Neonatal Health

Assoc Prof Zsuzsoka Kecskes

Dept of Neonatology

The Canberra Hospital

Topics covered

• Jaundice

• Hips

• Cardiac/Oxygen Saturation Monitoring

Neonatal jaundice

What is jaundice?

What is jaundice?

• Jaundice is bilirubin that is deposited in the skin

and mucous membranes. It is the end product

of haem breakdown.

• Lysis of red blood cells releases haem from

haemoglobin, haem is then converted to

bilirubin and excreted.

Bilirubin metabolism

Ente

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Uridine diphosphate glucuronyl transferase (UDPGT)Uridine diphosphate glucuronyl transferase (UDPGT)

• 65% of term newborns develop clinical jaundice in first week

• 80% of preterm infants

Prevalence of neonatal jaundice

Types of neonatal jaundice

Bilirubin exists in two main forms in serum

1. Unconjugated bilirubin reversibly bound

to albumin

2. Conjugated bilirubin readily excretable via

the renal and biliary systems

Best classified by age of onset and duration:

1. Early: within 24 hrs of life

2. Intermediate: 2 days to 2 weeks

3. Late: persists for >2 weeks

Causes of neonatal jaundice

Causes of neonatal jaundice

Early Intermediate Late/prolonged

• Haemolytic causes:

– Rh isoimmunisation

– ABO incompatibility

– G6PD deficiency

• Congenital infection

• Physiological jaundice

• Breast milk jaundice (inadequate intake)

• Sepsis

• Haemolysis

• Crigler-Najjar syndrome (glucuronyl transferase

absent/reduced)

• Polycythaemia, bruising

• Conjugated (dark urine, pale stools):

– Bile duct obstruction

– Biliary atresia

– Neonatal hepatitis

• Unconjugated:

– Physiological

– Breast milk jaundice

– Infection

– Hypothyroidism

Causes of neonatal jaundice

Early Intermediate Late/prolonged

• Haemolytic causes:

– Rh isoimmunisation

– ABO incompatibility

– G6PD deficiency

• Congenital infection

• Physiological jaundice

• Breast milk jaundice (inadequate intake)

• Sepsis

• Haemolysis

• Crigler-Najjar syndrome (glucuronyl transferase absent/reduced)

• Polycythaemia, bruising

• Conjugated (dark urine, pale stools):

– Bile duct obstruction

– Biliary atresia

– Neonatal hepatitis

• Unconjugated:

– Physiological

– Breast milk jaundice

– Infection

– Hypothyroidism

• Apparent before 24 hours of age

• Always pathological

• The main cause for early jaundice is

haemolysis such as:

– Rhesus isoimmunisation

– ABO incompatibility

– G6PD deficiency

Early jaundice

Investigations for early jaundice

• Serum bilirubin level

• FBC and film

• Blood group

• Maternal blood group

• Direct coombs test

• Consider G6PD level

Causes of neonatal jaundice

Early Intermediate Late/prolonged

• Haemolytic causes:

– Rh isoimmunisation

– ABO incompatibility

– G6PD deficiency

• Congenital infection

• Physiological jaundice

• Breast milk jaundice (inadequate intake)

• Sepsis

• Haemolysis

• Crigler-Najjar syndrome (glucuronyl transferase absent/reduced)

• Polycythaemia, bruising

• Conjugated (dark urine, pale stools):

– Bile duct obstruction

– Biliary atresia

– Neonatal hepatitis

• Unconjugated:

– Physiological

– Breast milk jaundice

– Infection

– Hypothyroidism

Physiological jaundice

• Physiological jaundice is jaundice that is present

between day 2 and day 10

• Physiological jaundice is jaundice that is present

between day 2 and day 10

Physiological jaundice

• Physiological jaundice is due to:

- High bilirubin production (fetal Hb,

high Hb)

- Reduced bilirubin excretion

(UDPGT concentrations at term are

1% of adult concentrations)

• Physiological jaundice is due to:

- High bilirubin production (fetal Hb,

high Hb)

- Reduced bilirubin excretion

(UDPGT concentrations at term are

1% of adult concentrations)

What are the factors that

exacerbate ‘physiological

jaundice’?

Factors that can increase the severity of physiological jaundice

• Prematurity • Sepsis• Bruising • Cephalohematoma • Polycythaemia • Delayed passage of meconium • Breast feeding • Certain ethnic groups, esp Chinese

Causes of neonatal jaundice

Early Intermediate Late/prolonged

• Haemolytic causes:

– Rh isoimmunisation

– ABO incompatibility

– G6PD deficiency

• Congenital infection

• Physiological jaundice

• Breast milk jaundice (inadequate intake)

• Sepsis

• Haemolysis

• Crigler-Najjar syndrome (glucuronyl transferase absent/reduced)

• Polycythaemia, bruising

• Conjugated (dark urine, pale stools):

– Bile duct obstruction

– Biliary atresia

– Neonatal hepatitis

• Unconjugated:

– Physiological

– Breast milk jaundice

– Infection

– Hypothyroidism

Prolonged Jaundice

• Jaundice persisting after 14 days in the term infants

Or

• Jaundice persisting after 21days in the preterm infant

When to start phototherapy?

• Serum bilirubin level

• Conjugated fraction of bilirubin

• Liver function test (GGT, ALT, AST, Albumin)

• Coagulation profile (PT, PTT, INR)

• Abdominal ultrasound (gallbladder)

• DISIDA / HIDA scan (with follow through)

• Thyroid function test (TSH, free T4)

• Metabolic screen (urine for reducing substance)

• Hepatitis screen (TORCH)

• Liver biopsy (bile duct proliferation)

• FBC and film

• Blood group

• Maternal blood group

• Direct coombs test

Investigations for prolonged jaundice

Kernicterus

• Also called bilirubin encephalopathy

• Neurological syndrome resulting from neurotoxic affects of unconjugated bilirubin on basal ganglia and brainstem nuclei

• Though infrequent, has at least 10% mortality and 70% morbidity

Mechanism of neurotoxicity in kernicterus

• Unbound (unconjugated) bilirubin is:

– neurotoxic at high levels

– lipophilic and can cross the blood-brain-barrier

Long term clinical sequelae

Bilirubin staining of affected areas

Dysfunction and death of neurons

Impairs mitochondrial functionsneurotransmitter synthesis

Binds to cell components

Bilirubin accumulates at nerve terminals

Kernicterus: signs and symptoms (first 24 hours)

• Phase 1:– Poor suck, hypotonia and lethargy

• Phase 2:– Hypertonia and opisthotonos

• Phase 3:– Less hypertonia, high pitched cry, hearing

and visual loss, poor feeding and athetosis

Kernicterus: signs and symptoms (long term)

• Choreoathetoid cerebral palsy

• Upward gaze palsy

• Sensorineural hearing loss

• Intellectual delay (less common)

• Depends on the cause and level and type of

bilirubin

• Unconjugated hyperbilirubinaemia:– Ensure adequate fluid intake

– Phototherapy

– IV immunoglobulin

– Exchange transfusion

• Conjugated hyperbilirubinaemia:– Ensure adequate nutrition

– Treat underlying problem

Treatment of Neonatal Jaundice

Phototherapy

BiliblanketBiliblanket

Phototherapy lightsPhototherapy lights

Phototherapy

Spectrum of light Blue is most effective (460 - 490 nm)

Spectrum of light Blue is most effective (460 - 490 nm)

Ultraviolet light(10 – 400 nm)

Ultraviolet light(10 – 400 nm)

Infrared light(400 – 700 nm)Infrared light

(400 – 700 nm)

Phototherapy is NOT:• Ultraviolet light• Infrared light

Bilirubin absorbance and transmittanceBilirubin absorbance and transmittance

Maisels MJ et al. N Engl J Med 2008;358:920-8Maisels MJ et al. N Engl J Med 2008;358:920-8

Bilirubin absorbanceBilirubin absorbance

When to start phototherapy?

Decision is based on:

1. Level of bilirubin

2. Rate of rise of bilirubin

3. Gestational age

4. Chronological age

5. Wellness of the baby

When to start phototherapy?

Take home message

• Jaundice before 24 hours is always

pathological

• Kernicterus is rare but serious complication of

Unconjugated hyperbilirubin

• Early diagnosis of biliary atresia impacts on

long-term outcome

Examination of the hips - Recommendations

• All newborns are to be screened by physical examination (AAP)

– properly trained health care provider with regular re-assessment of skills

– evidence is good, expert consensus is strong

– pre term babies at d/c health check or when acute needs have settled

• Examination at 2-4 days, 1, 2, 4, 6, 9 & 12 months - (CTFPHC)

– fair evidence

Examination of the hips

• Be aware of changes of physical exam with age– by 8-12 weeks Barlow & Ortolani tests are negative regardless of

the status of the femoral head

– at 3 months limitation of abduction is the most reliable sign associated with DDH (AAP). Asymmetry of thigh folds, leg length discrepancy & Galeazzi sign are also signs.

• Hips continue to dislocate through out the first year of life

• 1/5000 children have a dislocated hip detected at 18 months

Examination of the hips – High Risk

• Family history

• Breech presentation

• Foot deformities

• Oligohydramnios

• Primiparity

• Females

Examination of the hips - Incidence

• White neonates: 1% CDH• Varies with Race:Blacks/Koreans/Chinese

•F:M 4:1•LHS 60%•RHS 20%•Bilat 20%

Examination of the hips

Identify at Risk infants:

Examine the Hips for Dislocation and/or dislocatibility

• If hips are stable and there are no risk factors no further action

• If there is a strong family history (parents or siblings) of CDH, delivery was as breech, or other deformities are present:

hip ultrasound at 6 weeks and appointment at the post-natal clinic even if hips are stable during the immediate postnatal period.

• All babies with clicky hips MUST be reviewed by the Neonatal Registrar and NOT simply referred for U/S and follow-up. Clicky but stable hips are quite common in the first 48 hours of life!

Dislocated/able at birth

Pavlik Harnessfor 6 weeks

Normal exam,normal US,

remove harness

US and Clinic

No improvement,Harness for 6

weeks

No improvement,harness

US and Clinic

Discuss withOrthopaedic

Surgeon

X-Ray and Clinic

discharge

at 6 weeks

at 6months

at 3months

at 3/6months

normal

abnormal

Heart Examination

• Congenital heart disease (CHD) commonest congenital malformation (9 in 1000 live births)

• Contributes significantly to infant mortality and morbidity.

• Current post-natal screening CHD: physical examination after birth and repeat examination at 6-8weeks of age.

• Can miss up to 25% of infants with critical CHD

• Delayed or missed diagnosis associated with significant morbidity, the most significant being hypoxic / ischaemic brain injury.

• Observational studies suggest that there may be some benefit in screening with pulse oximetry

• To date no randomised controlled trials

• Rarity of undiagnosed cyanotic CHD and large number of patients required for studies a randomised controlled trial may not be feasible.

Heart Examination

Heart Examination

• Systematic reviews found overall sensitivity of 63% and specificity of 99.8% for pulse oximetry in diagnosing congenital heart disease in asymptomatic newborns

• False positives 0.2-0.9%

• Sensitivity of pulse oximetry screening 72% compared to 58% for clinical examination.

Heart Examination

• 2 other studies found that in asymptomatic babies a significantly higher number of babies with duct dependent heart disease were detected during screening by physical examination and pulse oximetry (86.2%) compared with those regions not using pulse oximetry screening (72%).

benefit of screening with pulse oximetry in addition to clinical examination. False positives for congenital heart disease will occur but may be expected to detect some other important medical problems, such as sepsis or RDS

Heart Examination

PULSE OXIMETRY SCREENING

• Within 4-24 hours of life on PNW or DS

If SaO2 < 90%

• Medical review

• If asymptomatic, repeat pulse oximetry in 2-3 hours

• If symptomatic further investigations (bloods, CXR, ECG and echocardiography), consider admission)

Heart Examination

• Screening introduced to TCH 12 months ago

• Audit after 4 months (976 neonates)

Heart Examination

• 40 (4%) of infants were missed

• 15 (1.5%) were found to have saturations<90%

– 3 (0.3%) incorrect assignment

– 7 (0.7%) -passed on screen 2

– 1 (0.1%) other diagnosis (Pierre Robin sequence)

• 4 (0.41%) failed both screens

– 3 (0.31%) had sepsis with normal sats on discharge

– 1 (0.1%) had PPHN and non critical congenital heart defect (ASD)

•  

Take home messages

• Nutrition (weight, urine output)

• Jaundice

• Physical exam (heart, hips)

• We are just at the end of the phone!!!!!