NEONATAL

JAUNDICE
PRESENTER:
Arief
Fateh
Amirul

24/02/2022
 Introduction
 Jaundice can be detected clinically when the level of bilirubin in the serum rises above
85micromol/l (5mg/dl).
 Hyperbilirubinemia can be benign at low levels but is harmful to the brain at higher levels.
 All newborns will have a serum bilirubin level that is higher than the adult norm, but the
level of hyperbilirubinemia requiring treatment is determined by age in hours and risk
factors for developing severe jaundice.
 Up to 75% of all babies develop jaundice
Topic one
TYPES OF JAUNDICE
 PHYSIOLOGICAL JAUNDICE
Increased production: Fetal erythrocytes have a higher rate of turnover; per
kilogram, newborn infants produce twice the daily adult amount of bilirubin.

Decreased clearance: Newborns have relatively low UDP-glucuronyltransferase

activity (increases until approximately 14 weeks). They also have slow
intestinal motility in the first few days as feeding becomes established, and
this increases small amounts of bilirubin reuptake by the enterohepatic
circulation.

20XX SAMPLE FOOTER TEXT

5
 PATHOLOGICAL JAUNDICE
Increase bilirubin load
 hemolytic
 non-hemolytic
Decreased / impaired bilirubin conjugation
Impaired bilirubin excretion
 Increased bilirubin load – hemolytic
• Immune mediated hemolysis:
Rh factor incompatibility:
ABO incompatibility:
• RBC enzyme defects: These are caused by a defect in the formation of RBCs.
G6PD deficiency.
Pyruvate kinase deficiency.
• RBC membrane defects: In hereditary spherocytosis and elliptocytosis, the spleen
recognizes these RBCs as abnormal and thus destroys the misshapen cells.
• Hemoglobinopathies: Thalassemia and sickle cell disease involve the production of
abnormal globin chains that destroy the RBC.
 Increased bilirubin load – nonhemolytic
 Extravascular blood: Examples include extensive bruising from birth,
cephalohematoma or subgaleal hematoma, or swallowed blood.

 Polycythemia: The most common are erythrocyte transfusion (fetal-maternal

transfusion, delayed cord clamping or twin-twin transfusion) and increased
intrauterine erythropoiesis usually caused by placental insufficiency and/or
chronic intrauterine hypoxia (e.g. from preeclampsia, or heavy maternal
cigarette consumption).

 Exaggerated enterohepatic circulation: A common cause is breast milk

jaundice, GI tract obstruction
 Decreased/impaired bilirubin conjugation

• Physiologic hyperbilirubinemia
• Crigler-Najjar syndromes type 1 and 2: autosomal recessive mutations
that result in total UDP-glucoronyltransferase deficiency (type 1), or
significantly reduced activity (type 2).
• Gilbert syndrome
• Congenital hypothyroidism:
• Breastfeeding jaundice
• Breast milk jaundice
Impaired bilirubin excretion
1. Biliary obstruction (biliary atresia, gallstones, neoplasm, bile duct
abnormalities)
2. Infection: Sepsis, meningitis, TORCH infections and others can all
cause liver impairment
3. Chromosomal abnormalities (including Turner’s syndrome, trisomy 18,
trisomy 21)
4. Metabolic disorders
RISK FACTOR OF SEVERE NEONATAL JAUNDICE
 Prematurity  Sepsis
 Low birth weight  Lactation failure in exclusive
breastfeeding
 Jaundice in the first 24 hours of
life  High predischarge bilirubin
level
 Mother with blood Group O or
Rhesus Negative  Cephalohematoma or bruises
 G6PD deficiency  Babies of diabetic mothers
 Rapid rise of total serum  Family history of severe NNJ in
bilirubin siblings
 HISTORY TAKING
1. Age of onset?
2. Any previous infants with NNJ, ET, kernicterus, neonatal deaths or G6PD
deficiency?
3. Mother’s blood group? Father’s blood group?
- higher risk of ABO incompatibility if one
parent is O+
4. Term or not?
- risk increases with prematurity
5. Assess for risk factors and severity
- apnea, respiratory distress
- poor feeding, feeding intolerance
- temperature instability
 PHYSICAL
EXAMINATION
 General condition, gestation and weight, hydration status
 Signs of acute bilirubin encephalopathy :BIND score
 Pallor, plethora, cephalohematoma, SAH
 Signs of intrauterine infection(eg: petechia, hepatosplenomegaly)
 Cephalo-caudal progression of severity of jaundice
 The adequacy of breastfeeding, weight and hydration status should be
assessed during 1st week of life. Babies with weight loss >7% should be
referred for further evaluation and closely monitored for jaundice.
Methods of detecting
jaundice
1.Visual assessment (Kramer Rule)
2.Transcutaneous bilirubinometer
3.TSB
KRAMER’S RULE
 INVESTIGATIONS

1. G6PD testing
2. Cord TSH, TFT (if indicated)
3. ABO / Coombs test
4. Full blood count + reticulocytes
5. Full blood picture
6. LFT – serum albumin, liver enzymes
7. Septic workup if suspecting infection
 MANAGEM
ENT
1. Jaundice within 24 hours of life
2. Jaundice below umbilicus (corresponds to serum bilirubin 200-250
μmol/L).
3. Jaundice extending to soles of feet: Urgent referral as it is a sign of severe
NNJ.
4. Family history of significant haemolytic disease or kernicterus
5. Any unwell infant with jaundice
6. Prolonged Jaundice of >14 days
7. Infants with conjugated hyperbilirubinaemia should be referred to a
hospital as soon as possible
COMPLICATION OF SEVERE JAUNDICE
If jaundice is severe and improperly treated, it can lead to a form of brain
damage known as bilirubin encephalopathy, which can progress to a more
serious injury known as chronic bilirubin encephalopathy or kernicterus.

American Academy of Pediatrics (AAP) recommends that the term acute

bilirubin encephalopathy (ABE) be used to describe symptoms manifesting in
the first few weeks of life, while kernicterus (or kernicterus spectrum
disorder) be reserved for more chronic manifestations of ABE.
ABE has been categorized into three phases:

Phase 1 typically begins around 3-5 days of life, and may involve decreased
alertness, feeding problems, hypotonia, and a weak Moro reflex.
Phase 2 typically begins later in the first week, and may involve stupor,
irritability, changes in muscle tone/movement, and a high-pitched cry.
Phase 3 typically begins after the first week, and may involve hypotonia, coma,
spasms, “sun setting eyes,” fever, feeding problems, and apnea. The rate of
mortality has been estimated to be at least 21%.
Kernicterus is a more permanent effect of hyperbilirubinemia that can develop
in babies who survive ABE. In the first year of life, it typically manifests with
abnormalities in muscle tone, persistence of tonic neck reflex, and 
delayed developmental milestones. After the first year of a child’s life or
sooner, the effects can vary widely, but may include the following:

• Vision and hearing impairments

• Cognitive impairments

• Dental issues

• Motor control issues such as cerebral palsy,  dystonia,

athetosis, and chorea 

 BIND SCORE
Bilirubin Induced Neurological
Dysfunction
Used to assess risk of acute
bilirubin encephalopathy (ABE)
in babies with severe neonatal
jaundice
WHY?
- to prevent irreversible brain
damage to the
baby
 Treatment of Neonatal
Jaundice

1 Prevention of severe NNJ & follow

up

2 Phototherapy

Exchange transfusion
3
 Phototherapy
It is commenced when TSB reaches the phototherapy threshold for
neonatal jaundice
•Exposure of jaundiced baby to certain wavelength of blue light (400-
500nm) changes the unconjugated bilirubin in skin & subcutaneous
tissues to non-toxic photo-isomers
•Effective phototherapy consists of :
• Blue light range : 400-500nm
• Optimal irradiance
• 15 µW/cm²/nm for conventional phototherapy
• 30 µW/cm²/nm for intensive phototherapy
• Distance of light source 30-50cm from baby
 How phototherapy works:

Light energy is absorbed by bilirubin as it circulates in skin capillaries, resulting in

conversion of insoluble bilirubin more soluble photo-isomers as follow :
Photoisomerization : to produce a water soluble nontoxic isomer of bilirubin “photo-
bilirubin” that can be excreted by liver into bile without need for conjugation.
 Structural isomerization : ( bilirubin lumibilirubin , that can be excreted in
bile without need for conjugation and to less extend in urine.
 Photo-oxidation : ( bilirubin Oxidized byproducts which are excreted in
urine)
 Care of babies during
phototherapy
• Regular vital sign monitoring ; temperature & hydration status
• Babies adequately exposed and place in supine position
• Babies’ eyes covered to prevent retinal damage
• Breastfeeding should be continued
• Turn off photolights and remove eyepads during feeding and blood
taking
• Irradiance of phototheraphy unit(non LED emitting Diode) should be
regularly checked
• LED phototherapy is preferred in preterm baby
 Prevention of severe NNJ
•All babies discharged <48 hours after birth should be seen by a healthcare
provider within 24 hours of discharge
Risk factors to be considered :
•Babies with severe jaundice admitted for treatment, early follow-up to detect
 ABO or Rhesus hemolytic
disease,G6PD
rebound jaundice after discharge  Exclusive breasteeding,if nursing
is not going well and/or weight
• Predischarge screening - to prevent severe NNJ in late preterm and term babies loss > 8-10%
 Previous children with jaundice
•Clinical risk factor assessment or/and predischarge bilirubin levels can be used as  Cephalohematoma or significant
bruising
predischarge screening
 East Asian race

•G6PD deficient babies should be admitted and monitored for NNJ during the first
five days of life

•Term G6PD deficient babies with birth weights >2500 g may be discharged earlier
on day four of life if the TSB is <160 μmol/L (9 mg/dL), and followed-up closely.
 Follow up
• Babies with acute bilirubin encephalopathy should have long-term
follow-up to monitor for neurodevelopmental sequelae

• Term and late preterm babies with TSB >20 mg/dL (342 μmol/L) or
exchange transfusions should have Auditory Brainstem Response (ABR)
testing done within the first 3 months of life

• Healthy term and late preterm babies with non-haemolytic

hyperbilirubinaemia and TSB< 25 mg/dL (428μmol/L) may have follow up
at primary care level

• Preterm babies with jaundice should be followed-up for

neurodevelopmental sequelae as per follow-up plans for all preterm babies
EXCHANGE TRANSFUSION
Indicated for severe hyperbilirubinemia
2 types:
 Double volume exchange
 Partial exchange
Indications:
Double volume exchange
• To lower SB level & reduce risk of brain damage
• Hyperammonimia
• To remove bacterial toxin in septicemia
• To correct electrolytes disorder (AKI)
Partial exchange
• Correct polycythemia with hyperviscosity
• Correct severe anemia without hypovolemia
Preparations prior to exchange transfusion

• Signed informed consent from parents

• Ensure resuscitation equipment ready / available
• Stabilize vital signs
• Obtain peripheral venous access for IV fluid maintenance
• Proper gentle restrain
• Continue feeding. Omit only the LAST feeding prior to ET
• If <4h from last feed, empty gastric contents by NG
aspiration before ET
 Types of blood used

Rh Other In
isoimmunizatio conditions emergencies
n

Cross-
ABO match with ‘O’ Rh
compatible, Rh baby and negative
negative blood mother’s blood
blood.
PROCEDURES
Volume to be exchanged is
2x the infant’s total blood
volume (2x80mls/kg)
Use (preferably irradiated)
Fresh Whole Blood
preferably < 5 days old or
reconstituted Packed Red
Blood Cells and FFP in a ratio
of 3:1
Connect baby to a cardiac monitor
Take baseline observation &record down on the
Neonatal Exchange Blood Transfusion Sheet -
apex beat, respiration, oxygen saturation
ET performed under aseptic technique
Cannulate the umbilical vein
Aliquot for removal and replacement – 5-6 mls/
kg (Not more than 5-8% of blood volume) Max
volume per cycle - 20 mls for term infants, not
to exceed 5 ml/kg for ill or preterm infants
Nurse keeps a record of the amount of blood
given or withdrawn, and medications given
ISOVOLUMETRIC / CONTINUOUS
TECHNIQUE
• Indication: where UVC cannulation is not possible e.g. umbilical sepsis, failed
cannulation
• Blood is replaced as a continuous infusion into a large peripheral vein while
simlutaneously removing small amount blood from an arterial catheter at
regular intervals, matching the rate of the infusion closely - e.g. in a 1.5 kg
baby, total volume to be exchanged is 240 mls
• Delivering 120mls an hour allowing 10 ml of blood to be removed every 5
mins for 2 hours
• Care and observation for good perfusion of the limb distal to the arterial
catheter should be performed as per arterial line care
INVESTIGATIONS : POST ET MANAGEMENT :

Pre exchange
• SB 1. Maintain intensive photo
• FBC 2. Monitor vital signs: Hourly for 4 -
• Blood c&s 6 hours, and 4 hourly subsequently
• Viral screening
Post exchange 3. DXT monitoring : Hourly for 2
• SB hours following ET
• FBC 4. Check SB 4- 6 hours after ET
• Capillary blood sugar
• Serum electrolytes & Ca 5. Long term follow up – for hearing
& neurodevelopmental assessment
 PROLONGED NEONATAL JAUNDICE
• Visible jaundice (SB >85 μmol/L or 5 mg/dL) that persists beyond 14 days of life in a term
baby (≥ 37 weeks) or 21 days in a preterm baby (≥35 weeks to < 37 weeks)
• Conjugated hyperbilirubinaemia is defined as the direct (conjugated) fraction of bilirubin more
than 34 μmol/L (2mg/dL), or more than 15% of the total bilirubin
• All babies with conjugated hyperbilirubinaemia must be referred to paediatric department
urgently to exclude biliary atresia
Causes of
Prolonged
Jaundice
 BREASTMILK JAUNDICE
• Breast milk jaundice is very common and remains a diagnosis of
exclusion.
• Infant must be well, gaining weight appropriately and breast-feed
well.
• The stool must be yellow with a normal physical examination.
• Management is to continue breast-feeding, to give warning signs
to parents and to review them periodically during 1 month and 2
months old immunization or at any subsequent medical
examinations.
•
BILIARY ATRESIA
Clinical features of biliary atresia are persistent or late onset jaundice, conjugated hyperbilirubinaemia,
pale stools, firm liver and hepatosplenomegaly.
• With early diagnosis and biliary drainage through a Kasai procedure by 4-6 weeks of age, successful
long-term biliary drainage is achieved in >80% of children. In later surgery good bile flow is achieved only
in 20-30%.
• Liver transplantation is indicated if there is failure to achieve or maintain bile drainage.
• Serum gamma glutamyl transpeptidase (GGT) – Good discriminating test between non-obstructive and
obstructive causes of neonatal hepatitis.
• A significantly elevated GGT (few hundreds) with a pale stool  biliary obstruction
• a low/normal GGT with significant cholestasis  non obstructive causes of neonatal hepatitis
• Ultrasound of liver –
• Dilated intrahepatic bile ducts (poor sensitivity for biliary atresia) and an absent, small or contracted gall
bladder even without dilated intrahepatic ducts is highly suspicious of extra hepatic biliary atresia in
combination with elevated GGT and pale stool.
• A normal gall bladder usually excludes biliary atresia BUT if in the presence of elevated GGT and pale
stool, biliary atresia is still a possibility.
• Choledochal Cyst
 METABOLIC CAUSES
• Classical Galactosemia
• Citrin deficiency
• Tyrosinaemia type 1
• Neonatal Haemochromatosis
• Primary bile and synthesis disorder
• Peroxisomal biogenesis disorder
• Mitochondrial depletion syndrome
 INFECTIVE CAUSES
1) Septicaemia
2) Urinary tract infection
3) Herpes simplex virus infection
• Consider in infants with liver failure within first few weeks of life.
• IV Acyclovir therapy while waiting for Herpes IgM results in affected infants may be justified.
4) Hepatitis B virus infection
• Can potentially present as early infantile liver failure but incidence is rare.
• Presence of positive Hepatitis B surface antigen, positive Hepatitis B virus envelope antigen
and high viral load confirms the diagnosis..
 ALAGILLE SYNDROME
• Consider in infants who have cardiac murmurs or dysmorphism.
• One of the parents is usually affected (AD inheritance, variable penetrance)
• Affected infants might not have typical dysmorphic features at birth due to evolving nature of the
syndrome.
• Important screening tests include :
• Slit eye lamp examination: look for posterior embryotoxon.
 May also help to rule out other aetiologies in neonatal hepatitis syndrome, e.g. retinitis in
congenital infection, cataract in galactosaemia.
• Vertebral x ray: To look for butterfly vertebrae.
• Echocardiography: look for branched pulmonary artery stenosis.
• Other known abnormalities - ASD, valvular pulmonary stenosis.
• Gene test: JAG1 gene mutation which can be done at IMR
 IDIOPATHIC NEONATAL HEPATITIS SYNDROME
• Follow up with LFT fortnightly.
• Watch out for liver failure and bleeding tendency (vitamin K deficiency).
• Repeat Hepatitis B and C virus screening at 6 weeks.
• Most infants with idiopathic neonatal hepatitis in the absence of physical signs of chronic liver
disease usually make a complete recovery.

LAB INVESTIGATION
• Total Serum Bilirubin with direct / indirect Bilirubin
• *heel prick capillary bilirubin is NOT helpful in the management
of prolonged jaundice
• Other initial tests that are simple and helpful for babies who are
low risk but persistent jaundice beyond 3 weeks of age would be:
• Repeat of SB with direct/ indirect
• FBC + reticulocyte count
• Free T4 & TSH
• Urine Dipstick and Microscopy
:) THANK YOU