemic lesions resulting from impaired perfusion at the watershed areas, which in premature infants are located in a periventricular location. It is likely that infection or vasculitis also play a role in pathogenesis.
early: periventricular white matter necrosis
subacute: cyst formation
late: parenchymal loss and enlargement of the ventricles
Clinical significance:
- PVL may manifest as cerebral palsy (\>50% in the setting of cystic PVL), intellectual disability or visual disturbance
- when cystic PVL is present, it is considered the most predictive sonographic marker for cerebral palsy
33
Indication for methenele blue in cyanosed newborns?
Methaemoglobinaemia
34
What do u give cyanosed babies to keep duct open?
Prostaglandin E1 (note - prostaglandin E2 has opposite effect- cuases closure of duct)
PGE1 MOA - prevents the ductus arteriosus from closing, creating an intentional shunt to allow mixing of deoxygenated with oxygenated blood
35
Case
- cyanosed newborn
- cxr showing large heart, oligaemic lung fields
?diagnosis
Pulmonary atresia with intact VSD
36
what sort of blood gas will long-term or high dose furosemide cause?
what medication should be paired with it to reduce risk of this
metabolic alkalosis with low Na and low K, high bicarb
Pair with K sparing diuretic (spironolactone)
37
Effect of antenatal steroids in women at risk of preterm labour?
reduced risk of serious resp illness and death
38
What gut condition is associated with beckwith wiedemann syndrome
Omphalocoele
39
Omphalocele VS gastroschesis.
What is the difference and which is associated with orther organ system anomalies?
Both result of abdominal wall defect. Can be detected antenatally.
In contrast to omphalocele, there is no sac covering the intestines in gastroschisis
Gastroschesis - no membrane covering intestines , isolated anomaly
vs omphalocele - membrane covered. associated with particularly renal, cardiac defects.
40
If you've got a high Co2, what change do you make to the freqyency on HFOV?
Reduce the frequency (greater volume of air displaced)
41
What is this CXR of and what is the appx survival of live births and what side has higher mortality?
is this condition assoc w other chromosomal abnormalities?
CDH
60-70% survival
R sided lesions have higher mortality rate (if liver in chest)
not assoc w chromosomal abnormalities. usually isolated defet.
42
Mec stained liquor in a preterm baby - what infection are you most concerned about and what is the gram stain appearance of this?
What might the sources of this be?
Listeria infection - GP bacilli
Sources - Unpasturised milk, soft cheese
Uncooked raw fruis/veg
Chicken
Over counter reheated foods
Uncooked, smoked meats
43
Blueberry muffin rash is consistent with what neonatal infection?
how is this condition diagnosed and what other clinical features are there?
CMV
Diagnosed on urien or serum PCR
Other clin ft
- Brain - cerebral periventricular calcifications, sensorineural deafness, dev delay, microcephaly, encephalitis
Skin: petechial rash (see above)
Liver: hepatosplenomegaly, jaundice
Lungs: pneumonitis
Eyes: **Chorioretinitis**, optic atrophy
Ears: **SNHL!!**
Teeth: dental defects
44
What is maternal lupus disease assoc with in the neonate?
Neonatal lupus
Congenital heart block
45
What is maternal diabetes assoc w in hte baby?
Macrosomia
Neonatal hypoglycaemia from hyperinsulinism
RDS
Polycythaemia
x2 risk congenital anomaly
Renal vein thrombosis
CHD (VSD, coarct, TGA, hypertrophic subaortic stenosis, hypertrophic obstructive cardiomyopathy)
46
Risk of hyperthyroidism in mother to baby and why does it occur?
What investigations would you do to diagnose this condition in the baby
WHat is the treatment?
Transient neonatal thyrotoxicosis
Mothers - generally on anti-thyroid meds, pmhx Graves
- Occurs secondary to transplacental Ab (TRAb) transfer in maternal graves disease (lasts 6-12 weeks)
- Neonate will have elevated T3, T4 and low TSH + anti-thyroid receptor Ab
Tx - beta blocker for sx control; carbimazole (PTU 2nd line) or radioactive iodine
47
48
Source of toxoplasmosis
Is an intracellular parasite
Vertical transmission from infected mother antenatally
Acquired from
- raw meat
- unwashed fresh fruit/veg
- cat faeces
Clinical features
- Brain: cerebral calcifications, round lesions, seizures, microchaly, dev delay, encephalitis
- Eyes: chorioretinitis, cataracts
- Liver: hepatosplenomegaly, jaundice
- Haem: anaemia
- Lungs: pneumonitis
49
What are the TORCH infections?
Common sx/signs
Toxoplasmosis
Other - Varicella
Rubella
CMV
HSV
Sx
- Fever
- Feeding difficulty
- Petechial rash (CMV, rubella)
- Hepatsplenomegaly
- Jaundice
- Sensorineural deafness (CMV, rubella)
- Chorioretinitis (CMV, toxo)
- Cataracts (Rubella)
- Meningitis/encephalitis
- Brain calcifications (CMV, toxo)
50
What is the highest risk period (to baby) for motehrs to acquire maternal chicken pox
What is the treatment?
Maternal chickenpox around delivery (last 4 weeks but especially \<5 days before delivery or \<2 days after delivery) can cause severe infection in infant due to high viral titres without maternal Ab yet to protect infant
Clnical features
BRAIN - microcephaly, brain aplasia, hydrocephaly
EYS - cataracts, chorioretinitis, microphtalmia
SKIN - zigzag scarring (cicatrix)
LIMBS - limb malformation, shortening, paresis
Tx
- babies are given VZIG (anti-varicella zoster IgG) IM as post-exposure prophylaxis
- IV aciclovir if any vesicles develop
51
Advantages of breast feeding
- Nutrition: improved neonatal brain development
- Immune protection, particularly maternal IgA (protects against resp and GI disease)
- Uterine involution and maternal weight loss expedited
- Contraceptive (lactation amenorrhoea) - as effective as OCP in first 6 months
- Cheap!
- Decr risk of breast cancer x4.3% per year of breast feeding
- Reduced incidence of atopy in childhood and teenage years
- Maternal-infant bonding
52
Risk factors for group B strep sepsis in neonate
Maternal fever
PROM
Preterm infant
Inadequate labour prophylaxis given (penicillin or clindamycin to vaginally colonised mothers)
53
what is this photo of, how is it aquired?
1. Erb palsy ie damage to upper roots C5 +/- 6, 7 -\> often from shoulder dystocia -\> presents with arm/wrist/hand in 'waiters tip' position
54
What is this photo of, how is it typically acquired?
Klumpke palsy
damage to lower nerve roots (C7, 8, T1) of brachial plexus during delivery
Wrist drop and paralysis of small muscles of hand ('claw' hand)
May be assoc w horner's syndrome
55
cephalohaematoma
- what is it?
- mx
- complications
Should self resolve over several weeks
Does NOT cross suture lines
- Is due to bleed beneath periosteum due to torn veins)
Cx
- jaundice
- calcification -\> permanent bump on head
- Assoc IVH
- underlying skull # (20% of cases but rarely needs treatment)
56
What is the most risky time period for acquisition of fetal varicella infection frmo mother?
Acquired from maternal genital tract during delivery (thus C/S has been proven to be effective in limiting transmission in mothers who are actively shedding viral particles)
- Primary maternal infection during pregnancy is most risky - up to 50% babies affected
- Recurrent maternal infection during pregnancy - only 3% babies affected
57
Fetal varicella infection clinical presentation
1. Skin/eyes/mouth - limitation of rash/vesicles to these areas.
2. CNS - encephalitis +/- skin
3. Disseminated disease - multi-organ failure. viral sepsis with resp, hepatic failure and DIC +/- CNS, skin, eye, mouth
58
Conjunctivitis in the newborn period
- ddx and how to distinguish clinically
Chlaymdia vs gonococcal
Both purulent
Chalmydia presents in 2nd week of life
Gonococcal presents days 1-2 of life
59
Definition and Treatment of Omphalitis
Complications
Definition: infection of umbilical stump
Treatment: MCS swab of umbi
Gentle cleansing
IV abx if signs of cellulitis around umbi
Cx - portal vein infection and subsequent portal HTN
60
Metabolic acidosis/alkalosis picture
vs Respiratory
Met acidosis - low pH, low bicarb (sick infant)
Met alkalosis - high pH, high bicarb (GI losses/vom/diarr, diuretics, renal/Bartters/Gitelmans)
Resp acidosis - low pH, high Co2 (underventilated)
Resp alkalosis - high pH, low Co2 (overventilated)
61
What is continuous mandatory ventilation?
Form of IPPV - full ventilation to baby
62
What is IMV (intermittent mandatory ventilation)?
Form of IPPV
Only occasional breaths gienv by ventilator
USed to wean babies who are making some resp effort off the ventilator
63
What is patient triggered ventilation (PTV)?
Form of IPPV
Ventilator assists breaths after they are triggered by baby - used to wean babies off ventilator
64
Indication for nitic oxide and MOA
SE
MOA - vasodilator that acts on pulmonary artery smooth muscle via increasing cGMP levels
Indication: PPHN to decrease pulm artery hypertension
SE - methaemoglobinaemia and can alter plt function
65
IUGR
- Causes of asymmetrical growth failure
IUGR: growth \<3rd centile for dates
Asymmetrical: weight \< HC centile due to relative sparing of brain
- Cause: placental failure LATE in pregancy -\> growth post birth accelerates
- \> pre-eclampsia, multi gestation, maternal cardiac/renal disease affecting blood supply to placenta, uterine malformation
66
IUGR - causes of symmetrical growth failure.
Symmetrical: HC, weight, length all equally affected
- Cause: CHRONIC intrauterine growth failure -\> growth post birth continues to be poor
- Cause: smoking, malnutrition, chornic disse; chromosomal disorder, congenital infection
67
Definition of large for gestational age and causes
LGA weight \> 90th centile
Causes
- diabetic mother
- Familial (large parents)
- Beckwith-wiedemann syndrome
68
Problems associated with prematurity
Temperature instability
Brain - HIE, intracranial haemmhorage, lack of primitive reflexes (suck)
Lungs - apnoeas, RDS, pneumothorax, haemmhorage, BPD
Heart - PDA, PPHN
GI - feeding intolerance, GORD, NEC
Liver - Jaundice
Kidneys - impaired concentration ability, impaired acid excretion
Immunity - impaired immune system, susceptible to infx
Eyes - ROP
Metabolic - low sugars, low Ca, osteopaenia of prematurity
Haem - anaemia
Surgial - hernias
69
What is RDS?
Definition
RF
Protective
Clinical features
CXR findings
Mx
Defintion: Resp distress caused by insufficient surfactant
=\> Non-compliant/stiff lungs -\> hypoxia and resp aciodis -\> severe cases can lead to PPHN
RF - prem, hypoxia, acidosis, shock, asphyxia, diabetic mother, APH, second twin, male
Protective - maternal steroids (given to mohers 48hrs before delivery if prem delivery expected)
Clnical ft
- Resp distress from 6 hours of age
- Worsens over 2-3 days then improves over 1-2 weeks
CXR: ground glass apperaance, air bronchograms
Mx:
- surfactant via ETT or MIST
- resp support: O2, CPAP or positive pressure ventilation
+/- abx
- Minimal handling
70
Surfactant - what is it produced by and what is its role?
At what age does it begin to be produced?
Lowers surface tension and increases compliance in alveoli -\> decreased pressure required to open alvoeli
Produced by type II pneumocytes
Produced around 24 weeks of age
71
What is bronchopulmonary dysplasia?
Definition
Clinical features
CXR
Mx
Now known as CLD
Chronic lung damage with persistent Xray changes secondary to severe RDS or other neonatal lung disease
Persisting O2 requirement on day 28 of life or at 36 weeks gestation
Clinical features:
- Chest hyperinflation, WOB (IC and SC recession)
- Lung Creps
CXR: honeycomb lung (cystic pulm infiltrates in reticular pattern), areas of emphysema, collapse and fibrosis and thickening of pulonary arterioles
Mx
- Steroids and diuretics if vnetilator dependent
- Bronchodilators if wheezy
+/- home O2
72
Wilson MIkity Syndrome
A form of BPD
- Respiratory distress (WOB, hypoxia, apnoea) that develops SLOWLY over the first month in PREMATURE/LWB (\<1.5kg) babies who have no history of severe resp distress earlier in life
Characteristic CXR findings: streaky infiltrates and cysts
73
TTN:
Definition
Cause
RF
CXR
Tx
Definition: resp distress at birth resolving over FIRST 24HRS
Due to excess retained fetal lung fluid -\> self-limiting cdtn
RF: LUSCS, diabetic mother, heavy maternal analgesia
CXR: streaky, fluid in fissures, pleural effusions
Tx - supportive (O2) +/- abx
74
Common causative agents for neonatal pneumonia
Group B haemolytic strep
GNB ( e coli, klebsiella, pseudomonas, serratia)
Staph saprophyticus
Rarer - lysteria, chlamydia, mycoplasma, CMV, coxsackie, RSV
75
What can inhaled meconium result in
Airway plugging with distal atelectasis, air leaks
Secondary pneumonia
Chemical pneumonitis (toxic to lung tissue)
Hypoxia, resp/metabolic acidosis
PPHN if severe
76
CXR features of Mec aspiration syndrome
Hyperinflation
Diffuse patchy opacification
77
Causes of neonatal cyanosis
Cardiac - cyanotic CHD (tof, ta, pa, ebstein anomaly, tga, tapvd, hypoplastic l heart, double inlet ventricle)
Respiratory - severe lung disease, PPHN
- airway obstruction
Brain - asphyxia, seizures, neuromuscular
Haem - methaemoglobinaemia
78
PPHN - Definition/Cause
Clinical features
Gas finding
CXR findings
ECho findings
Mx
IE 'Persistent foetal circulation' - failure of pulmonary vascular resistance to fall after birth
Results in blood continuing to be shunted AWAY from lungs (high pressure -\> lower pressure systemic) via ductus arterius and foramen ovale (R -\> L shunt)
Clinical features:
- Central cyanosis
- LOUD P2
- Little impr in sats with 100% O2
- Preductal paO2 \> 5mmHg than postductal paO2
Gas: low pO2, relaitvely normal pCO2
CXR: NORMAL heart size, oligaemic lung fields
Echo: structurally NORMAL heart (not CHD), high pulmonary art pressures
Mx - resp support, NO
79
Signs of fetal distress inutero
Fetal bradycardia with reduced variability
Late decelerations
Reduced fetal movements
Meconium
80
What is HIE?
Clinical features
Prognosis
Hypoxic ischaemic encephalopathy
- results from ischaemic injury to brain
- affects WATERSHED zones between major arteries as these are most susceptible to hypoperfusion
Clinical features: develops over a few days - low apgars, floppy, seizures and irreg breathing
Incr risk of ID, seizures, cerberal palsy and death in severe disease.
81
Causes of HIE
Maternal - pre-eclampsia, eclampsia, hypotension
Palcental - abruption, cord prolapse, insufficiency
Foetal - prem, post-dates, obstructed labour (breech, shoulder dystocia)
82
Commonest type of intracranial haemorrhage in neonates?
Cause and location of these bleeds?
Timing
Risk factors
Name: **Periventricular haemorrhage (PVH)**
Location :The highly vascularised **subependymal germinal matrix** (at head of caudate nucleus)
Cause: Unstable cerebtral circulation (blood vessels of the germinal matrix are weak-walled and predisposed to haemorrhage). Significant stress experienced by a premature infant after birth may cause these vessels to rupture into periventricular areas and may progress to IVH (grades 2-4).
Timing: Usually occur within **1st week of life** (90% in first 4 days) in *premature/LBW infants*
Other RF:
* - RDS*
* - IPPV*
* - Metabolic acidosis*
* - Hypercapnoea*
* - Coagulation disorder*
83
Grading/classification of Perivenricular haemorrhage
- Grade 1 -4
Grade 1: periventricular/subependymal bleed (confined to seminal matrix)
GRade 2: IVH (extension into lateral ventricle WITHOUT ventriculomegaly)
Grade 3: IVH with ventriculomegaly
Grade 4: Bleeding into brain parenchyma
84
Treatment and Complications of intracranial bleeds
Tx - supportive
Cx
- Hydrocephalus (may require supportive shunt)
- Porencephaly (cyst or cavity filled with cerebrospinal fluid develops in the brain - see image)
- Cerebral palsy
85
Causes of jitteriness
- Hypoglycaemia
- Hypocalcaemia
- Sepsis
- Drug withdrawal (opiates, benzos in particular; also SSRIs eg sertraline)
86
How to distinguish between seizures and jitteriness clinically?
**Jitteriness** - Rhythmic movements
Can occur when baby is alert or asleep
Normal eye movements
Movement stops when limb is held.
**Seizures** - Can be multifocal, w tone alteration +/- apnoea
Altered conscious state
Eye deviation
Movements do NOT stop when limb is held
87
Causes of neonatal seizures
Congenital brain anomaly
Other brain stuff- CVA, subarachnoid haemorrhage
Asphyxia/respiratory
Infection/sepsis/meningitis
Metabolic/electrolyte disturbance (low glucose, low Ca, low Mg)
Inborn error of metabolism (pyridoxine deficiency etc)
88
Management of opiate withdrawal in neonates
Opiates - morphine weaning regime (sometimes use other sedatives such as diazepam, chlorpromazine) over several weeks
Do NOT give naloxone - can induce immediate withdrawal which can be life threatening
89
Short and long term complications of NEC
Short - perforation, obstruction, gangrenous bowl, intrahepatic cholestasis, sepsis, DIC
Long - stricture, short bowel syndrome (resection of diseased bowel), lactose intolerance
90
Meconium ileus - clinical presentation and significance
Intestinal obstruction of **terminal ileum** due to THICK INSPISSATED MEC
Failure to pass mec within 48hrs of birth
Clinical features of obstruction - bilious vomiting, abdo distension
AXR: dilated loops of bowel centrally (small intenstine) WITHOUT air fluid levels. Bubbly appearance of intestinal contents in the right lower quadrant.
COntrast enema for diagnosis - microcolon; contast unable to get past point of transverse colon
10% of CF cases present with this
91
What is osteopaenia of prematurity caused by and what investigations support its diagnosis?
Premature and IUGR/VLBW kids are susceptible:
- Mineral deficiency (particularly phosphate) due to
- Placental insufficiency
- Inadequate Ph levels in breast milk or feeds
- Meds: Long-term steroids or diuretics
Ix =
- Phosphate L
- PTH H (response to low Ph)
- ALP H (released from resorbing bone w high bone turnover)
- Note: Ca can be N/L/H
Mx
- Vitamin D
- Low BW formula (contains more Ph)
- Ph supplements if breast feeding
92
Hydrops - Severe oedema, ascites and pleural effusions at birth
**Causes**
1. Intrauterine anaemia due to
- \> severe haemolytic disease of newborn (Rh, blood group incompatibilities)
- \> twin-twin transfusion syndrome
- \> fetomaternal haemorrhage
- \> Hb barts
2. Congenital infection (parvovirus B19)
3. Cardiac failure
4. Hypoproteinaemia (congenital nephropathy Finnish, Maternal pre-eclampsia)
5. Congenital malformations such as obstructive uropathy
93
What is the kleihauer test used for?
Used to diagnose and quantitate a fetomaternal hemorrhage
Looks for foetal cells (HbF) in maternal circulation
May be done in case of hydros foetalis or in case of foetal anaemia (if mother is Rh neg and baby Rh positive as these are incompatible)
94
5Ts of cyanotic CHD
Tetralogy of Fallot
Transposition of the great vessels
Tricuspid valve anomalies
Total anomalous pulmonary venous return
persistent Truncus arteriosus
Also
Hypoplastic L heart
Ebstein anomaly
95
What is this condition?
Boot shaped heart
= TOF
**RVOTO**
Right ventricular hypertrophy
Ventricular septal defect
Overriding aorta are the characteristics of
Tetralogy of FallOt.
96
What is this condition?
Transposition of the great arteries
'egg on a string'
Narrow mediastinum (due to stress induced mediastinal atrophy)
globar large heart on its side
97
SIMV
Delivers SET amount of breaths to neonate that are supported by ventilator
Synchronised w pt's own breaths
If pt breaths ABOVE set rate, those breaths are not supported by ventilator (useful when weaning ventilation)
98
SIPPV/PCAC
Supports **every breath** the infant makes (even if breaths above set number of delivered breaths)
The set ventilator respiratory rate is the backup number of breaths that will be mechanically administered if the infant makes no spontaneous breaths
99
Volume guarantee
This mode of ventilation can be used with SIMV or SIPPV.
The ventilator aims to deliver tidal volumes (VT) *[=volume guarantee)* set by the clinician. A maximum peak inspiratory pressure (Pmax) is set, the ventilator’s PIP will vary to reach the target volume
VG = Usually around 4-5ml/kg
Pmax: In the volume-controlled mode this is the maximum peak inspiratory pressure you wish the ventilator to administer to reach target tidal volumes. Usually set 5 cmH2O higher than the average PIP used to achieve the set tidal volume.
Benefits are that it reduces the:
- Duration of ventilation
- Risk of pneumothorax
- Risk of grade 3/4 intraventricular haemorrhage,
- Risk of chronic neonatal lung disease.
100
INdications for surfactant administration
Reccomended in:
- Neonates with clinical and radiographic evidence of RDS
- neonates at risk of developing RDS (e.g. \<32 weeks or low birth weight \<1300g)
- neonates who are intubated, regardless of gestation, and requiring FiO2 \>40%
Considered in:
- Severe meconium aspiration syndrome with severe respiratory failure – may improve oxygenation and reduce the need for extracorporeal membrane oxygenation (ECMO)
- Pulmonary haemorrhage with clinical deterioration
- Severe RSV-induced respiratory failure - may improve gas exchange and respiratory mechanics and shorten the duration of invasive mechanical ventilation
101
projectile vomiting in 6-8 week old hungry infant ?diagnosis
?picture on gas
Pyloric stenosis
Hypocl hypoK metabolic alkalosis
102
Bilious vomiting in baby with trisomy 21
?diagnosis
?AXR finding
Duodenal atresia
See double bubble on AXR
103
Sudden bilious vomiting in neonate with abdominal distension and tenderness and followed by haematemesis
?diagnosis and how to you diagnose this
?mgmt
Malrotation and volvulus
UGI contrast study is diagnosistic
Surgical emergency - ischaemia can lead to small bowel infarction req surgical resection -\> extensive resection = poor prognosis
104
BIlious vomiting, abdo distension and failure to pass mec in a neonate
?diagnosis
?what do you need to check for in these pts
Mec ileus
CHeck for CF (95% of infants with mec ileus have underlying CF)
ABdo xray findings - dialted loops of bowel, air fluid levels, ground glass soap-appearance of mec. may see intraabdominal calcification
105
Causes of Cerebral palsy
Prematurity (80%) - Periventricular leukomalacia
IUGR (34%)
Intrauterine infection (30%)
Antepartum haemorrhage (27%)
Severe placental pathology (21%)
Multiple pregnancy (20%)
106
Most likely outcome of PVL at 18 months
Unable to walk independently -\> progression to spastic diplegia or quadriplegia
107
Renal function in preterm vs term infant
- preterms have **lower GFR** (10 vs 30). Reach adult GFR (120) in first 2 years of age.
- renal function is optomised for **RETENTION of dietary solutes** for growth, not for excretion
- **reduced urine concentration** ability in preterms
- preterm kidney **LOSES Na** (prone to hypoNa) whereas the term kidney conserves N
- Preterms have r**educed ability to RESORB bicarb** (therefore prone to low bicarb/met acidosis due to bicarb wasting)
- **Reduced ability to EXCRETE K** (prone to hyperK)
108
Causes of HTN in the neonate
1. Renovascular
- UAC assoc thrombus (forms on tip of catheter)
- REnal vein thrombosis (flank mass, gross haematuria, thrombocytopaenia)
- Renal artery stenosis (Fibromuscular dusplasia msot common; NF and Williams syndr)
2. Renal parenchymal
- PCKD (ARPKD and ADPK)
- AKD
- Nephrocalcinosis (if obstructive calculus forms)
- Obstructive uropathy
3. Cardiovascular (coarctation of aorta)
4. Bronchopulmonary dysplasia (mechanism unclear)
5. Endocrine (hyperthyroid)
109
Pierre Robin Sequence
What is it
Mgmt
Due to mandibular hypoplasia (occurs \< 9 weeks of development)
**Features include:**
* Micrognathia
* Cleft palate
* Posteriorly displaced tongue -\> can lead to airway obstruction and feeding difficulties
**Management**
* Prone positioning
* NPA
* May need CPAP
* Need palatal surgery by 1 year of age
* Feeding can be problematic - can feed with special teats/bottle tops but may require NG feeding initially
110
Infant of hyperparathyroid mother
-What to expect postnatally?
Mother with hypoparathyroidism -\> hypercalcaemia if not poorly controlled
Transplacental transport of calcium -\> foetal hypercalcaemia
Foetal hypercalcaemia -\> suppression of PTH secretion
Postnatally, hypocalcaemia ensues
111
Infant of mother with Graves, what to expect post-natally?
Transplacental passage of TSHR-Ab causing hyperthyroidism
112
Acute prognosis - at risk of:
- birth asphyxia
- hypoglycaemia
- hypothermia
- polycythaemia
- mortality
Chronic prognosis
- Most show catch yp growth but some will remain short and thin
- Incr risk of learning difficulties
- incr risk of chronic diseases DMT2, CHD, HTN, stroke
-
113
Causes of IUGR
**Fetal**
- chromosomal abnorlities (T21, 18 etc)
- structural malformations
- congenital infx (CMV, toxoplasmosis, rubella)
**Maternal**
- Malnutrition
- Maternal hypoxia (CHD, chronic resp disease, altitude)
- Drugs, alchohol, smoking etc
**Placental**
- Compromised vascular supply - Pre-eclampsia, HTN, DM, renal disease
- Thrombosis, infarction - maternal SLE, anti-PLD syndrome, sickle cell anaemia
- Sharing of uterine vasculatiy (multi-gestation)
114
HFOV vs PCOC in terms of outcomes/prognosis
No effect on mortality at 28-30 days of life, or at term equivalent age
Significant reduction in CLD in survivors at term equivalent GA in the HFOV group, however to an inconsistent degree
Increased risk of pulmonary air leaks (pneumothorax, pneumediastinum, pulmonary interstitial emphysema, pneumopericardium) in the HFOV group
Reduced risk of severe retinopathy of prematurity
No difference in severe grade intracranial haemorrhage or periventricular leukomalacia
Potential reduction in the risk of cerebral palsy and poor mental development
