Neonatology Flashcards
1
Q
What is neonatal sepsis?
A
- Bacterial or viral infection in the blood that affects babies within the first 28 days of life
2
Q
How is neonatal sepsis catergorised?
A
- Early onset- within 72 hours of birth
- Late onset- between 7-28 days of life
3
Q
Causes of neonatal sepsis?
i.e. bugs
A
- GBS and E.coli
- Early onset: GBS is most common cause
- Late onset: Staphylococcus epidermis, Pseudomonas, Klebsiella and Enterobacter
- Less common causes incl: Staph aureus, Enterococcus, Listeria, Viruses e.g. herpes and enterovirus
4
Q
Risk factors for sepsis in the neonate?
A
- Mother who had previous baby with GBS infection, current GBS colonisation from prenatal screening, current bacteruria, intrapartum temperature greater than or equal to 38 degrees, membrane rupture greater than or equal to 18 hours, or current infection throughout pregancy
- Premature (< 37 weeks)
- Low birth weight (< 2.5kg)
- Evidence of maternal chorioamnionitis
5
Q
Presentation of neonatal sepsis?
A
- Respiratory distress (grunting, nasal flaring, use of accessory resp muscles, tachypnoea)
- Tachycardia
- Apnoea
- Apparent changes in mental status/lethargy
- Jaundice
- Seizures- if meningococcal
- Poor/ reduced feeding
- Abdo distension
- Vomitting
Clinical presentation can vary from being very subtle signs of illness to clear septic shock
Frequently, symptoms will be related to source of infection e.g. pneumonia+ resp symptoms
6
Q
What investigations would you do for neonatal sepsis?
A
- Blood cultures- ideally 2 to distinguish from contamination
- FBC- associated with abnormal neutrophil count (neutropenia or neutrophilia), but otherwise used to distinguish from healthy neonates
- CRP- usually raised (persistently normal will be used to exclude sepsis)
- Blood gases- metabolic acidosis is concerning, esp with a base deficient of greater than/equal to 10mmol/L
- Urine MC&S- more useful in late onset (rarely positive in early onset), will show signs of infection e.g. raised leukocytes, haematuria,positive culture etc.)
- Lumbar puncture: particularly if worried about meningitis
7
Q
Management of neonatal sepsis?
A
- IV benzylpenicillin with gentamicin
- Re-measure CRP 18-24 hours after presentation to monitor progress
- Abx can be ceased at 48 hours in neonated who have CRP of < 10mg/L and a negative blood culture at presentation and at 48 hours
- Other neonates- duration depends on severity etc. usually around 10 days
- Maintain adequate oxygenation status
- Maintain normal fluid and electrolyte status- if v ill may need volume and/or vasopressor support
- Prevention/ management of hypoglycaemia
- Prevention and/or management of metabolic acidosis
8
Q
What is neonatal hypoglycaemia?
A
- < 2.6 mmol/L is used in many guidelines
- Transient hypoglycaemia in the first hours after birth is common
9
Q
What causes persistent/ severe neonatal hypoglycaemia?
A
- Pre-term birth
- Maternal diabetes mellitus
- IUGR
- Hypothermia
- neonatal sepsis
- Inborn errors of metabolism
- Nesidioblastosis
- Beckwith-Wiedemanna syndrome
10
Q
Features of neonatal hypoglycaemia?
A
- May be asymptomatic
- Autonomic
- Jitteriness
- irritable
- tachypnoea
- Pallor
- Neuroglycopenic
- Poor feeding/sucking
- weak cry
- drowsy
- hypotonia
- seizures
- other features
- apnoea
- hypothermia
11
Q
Management of neonatal hypoglycaemia?
A
Asymptomatic:
* encourage normal feeding
* monitor blood glucose
Symptomatic or very low blood glucose
* admit to neonatal uni
* IV infusion of 10% dextrose
12
Q
What is hypoxic ischaemic encephalopathy?
A
Occurs in neonates
Due to hypoxia during birth
Hypoxia = lack of O2
Ischaemia = restricted blood flow to brain
Encephalopathy = malfunctiong of brain (i.e. brain damage)
13
Q
What examination/ investigation findings would make you suspect HIE?
A
- umbilical artery blood gas (ABG) showing acidosis (pH = < 7)
- poor Apgar scores on examination
- evidence of multi organ failure
14
Q
What are causes of HIE?
A
Anything that leads to deprivation of oxygen to the brain:
* placental abruption pre partum
* maternal shock
* intrapartum haemorrhage
* prolapsed cord –> causes cord compression during birth
* nuchal cord (cord wrapped around baby’s neck)
* prolonged respiratory arrest post partum
15
Q
How does HIE present? Group into mild, moderate and severe presentation
A
Presentation varies on the severity of cerebral hypoxia
Generally - baby will be unwell from birth, need resuscitation
Encephalopathy develops within 24hrs of birth
16
Q
What investigations are done following suspected HIE?
A
EEG - monitor brain activity
Multiple MRI brain scans
17
Q
How is HIE managed?
A
- specialist management in neonatal unit
- supportive management
- resuscitation and optimal ventilation
- acid base balance
- monitor and treat seizures
- theraputic hypothermia
- follow up by paeds and MDT to assess development and support any lasting disability
18
Q
Describe theraputic hypothermia in HIE
Describe process and the aim of this therapy
A
- where you actively cool core temp of baby based on a protocol
- target 33-34 degrees C - measured using a rectal probe
- continue for 72hrs
- then warm baby to a normal temp over 6 hrs.
- Aim = reduce inflamaion and neurone loss after an acute hypoxic injury. Reduces risk of cerebral palsy, developmental delay, learning disability, blindness and death
19
Q
What is prematurity?
A
Birth before 37 completed weeks’ gestation (8% of all births).
The WHO classify prematurity as:
* Under 28 weeks: extreme preterm
* 28 – 32 weeks: very preterm
* 32 – 37 weeks: moderate to late preterm
Most problems seen in infants born <32 completed weeks
20
Q
What are some predisposing factors for prematurity?
A
- Idiopathic (40%).
- Previous preterm birth.
- Multiple pregnancy.
- Maternal illness, e.g. chorioamnionitis, polyhydramnios, pre-eclampsia, diabetes mellitus.
- Premature rupture of membranes.
- Uterine malformation or cervical incompetence.
- Placental disease, e.g. dysfunction, antepartum haemorrhage (APH).
- Poor maternal health or socio-economic status.
21
Q
What are some problems / complications that happen with premature neonates early in life?
lots! thing resp, CNS, GI etc.
A
Respiratory - surfactant deficiency:
* respiratory distress (RDS)
* apnoea of prematurity
* chronic lung disease
CNS:
* intraventricular haemorrhage
* retinopathy of prematurity
GI:
* Necrotizing enterocolitis
* Inability to suck
* poor milk tolerance.
other systems:
* Hypothermia.
* Immunocompromise with ↑ risk/severity of infection.
* Impaired fluid/electrolyte homeostasis (↑ transepidermal water loss, poor renal function).
* Patent ductus arteriosus
* Anaemia of prematurity
* Jaundice (liver enzyme immaturity)
* Birth trauma
* Perinatal hypoxia (Hypoxic–ischaemic encephalopathy)
22
Q
What are some long term problems that might effect a premature neonate?
A
- Chronic lung disease of prematurity (CLDP)
- Susceptibility to infections, particularly respiratory tract infections
- adverse neurodevelopmental outcome e.g. cerebral palsy
- hearing and visual impairment e.g squint + retinopathy
- behavioural and learning problems
- sudden infant death syndrome (SIDS),
- non-accidental trauma (NAT), and/or parental marriage break-up.
zero to finals and ox handbook of paeds
23
Q
What are some associated factors for prematurity ?
e.g. environment, maternal factors (zero to finals)
A
- Social deprivation
- Smoking
- Alcohol
- Drugs
- Overweight or underweight mother
- Maternal co-morbidities
- Twins
- Personal or family history of prematurity
24
Q
Antenatal management of prematurity?
Dr Tom if preterm labour is suspected or confirmed what can you do to im
A
- planned delivery in centre that has preterm care facilities
- Tocolysis with Nifedipine (CCB - suppresses labour)
- IM maternal corticosteroids <34 -35 weeks ( reduced RDS, pervientricular hameorrage and NEC) if given >24 hours before birth
- IM magnesium sulphate - <34 weeks to protect babys brain
- delayed cord clamping or cord milking - increase circulating blood colume and hb in baby
25
Dr Tom: In women with a history of preterm birth or an ultrasound demonstrating a cervical length of 25mm or less before 24 weeks gestation there are two options of trying to delay birth: what are they?
* Prophylactic vaginal progesterone: putting a progesterone suppository in the vagina to discourage labour
* Prophylactic cervical cerclage: putting a suture in the cervix to hold it closed
26
Postnatal care of premature infants?
* Stabilisation (or resuscitation if needed)
* senior paediatrician at birth <28 weeks
* delay cord clamping 1 min if not compromised
* immediately place in food grade plastic bag under radiant heater w/ woollen hat
* Resp support NIV, MV as required
* measure weight and temp on NICU admission and monitor
* Nurse in 80% humidity for first 7 days if <30 weeks
* monitor blood glucose with feeds, encouarge mothers to breast express from day 1
* start broad spectrum antibiotics if suspect infection
* treat complications of prematurity e.g. surfactant for RDS
* support parents e.g. www.bliss.org.uk
27
In additon to immediate post birth resuscitation what 4 things are you trying to reduce to reduce potentatial morbidity of a premature neonate?
reduce excessive oxygen exposure, hyperventilation, hypothermia, and hypoglycaemia
28
Why is gestational age so important to re-assess with a premature infant, how?
important for:
* designating future care of newborn
* adverse outcome risk varies inversley with gestational age
How measure?
* Maternal last menstrual period
* EDD on 12 week first trimester ultrasound (most accurate) crown-rump length, fetal femur length, head and abdominal circumference, biparietal diameter.
* New Ballard score - measures neuromuscular and physical maturity to complement maternal histoty and US.
29
If you examined a extremely premature baby (<28 weeks) what would you expect to find?
* weight
* tone / posture
* hair / skin / anatomical features
| see BMJ BP 'premature newborn care' other premature stages
* weight is typically <1000 g.
* decreased tone, extended rather than flexed extremity posture with a wide range of motion throughout.
* little lanugo hair coverage
* thin transparent/pink skin with visible veins
* limited or no plantar creases
* flat areolae with nearly imperceptible breast tissue
* fused or open eyelids
* folded ear pinna cartilage
* preterm genitalia (male: scrotum empty with faint or no rugae, female: prominent clitoris and small or flat labia minora).
30
Differencials for prematurity
Small for gestational age
intrauterine growth restriction
31
What are two main causes (subgroups) of neonatal jaundice?
1. increased production of bilirubin
2. decreases clearance of bilirubin
32
What are causes of neonatal jaundice based on increased bilirubin production?
* Haemolytic disease of the newborn
* ABO incompatibility
* Haemorrhage
* Intraventricular haemorrhage
* Cephalo-haematoma
* Polycythaemia
* Sepsis and disseminated intravascular coagulation
* G6PD deficiency
33
What are causes of neonatal jaundice based on decreased clearance of bilirubin?
* Prematurity
* Breast milk jaundice
* Neonatal cholestasis
* Extrahepatic biliary atresia
* Endocrine disorders (hypothyroid and hypopituitary)
* Gilbert syndrome
34
# in neonates
When is jaundice pathological?
in first 24 hours of life
Note: jaundice in neonate 2-14 days = common, usually physiological
35
What are RF associated to significant neonatal jaundice?
* preterm delivery
* low birth weight
* having jaundice in first 24hrs of life
* male
* visible bruising
* cephalhaematoma
* maternal age over 25
* maternal DM
* breastfeeding
* having previous babies born with jaundice and needing phototherapy
* ethnicity - asian, european, native american
* dehydration
* neonatal weight loss / poor feeding
36
Prevalence of neonatal jaundice?
* 60% term and 80% preterm in 1st week of life
* 10% breastfed babies jaundiced at 1month
37
What is prolonged jaundice in:
1. full term babies
2. preterm babies
1. 14 days
2. 21 days
38
What investigations would you do for neonate presenting with prolonged jaundice?
FBC and blood film
* high or low WCC or thrombocytopenia = potential sepsis
* low HB / haematocrit = anaemia (potential haemolytic anaemia)
* increased reticulocytes = haemolysis
* blood film = haemolysis
Blood group - for mum and baby
* ABO incompatibility
* rhesus incompatibiltiy
Direct Coombs test (direct antiglobulin test)
* haemolysis
Bilirubin
* high levels of conjugated bilirubin = hepatobiliary cause of jaundice
LFTs
* hepatobiliary cause
G6PD levels
* G6PD deficiency
Blood cultures and urine for M,S, U
* looking for source of sepsis
Thyroid function test
* hypothyroid
39
Causes of prolonged jaundice?
1. biliary atresia
2. hypothyroidism
3. galactosaemia
4. urinary tract infection
5. breast milk jaundice
* jaundice is more common in breastfed babies
* mechanism is not fully understood but thought to be due to high concentrations of beta-glucuronidase → increase in intestinal absorption of unconjugated bilirubin
6. prematurity
* due to immature liver function
* increased risk of kernicterus
7. congenital infections e.g. CMV, toxoplasmosis
40
What causes physiological jaundice?
Elevated bilirubin due to:
* babies being polycythaemic at birth (having lots of RBC at birth so break lots down)
* short RBC life span compared to adults
* hepatic bilirubin metabolism is less efficient in first few days of life
41
Why are breastfed babies more likely to have jaundice?
Componenets of breast milk inhibit liver to process bilirubin.
42
Why may premature neonates have jaundice?
Immature liver - so can not process bilirubin.
43
Why may premature neonates have jaundice?
Immature liver - so can not process bilirubin.
44
Why may premature neonates have jaundice?
Immature liver - so can not process bilirubin.
45
Describe pathophysiology of haemolytic disease of the newborn
* caused by incompatibility between rhesus antigens on surface of RBC of mother and fetus.
* woman who is Rh D -ve becomes pregnant. Her child may be Rh D +ve.
* at some point, blood from baby can enter mum's bloodstream
* baby's red blood cells (in mum's blood) display the Rh D antigen
* mum's immune system recognise this as FOREIGN --> so make antiodies to the antigen = mum become sensitised to Rh D antigens!!
This does not case a problem until second pregnancy, or in antepartum haemorrhage.
Subsequent pregnancies:
* mother's **anti-D antibodies** cross the placenta into the fetus
* if this second fetus is RhD +ve = antibodies attach to RBCs of the fetus = immune system of the fetus attacks own RBCs.
* Sooooo.... get haemolysis = anaemia and high bilirubin level.
This is called haemolytic disease of the newborn
46
What should you ask for Hx and Ex in neonate presenting with jaundice?
* obstetric Hx including mum's Rhesus status and blood group, baby's gestational age at birth
* age at onset and duration of jaundice
* feeding history
* number of wet or dirty nappies per day - ask about dark urine and pale stools
* signs of illness - vomiting, fever, lethargy, weight loss, irritability
* family hx of relevant conditions - e.g. G6PD deficiency. Have any siblings or close family needed phototherapy or blood transfusion for neonatal jaundice
On examination:
* signs of illness - fever?
* weight gain
* bruising
* jaundice - usually spreads from head downwards with increasing severity
47
How is neonatal jaundice ***initially*** managed?
| i.e when do you send baby in and with what urgency?
Emergency admission (999) if have jaundice with features of bilirubin encephalopathy (atypical sleepiness, poor feeding, vomiting, hypotonia followed by hypertonia)
Urgent admission to neonatal or paeds unit in 2 hours if jaundice appears less than 24hrs of age
Urgent admission to neonates or peads to be seen in 6 hours if:
* jaundice appears after 7 days old
* neonate is unwell
* gestational age < 35 weeks
* prolonged jaundice is suspected
* concerns about weight/ feeding problems
* pale stools or dark urine
* reassure parents and carers and encourage breastfedding mothers to continue
48
How is neonatal jaundice managed (once baby is in secondary care and has been initially managed)?
Total bilirubin levels are monitored and plotted on a treatment threshold chart. Depending on level, management is decided.
* level below treatment threshold = no treatment needed
* **phototherapy**
* **exchange transfusion **- usually indicated if baby has signs of bilirubin encephalopathy / jaundice doesn't respond to phototherapy. (Exchange transfusions involve removing blood from the neonate and replacing it with donor blood.)
* early surgical treatment - if biliary atresia.
* treat any underlying illness, e.g. infection.
49
What is phototherapy?
| for neonatal jaundice
* converts unconjugated bilirubin into isomers that can be excreted in the bile and urine without requiring conjugation in the liver.
* involves removing clothing down to the nappy to expose the skin and eye patches to protect the eyes.
* Blue light is the best at breaking down bilirubin.
* A light-box shines blue light on the baby’s skin. Little or no UV light is used.
* Bilirubin is closely monitored during treatment.
* Once phototherapy is complete, a rebound bilirubin should be measured 12 – 18 hours after stopping to ensure the levels do not rise about the treatment threshold again.
50
What are complications of neonatal jaundice?
* Kernicterus = rare but serious complication of untreated jaundice. High levels cause brain damage (esp basal ganglia). (on NICE, this is a clinical term to describe both acute and chronic bilirubin encephalopathy - i.e. the features and the long standing effects)
* Acute bilirubin encephalopathy = severe hyperbilirubinaemia
* Chronic bilirubin encephalopathy (features left behind due to acute bilirubin encephalopathy)
51
Why can high bilirubin levels cause damage to CNS?
Bilirubin can cross blood brain barrier
52
What are features of kernicterus
| complication of neonatal jaundice
less responsive, floppy, drowsy baby with poor feeding.
| Note: this complication is rare nowadays
53
What permenant damage is caused by kernicterus (having v high bilirubin levels)?
Cerebral palsy
Learning disability
Deafness and vision problems
Seizures
54
What are features of G6PD deficiency?
* neonatal jaundice is often seen
* intravascular haemolysis
* gallstones are common
* splenomegaly may be present
* Heinz bodies on blood films. Bite and blister cells may also be seen
55
What is inheritence pattern of G6PD deficiency?
X linked recessive
56
What is pathophysiology of G6PD deficiency?
i.e. how does it cause haemolytic anaemia (presents as neonatal jaundice)
* G6PD is the first step in the pentose phosphate pathway, which converts glucose-6-phosphate→ 6-phosphogluconolactone
* this reaction also results in nicotinamide adenine dinucleotide phosphate (NADP) → NADPH
i.e. glucose-6-phosphate + NADP → 6-phosphogluconolactone + NADPH
* NADPH is important for converting oxidizied glutathine back to it's reduced form
* reduced glutathine protects red blood cells from oxidative damage by oxidants such as superoxide anion (O2-) and hydrogen peroxide
* ↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress
57
What are triggers for G6PD deficiency?
Intercurrent illness or infection (often forgotten)
Fava beans: the disease was historically known as favism
Henna
Medications: primaquine (anti-malarial), sulfa-drugs (sulphonamides, sulphasalazine, sulfonylureas), nitrofurantoin, dapsone, and NSAIDs/Aspirin, ciprofloxacin
58
What investigations would you do for G6PD deficiency?
G6PD enzyme assay = diagnostic test
Blood film = typically shows Heinz bodies and bite cells
59
Compare and contrast G6PD deficiency and hereditary spherocytosis
* gender and inheritence pattern?
* ethnicity?
* typical Hx features?
* blood film results?
* diagnostic test?
see table:
60
What is cephaloheamatoma?
* a swelling on the newborns head.
* It typically develops several hours after delivery and is due to bleeding between the periosteum and skull.
* The most common site affected is the parietal region
* takes 3 months to resolve
* main differential = caput succedaneum
61
What is pathophysiology / cause of neonatal respiratory distress syndrome (NRDS)?
* who does it affect?
* levels of ---what?--- are low?
* affects premature neonates
* born before lungs start producing adequate surfactant
* Surfactant is a phospholipid-containing fluid produced by type 2 pneumocytes.
* It acts to lower the surface tension in the alveoli helping to keep them open. A lack of surfactant increases surface tension and causes alveoli to collapse, triggering respiratory distress.
* Lungs start to make surfctant at 26 weeks and levels are adequate at ~ 35 weeks gestation.
* This means premature babies are at increased risk of NRDS.
Summary:
* Inadequate surfactant leads to high surface tension within alveoli.
* This leads to atelectasis (lung collapse), as it is more difficult for the alveoli and the lungs to expand.
* This leads to inadequate gaseous exchange, resulting in hypoxia, hypercapnia (high CO2) and respiratory distress.
62
How is NRDS diagnosed?
Newborn respiratory distress syndrome (NRDS)
* clinical diagnosis usually
* CXR = ground glass appearance
63
How is NRDS managed?
* antenatal steroids (i.e. dexamethasone) given to mothers with suspected or confirmed preterm labour increases the production of surfactant and reduces the incidence and severity of respiratory distress syndrome in the baby.
Premature neonates may need:
* Intubation and ventilation to fully assist breathing if the respiratory distress is severe
* Endotracheal surfactant, which is artificial surfactant delivered into the lungs via an endotracheal tube
* Continuous positive airway pressure (CPAP) via a nasal mask to help keep the lungs inflated whilst breathing
* Supplementary oxygen to maintain oxygen saturations between 91 and 95% in preterm neonates
* Support with breathing is gradually stepped down as the baby develops and is able to maintain their breathing, until they can support themselves in air.
64
What are complications of NRDS?
| list short term and long term ones
Short term complications:
* Pneumothorax
* Infection
* Apnoea
* Intraventricular haemorrhage
* Pulmonary haemorrhage
* Necrotising enterocolitis
Long term complications:
* Chronic lung disease of prematurity
* Retinopathy of prematurity occurs more often and more severely in neonates with RDS
* Neurological, hearing and visual impairment
65
What are steps to do in newborn resuscitation?
| Have included pass med 5 step and complex algorithm - resus council
1. Dry baby and maintain temperature
2. Assess tone, respiratory rate, heart rate
3. If gasping or not breathing give 5 inflation breaths*
4. Reassess (chest movements)
5. If the heart rate is not improving and <60bpm start compressions and ventilation breaths at a rate of 3:1
*Inflation breaths are different from ventilation breaths. The aim is to sustain pressure to open the lungs.
66
Hypoxia and neonatal resuscitation?
1. can normal labour lead to hypoxia?
2. What does extended hypoxia lead to (think of the steps of what is happening as it progresses
3. what is a life long consequence of extended hypoxia?
1. yes - when contractions happen placenta cannot carry out normal gas exchange
2. Extended hypoxia -> anaerobic respiration -> bradycardia -> reduced consciousness + reduced resp effort -> worsening hypoxia -> extended in brain -> hypoxic-ischaemic encephalopathy (HIE)
3. life long: cerebral palsy
67
What are some other issues in neonatal resusciation need to be aware of
clue: heat regualtion, meconium
* Babies have a large surface area to weight ratio, and get cold very easily
* Babies are born wet, so they loose heat rapidly
* Babies that are born through meconium may have this in their mouth or airway
68
Neonatal resucitation : Warming the baby
How is it done ? Why is it helpful? <28 weeks?
* Get the baby dry as quickly as possible.
* Vigorous drying also helps stimulate breathing.
* Keep the baby warm with warm delivery rooms and management under a heat lamp
* Babies <28 weeks are placed in a plastic bag while still wet and managed under a heat lamp
69
Neonatal resus: APGAR score
when is it calculated? why is it used?
* This is done at 1, 5 and 10 minutes whilst resuscitation continues
* This is used as an indicator of the progress over the first minutes after birth
* It helps guide neonatal resuscitation efforts
70
Neonatal resus: Stimulate Breathing
How is it done? baby postitioning? is unable what to consider?
* Simulate the baby to prompt breathing, for example by drying vigorously with a towel
* Place the baby’s head in a neutral position to keep airway open. A towel under the shoulders can help keep it neutral.
* If gasping or unable to breath, check for airway obstruction (i.e. meconium) and consider aspiration under direct visualisation
71
Neonatal resus: inflation breaths
when use? How? technique? air or 02?
When:
* not gasping or breathings despite stimulation
How:
* two cycles of 5 inflation breaths lasting 3 seconds each
* if no response and HR low - 30 seconds of ventilation breaths
* no response - chest compressions and ventilation breaths
Technique:
* experience needed
* neutral head position
* seal around mouth and nose
* rise and fall in chest
Air / 02?
* Term / near term babies: air
* Pre-term babies : mix of air and 02
72
Neonatal resus: chest compressions
When do? How ?
* Start chest compressions if heart rate remains below 60 bpm despite resuscitation and inflation breaths (see protocol)
* Chest compressions are performed at a 3:1 ratio with ventilation breaths
73
Neonatal resus: in severe situations what else can be considered?
* big worry is risk of hypoxic-oischaemic encephalopathy (HIE)
* IV drugs e.g. adrenaline, crystalloids, sodium bicarbonate and intubation can be considered
* babies near or at term with possible HIE - consider theraputic hypothermia with active cooling
74
APGAR score:
What points does it look at?
What is the lowest / highest score ?
75
Benefits of delayed umbillical cord clamping?
negative effects?
* after birth lots of fetal blood in placenta
* delay gives time for this blood to enter fetal ciruclation (placental transfusion)
Benefits:
* improved hb, iron stores and blood pressure
* reduce intraventricular haemorrhage
* reduce necrotising enterocolitis
Negative:
* increase in neonatal jaundice - bit more phototherapy
76
Why might a neonate need nasogastric feeding?
and sick infant who is too ill to feed e.g. respiratory distress child
77
Nasogastric feeding for a neonate
What feed them? Where does the tube sit? how to confirm
* expressed breast milk or formula fed through as bolus or continuous infusion
* after entering stomach, the tube enters the jejunum by peristalsis
* confrim with xray
78
Nasogastric feeding for a neonate
When can you start weaning baby off this ? how? what signs might be too soon?
* as baby improves start some PO feeds (by mouth)
* increase ratio of oral to NG feeds
Too soon:
* during oral feeds: cyanosis, bradycardia, desaturation, apnoea, vomitting
79
What is trophic feeding for infants ? what is the rationale ?
* also called minimal enteral feeding / gut priming
* give minimal volume of milk feeds (1 ml/kg/h) (give too much can get NEC)
Rationale:
* if premature infants go for weeks with no oral feeds, normal GI structure and funciton is lost despite an anabolic body state
* Villi shorten, mucosal DNA lost, enzyme activity is reduced
* helps promote gut motility, bile secretion, lactase activity
* reduce sepsis and cholestatic jaundice
| LAST few bullet points extra detail but just to help understands
80
What would be the indications for parenteral nutrition in a neonate?
* preterm
* low birth weight
* too unwell for enteral nutrition (e.g. high dose inotropes)
* gut malfunction - need to rest e.g. NEC, septic ileus
81
if a neonate is on parenteral nutrition - what monitoring is needed?
* Sterility always vital
Daily:
* Weight
* fluid balance
* U&E
* glucose
* calcium
* glycosuria
Weekly:
* length / head circumference
* Mg / P04
* ALP
* trigylcerides
* LFTs / bilirubin
* FBC / CRP (line sepsis detection)
82
if a neonate is on parenteral nutrition - what are possible complications?
Why do you need to graduate slowly into enteral feeds?
* electrolyte imbalance
* thrombophlebitis
* sepsis
* PN associated liver disease
* acidosis
graduate slowly into enteral feeds? - to prevent hypoglycaemia
83
What is chronic lung disease of prematurity? Who is it common to see with?
Also called Bronchopulmonary dysplasia (BPD)
* defined as persistent oxygen requirement after 28 postnatal days or 36 weeks corrected gestational age (whichever first)
common in:
* premature infants / <1kg brithweight
* prolonged mechanical ventilation to treat respiratory distress sundrome
84
What might you find when investigating a child for Chronic lung disease of prematurity?
* blood gas: hypoxia and hypercapnia
* CXR - hyperinflation, atelectasis
* Histology: necrotizing bronchiolitis with alveolar fibrosis ( due to prolonged high 02 delivery)
85
What physical findings for a child with Chronic lung disease of prematurity?
* crackles, wheezing, & decreased breath sounds
* increased bronchial secretions
* frequent lower respiratory infections
* delayed growth & development
* cor pulmonale
86
Early and late consequences of chronic lung disease of prematurity?
Early
* ventilator dependance
* pulmonary HTN
* tracheobronchomalacia
* feeding problems (+ 02 desaturation when feeding)
* severe RSV bronchiolitis
* GORD
Later
* reduced IQ
* cerebral palsy
* adolescence - asthma / exercise limitation
87
How do you prevent chronic lung disease of prematurity?
Steroids (antenatal and postnatal)
suitably high calorie feeding
| from ox handbook
88
What is necrotising enterocolitis (NEC)?
* disorder affecting premature neonates
* Part of bowel becomes necrotic
* This leads to perforation--> peritonitis and shock
89
Risk factors for NEC?
* Very low birth weight or very premature
* Formula feeds (it is less common in babies fed by breast milk feeds)
* Respiratory distress and assisted ventilation
* Sepsis
* Patient ductus arteriosus and other congenital heart disease
90
Presentation of NEC?
* Intolerance to feeds
* Vomiting, particularly with green bile
* Generally unwell
* Distended, tender abdomen
* Absent bowel sounds
* Blood in stools
* When perforation occurs there will be peritonitis and shock and the neonate will be severely unwell.
91
Blood tests for NEC?
Full blood count for thrombocytopenia and neutropenia
CRP for inflammation
Capillary blood gas will show a metabolic acidosis
Blood culture for sepsis
92
Imaging for NEC?
* Abdominal XR: supine, lateral, lateral decubitus
* Findings:
Dilated loops of bowel
Bowel wall oedema (thickened bowel walls)
Pneumatosisi intestinalis- gas in bowel wall- sign of NEC
Pneumoperitoneum- free gas in peritoneal cavity and indicated perforation
Gas in portal veins
93
Management of NEC?
* NBM
* IV fluids, TPN and abx
* NG tube to drain stomach and intestines
* Immediate referral to surgery team- may need surgery to remove dead bowel and a temporary stoma
*
94
Complications of NEC?
* Perforation and peritonitis
* Sepsis
* Death
* Strictures
* Abscess formation
* Recurrence
* Long term stoma
* Short bowel syndrome after surgery
95
What is the most common facial malformation? What is the pathophysiology?
* Orofacial clefts
* Results from failure of fusion of maxillary and premaxillary processes (during week 5)
96
Causes of orofacial cleft?
* Genes
* Benzodiazepines
* Antiepileptics
* Rubella
Can co-exist with other malformations:
* trisomies 18,13-15
* Pierre-Robin short mandible
97
Prevention of orofacial cleft pre-birth?
* Quit smoking pre-pregnancy
* Folic acid 5mg/day preconception +/- multivits if a woman has an affected child
98
Treatment of cleft palate?
* Interdisciplinary treatment incl orthodontist, plastics, oral surgeon, GP,paeds, speech therapist
* Usually lip repair at 3 months and palate at 6 months
99
Complications of orofacial palate?
* otitis media
* aspiration
* post-op palatal fistulae
* poor speech
* social adjustment reduces
100
What are some eye malformations?
* Anophthalmos: no eyes, rare part of trisomy 13-15
* Ectopia lentis: presents as glaucoma with poor vision: Marfans, Ehlers-danlos,homocystinuria
* Cataract: Rubella, Down's
* Microphthalmos: small eyes- rubella or genetic
101
What condition are low set ears associated with?
* Down syndrome
* Congenital heart disease too
102
What is choanal atresia?
* nasal catheter doen't go into nasal pharynx because of nasal malformation
* post-natal cyanotic attacks
103
What is brachycephaly?
Short broad skull from early closure of coronal suture
Down's associated or can be genetic
104
What causes microcephaly?
* genetic
* intrauterine viruses e.g. rubella, zika
* hypoxia
* maternal alcohol
* xrays
105
Features of fetal alcohol spectrum disorder?
* microcephaly
* short palpebral fissures
* hypoplastic upper lip
* absent philtrum
* small eyes
* low IQ
* cardiac malformations
106
Causes of hydrocephalus?
* Ante or neonatal injury
* infection
* genes that may cause aqueduct stenosis
* Dandy-walker syndrome
* arnold-chiari malformation
107
How does a child with fragile x look?
* long, narrow face
* large ears
* prominent jaw and forehead
* unusually flexible fingers
* flat feet
* enlarged testicles after puberty
108
Peak incidence of SIDS?
| sudden infant death sydrome
## Footnote
aka cot death
1-4 months
109
Risk for SIDS?
* poor
* parents are smokers
* male baby
* twin
* premature
* co-sleeping
* winter
* previous sibling affected by SIDS
110
How to prevent SIDS?
* Sleeping- most imp:
Always sleep supine
Room sharing (not co-sleeping)
Remove loose blankets or pillows
Prevent overheating
Avoid heaters, extra blankets
Never tuck higher than the armpits
* breastfeeding
* Reduce cigarette smoke eposure
111
What do we need to exclude if ?SIDS
Sepsis
Metabolic defects
heart defects
112
Pt has come in to ED with ?SIDS, resusciation has failed and the baby has died. How do you proceed?
* Document all intervention, venepuncture sites and marks on baby
* Keep all clothing and nappy
* Explain clearly to parents that the baby has died
* Be non-committal about cause of death- explain baby must have a post-mortem
* Contact consultant on call, police, child protection team, coroner, GP, health vistor and anyone else
113
What 4 things does the Healthy Child Programme offer families?
| dr luyt lecture
1. screening tests - for early detection
2. immunisations - for disease prevention
3. developmental reviews - especialy specific screening at 2 years when less obvious developmental concerns may arise with language skills
4. health promotion - support parenting. and healthy choices
114
Describe screening for Down syndrome
* first trimester test
* integrated test
| how many weeks? what blood markers?
115
Thinking about newborn screening timeline, when is:
1. newborn physical exam?
2. newborn blood spot?
3. newborn hearing screen?
4. infant physical examinaton?
| i.e. how many hours, days, weeks old are these done?
116
What are aims of NIPE (Newborn & Infant Physical Examination Screening Programme)?
Aims:
* Screen for congenital abnormalities.
* Refer where appropriate.
* Reassure parents.
117
In NIPE, what parts of baby are examined and why?
Rationale for NIPE:
* **Eyes:** 2-3 in 10 000 babies have eye problems. Primary focused to identify congenital cataracts.
* **Heart:** 8 in 1000 babies have a heart problem. Critical CHD in 15-25%; leading cause of morbidity/mortality
* **Hips** 1-2 in 1000 babies have hip problems that need treatment
* **Testes:** 1 in 100 baby boys have problems with testes that require treatment
118
RF for congenital cataracts?
* Family history of congenital or hereditary cataracts (1st degree).
* Maternal exposure to viruses (rubella, CMV) in pregnancy.
* Prematurity.
* Trisomy 21.
119
What do you look for when examining eyes in newborn exam (NIPE) ?
Ability to fully open eyelids.
Both eyes same size.
Roundness and symmetry of pupils.
Presence of red reflex.
* Absence - ? Cataracts
* White - ? Retinoblastoma
120
In 6-8 week examination, what do you look for for eyes (i.e. when examining eyes at 6-8 weeks, what are you examining for ?)
* Smiles as visual response.
* Ability to fix steadily (without nystagmus)
* Ability to fix and follow (large bright object)
* Alignment of eyes. (can be variable, consistent abnormal)
121
In NIPE, you find this. What is shown?
Absent red reflex with central opacity in patient with congenital cataract
122
In NIPE, you find this. What is shown?
Asymmetric red reflex with leukocoria in left eye – unilateral retinoblastoma
123
what are RF for congenital heart disease?
Risk factors:
* Family history of congenital heart disease (1st degree).
* Cardiac anomaly suspected from antenatal scan.
* Trisomies.
* Maternal exposure to viruses e.g. rubella in first trimester.
* Maternal conditions e.g. Type 1 diabetes, epilepsy, SLE.
* Teratogenic drugs during pregnancy e.g. anti-epileptics.
* Maternal drug or alcohol abuse.
124
In NIPE exam, what do you look for on examinatuon of the heart
| observation, palpation, auscultation
Observation
* General tone.
* Central and peripheral colour.
* Chest inspection: size and shape; symmetry of movement.
* Use of diaphragm and abdominal muscles.
* Signs of respiratory distress: respiratory rate, recession/grunting.
Palpation
* Assess perfusion through capillary refill time.
* Femoral and brachial pulses for strength, rhythm and volume.
* Position of cardiac apex (dextrocardia); any abnormalities (thrill).
* Abdomen to assess liver size (enlarged in congestive heart failure).
Auscultation - abnormal heart sounds, murmer
125
What features in a NIPE heart exam would suggest critical or major congenital heart anomaly?
* **Tachypnoea** at rest (normal newborn RR 40-60 breaths/min).
* **Apnoea** lasting >20 seconds or associated colour change.
* Recession and nasal flaring.
* Central **cyanosis**.
* **Visible** pulsation over precordium, heaves, thrills.
* Absent or weak **femoral** pulses.
* Presence of cardiac **murmur** or extra heart sounds.
Note:
* Many babies will have a murmur at birth without a heart defect.
* BUT, murmurs can be absent with a significant heart defect.
126
Compare and contrast a significant murmer to a benign murmer (in NIPE exam)
127
What are RF for congenital hip problems?
Family history of hip problems (1st degree).
Breech presentation at or after 36 weeks gestation.
128
In NIPE exam, what are examination findings in baby with congenital hip problem?
Observation
* Differences in leg length.
* Knees at different levels when hips and knees are bilaterally flexed.
* Buttock/posterior thigh skin folds asymmetry on ventral suspension.
* Difficulty in adducting the hip to 90 degrees.
Manipulation
* Palpable ‘clunk’ when undertaking Barlow and Ortolani manouvres.
129
What are RF for congenital problems with testes?
* Family history of cryptorchidism (1st degree).
* Low birth weight.
* Small for gestational age or preterm delivery.
130
What are associations of cryptorchidism?
* Significant increase in the risk of **testicular cancer**.
* **Reduced fertility** when compared to normally descended testes.
* Associated other urogenital problems: hypospadias, testicular torsion
131
In NIPE exam, what should you look for when examining testes?
* Observe scrotum for symmetry, size and colour.
* Palpate scrotal sac to locate testes; if none check inguinal canal.
132
What conditions are screened for in newborn blood spot screening?
133
For the following conditions, describe:
* the presentation of them if not detected
* the management
1. pheylketonuria
2. MCADD
3. Isovaleric acidaemia
4. GLutaric aciduria T1
5. Homocystinuria
6. maple syrup urine disease
| (unsure how much we need to know but have added from slides)
134
Describe the aeitology of congenital hypothyroidism
Primary = an issue to do with thyroid gland (e.g. dysgenesis, or inborn error of thyroid hormone)
Secondary = an issue with HPA axis (e.g. TSH deficiency)
135
What levels of TSH (in a newborn blood spot screen) would prompt you to either repeat bloodspot, refer the newborn or no further action?
136
Why is congenital hypothyridism usually asymptomatic?
* Maternal thyroxine (T4) crosses placenta, so umbilical cord serum T4 approximately 25-50% of normal infants.
* Many infants have some, although inadequate, functioning thyroid tissue.
137
What are manifestations of congenital hypothyroidism at birth?
Birth length and weight within normal range.
Weight often at relatively higher centile because of myxoedema.
Delayed calcification in epiphyses.
138
How does hypothyroidism present in newborns? (congenital)
How does it presented if acquired (delayed presentation)?
## Footnote
Dr Luyt says can always say as ddx for prolonged jaundice pres
139
What diagnostic testing would you for cystic fibrosis?
| there are three
140
Describe how the CF sweat test is done, and what is number is abnormal?
141
How does CF present in
* neonates?
* young children?
* adults?
142
What is inheritamce pattern of CF?
What is prognosis?
* Most common life-shortening **autosomal recessive** condition.
* Occurs more commonly in Caucasians (1 in 2500) but increasingly recognised in South and East Asia, Africa and Latin America.
* Median predicted survival is **39 years**.
143
Describe the pathway in the newborn screening test
144
Define Meckel's diverticulum
* most common congenital gastrointestinal abnormality due to a remnant of the vitello-intestinal duct of the embryo (the attachment between the vitello-intestinal duct and the yolk sac would normally disappear at 6 weeks gestation).
* It is on the anti-mesenteric border of the ileum and is a true diverticulum (i.e. contains all 3 layers of the bowel wall).
145
When does Meckel's present?
Infants and toddlers
Not neonatal period (but is congenital so have added here)
146
What are Rule of 2s in Meckel's diverticulum?
* M:F = 2:1
* 2 inches in length
* 2 feet proximal to the caecum / ileocaecal valve
* occurs in 2% of population
147
How can Meckel's diverticulum present?
* painless rectal bleeding - in passmed
* symptomless so only found in other op/post mortem
* Patent vitello-intestinal duct of the embryo – may be discharging intestinal contents
* Abdominal pain - due to diverticulum inflammation. Pain mimics appendicitis.
* Intussusception – may act as the apex for ileoileal type
* Obstruction - if it becomes trapped in a hernia it (then termed a Littre's hernia)
* Perforation by a foreign body
148
What inv would you do for suspected Meckel's diverticulum?
* CT scan
* 99mTC scan – taken up by gastric mucosa if present ectopically
* (Small bowel enema)
149
How is Meckel's managed?
* Treat clinical presentation appropriately (e.g. obstruction with NG tube)
* Resection of diverticulum (wedge excision or small bowel resection and anastomosis)
150
What are complications of meckel's diverticulum?
* Intussusception – may act as the apex for ileoileal type
* Obstruction - if it becomes trapped in a hernia it (then termed a Littre's hernia)
* Perforation by a foreign body
151
After premature infant has been stabilised and taken to neonatal unit. what are some lab tests might be done on them during their stay and why ?
152
After premature infant has been stabilised and taken to neonatal unit. what are some IMAGING options might be done to them and why?
153
Neurodevelopmental outcomes for premature babies?
* The earlier a baby is born, the higher the likelihood that they will have some neurodevelopmental impairment.
* Gross motor delay
* fine motor impairment
* speech and language delay
* learning and behavioural difficulties.
154
Support for parents of premature babies
* Delivery of a preterm infant can cause a mix of emotions for parents and the wider family
* Invite parents to be involved in the care of their infant and when the baby is stable enough, periods of kangaroo care/skin-to-skin should be encouraged
* Local support groups available for parents and charities such as **Bliss** that offer information and support to families of preterm and sick infants.
155
Support for parents of premature babies
* Delivery of a preterm infant can cause a mix of emotions for parents and the wider family
* Invite parents to be involved in the care of their infant and when the baby is stable enough, periods of kangaroo care/skin-to-skin should be encouraged
* Local support groups available for parents and charities such as **Bliss** that offer information and support to families of preterm and sick infants.
