NEONATAL JAUNDIC

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 Objectives
• Definition of jaundice

• Metabolism of bilirubin

• Types of jaundice

• Causes of neonatal jaundices

• Management of neonatal jaundice

 Neonatal hyperbilirubinemia
Definition :
Jaundice is the yellow color of the skin and sclerae caused
by deposits of bilirubin

When is visible ?
Adult sclera > 2mg /
dl Newborn skin > 5 mg /
dl
Incidence of neonatal jaundice

Ter : Occurs in 60%

m

Preterm : 80% of preterm neonates

Jaundice is the most common condition that requires
medical attention in newborns.
 Bilirubin
Metabolism
 Types of bilirubin
Unconjugated bilirubin Conjugated bilirubin
(Indirect ) (Direct )

• Bind to albumen • Conjugated with glucoronic

acid

• Fat soluble • Water soluble

• Can cross blood brain barrier • Excreted in urine and stool

• Toxic in high level to brain • Not toxic

 Mechanisms of Neonatal Jaundice

1. Increased Bilirubin Load due to a high hemoglobin concentration.

• The normal newborn infant
• Hemolysis
• Cephalhematoma or bruising , Polycythemia

2. Decreased Bilirubin Conjugation in the liver

• Decreased uridine glucuronyl transferase Activity
• Glucuronyl Transferase Deficiency Type 1 (Crigler Najar Syndrome)

3. Defective Bilirubin Excretion

Etiology Of Jaundice
CAUSES
 • Appears after 24 hours
• Total bilirubin rises by less than 5 mg/dl per

Physiologica
day
• Maximum intensity by 4th-5th day in term &
7th day in preterm
l Jaundice • Serum level less than 15 mg / dl
• Clinically not detectable after 14 days

• Appears age Appears within 24 hours of age

• Increase of bilirubin > 5 mg / dl / day

Pathologica • Serum bilirubin > 15 mg / dl

• Jaundice days Jaundice persisting after 14 days

l jaundice
• Stool clay / white colored and urine staining
yellow staining clothes
• Direct bilirubin > 2 mg / dl
 Physiological Jaundice
Characteristics
• Appears after 24 hours

• Total bilirubin rises by less than 5 mg/dl per day

• Maximum intensity by 4th-5th day in term & 7th day in

preterm

• Serum level less than 15 mg / dl

• Clinically not detectable after 14 days

 Why does physiological jaundice develop?

• Increased bilirubin load

• Defective conjugation

• Increased entero-hepatic circulation

• Incidence
– Term in 60%

– Preterm 80%
 Breast milk jaundice
– It is caused by prolonged
increased enterohepatic
circulation of bilirubin. (β-
GD↑)
– Bilirubin peaks at 10-15 days of
age.
– The level of unconjugated bil. is Breast-milk jaundice; is
at 10-30 mg/dL the commonest cause of

– If nursing is interrupted for 24 Prolonged jaundice in

hours, the bilirubin level falls term infants
quickly
β glucuronidase present in the breast milk of some mothers)
Pathologica
l Jaundice
 Pathological jaundice
1. Appears age Appears within 24 hours of age

2. Increase of bilirubin > 5 mg / dl / day

3. Serum bilirubin > 15 mg / dl

4. Jaundice days Jaundice persisting after 14 days

5. Stool clay / white colored and urine staining yellow staining

clothes

6. Direct bilirubin > 2 mg / dl

 Pathological Jaundice

Unconjugated Conjugated
Bilirubin (indirect) Bilirubin
Pathological

Hemolytic Non-hemolytic
Post-
Hepatic hepatic
 Pathological jaundice
1-Unconjugated (Indirect) hyperbilirubinemia
1. Hemolysis
• Rh , ABO and other blood group incompatibilities
• spherocytosis , elliptocytosis, Alpha thalassemia
• Sepsis ,DIC
• Hematomas
• Polycythemia

2. Non hemolytic
• Breast milk jaundice
• Crigler-Najjar syndrome, types I and II
• Gilbert syndrome
Hemolytic disease of
the newborn

– It is an isoimmunity
hemolysis associated
with
– Rh
incompatibility
or
– ABO
Erythroblastosis
Fetalis
 Hemolytic disease of the newborn due to ABO
incompatibility
•Mothers with type O blood may have circulating
antibodies of Ig G class to other red cell antigens that can
cross the placenta and cause hemolytic disease in a baby
with a different blood type, such as blood type A or B.
(ABO incompatibility )

•The baby develop jaundice in the 1st day of life

 2-Conjugated hyperbilirubinemia
1. Hepatic
• Idiopathic neonatal hepatitis
• Infections - TORCH, sepsis
• Inborn errors of metabolism
• Galactosemia
• Tyrosinemia

2. Post hepatic
– Biliary atresia, choledochal
cyst
 Common causes of jaundice
• Physiological
• Blood group incompatibility
• G6PD deficiency
• Breast milk jaundice
• Cephalhaematoma
• Infections
 Jaundice accompanying G6PD deficiency:

• Patients having G6PD deficiency also have

genetic defects in bilirubin conjugation
as seen in gilbert's disease.

• It is uncommon to have jaundice in G6PD def

infant due to severe hemolytic attack.

• Those G6PD deficient neonates who develop

higher maximal serum total bilirubin values had
significantly lower serum conjugated bilirubin
fractions.
• Crigler-Najjar syndrome type I - rare
• Complete absence of UDPGT and is characterized by
severe hyperbilirubinemia with the ongoing risk of
kernicterus at any point during an individual's lifespan.
• Currently, liver transplantation is the only definitive therapy

• Patients with Crigler-Najjar syndrome

type II partial absence of UDPGT similar to type I but
dramatically respond to therapy with phenobarbital, which is
how the diagnosis is made

Glucuronyl transferase
 Risk factors for jaundice
• J - jaundice within first 24 hrs of life or premature
• A - a sibling who was jaundiced as neonate
• U - unrecognized hemolysis (ABO)
• N nursing – non-optimal sucking/nursing
• D - deficiency of G6PD , DRUGS , Ceftriaxone,
• I - infection
• C – Cephalhematoma /bruising
• E - East Asian/North Indian
 Differential Diagnosis of
jaundice
↑production ↓conjugation Impaired Biliary
obstruction
excretio
n
↑ Unconjugate ↑ Unconjugate ↑ Conjugated ↑ Conjugated

Physiological Gilbert’s Rotor’s Biliary Atresia

Hemolysis Crigler-Najarr Dubin Johnson Hepatitis
Hematoma Hypothyroidis Hepatitis
Polycythemi m Prematurity Metabolic
a
Sepsis
 Approach to jaundiced baby

1. Determine birth weight, gestation and postnatal age

2. Assess clinical condition (well or ill) ,degree of jaundice

3. Decide whether jaundice is physiological or pathological

4. Look for evidence of kernicterus in deeply jaundiced NB

 Clinical assessment of jaundice

Area of body Bilirubin

levels/dlmg

• Face 6

• Upper trunk 9

• Lower trunk & thighs 12

• Arms and lower legs 15

• Palms & soles > 15

High bilirubin level kernicterus
(Bilirubin Encephalopathy)
Major Clinical Features of Acute Bilirubin Encephalopathy

• Lethargy
• poor sucking
• poor or absent Moro's,
• Retrocollis-opisthotonos
Convulsions

Retrocollis (backward arching of the neck)

Opisthotonus (backward arching of the back
This infant presented at age 30 days with a serum bilirubin level of 30 mg/dL (513 μmol/L)
secondary to the Crigler-Najjar syndrome type I. He demonstrates retrocollis and
opisthotonos, signs of the intermediate to advanced stage of acute bilirubin
encephalopathy.
 Major clinical features of chronic
bilirubin encephalopathy

• Athetosis

• Upward gaze

• Sensorineural hearing loss

• Intellectual deficits, mild MR

 Laboratory tests
• Total & direct bilirubin*
• Blood group and Rh for mother and baby*
• Hematocrit, retic count and peripheral smear*
• G6PD assay
• Coomb’s test
• Sepsis screen
• Liver and thyroid function
• TORCH titers
• Liver scan when conjugated hyperbilirubinemia
• Ultrasonography of the liver and bile ducts in cholestatsis

(must in all)*
Measurement of
bilirubin by
jaundice meter
 Laboratory Diagnosis
Laboratory Features Of Hemolytic Disease
Laboratory Features Rh ABO

blood type of Mother Rh negative O

blood type of Infant Rh positive A or B

Anemia Marked Minimal

Direct Commb’s test Positive Negative

Indirect Commb’s test Positive Usually positive

Reticulocyte count (>7 mg/dL) can indicate the presence of an
ongoing hemolytic process in neonates

Direct hyperbilirubinemia in the neonate is defined as a direct fraction

more than 2 mg/dL
• or more than 20% of the total bilirubin concentration
is always pathologic.
Liver function tests:
• Aspartate aminotransferase (ASAT or SGOT) and alanine
aminotransferase (ALAT or SGPT) levels are elevated in hepatocellular
disease.
• Alkaline phosphatase and γ -glutamyltransferase (GGT) levels are
often elevated in cholestatic disease.
• A γ -GT/ALAT ratio of more than 1 is strongly suggestive of biliary
obstruction. However, it does not distinguish between intrahepatic and
extrahepatic cholestasis (do ultrasoud)

Reducing substance in urine: is a useful screening test for galactosemia,

provided the infant has received sufficient quantities of milk.
Other Tests
• Hearing tests (Brainstem auditory-evoked potentials)
should be obtained in aftermath of severe neonatal
jaundice to exclude sensorineural hearing loss.

• MRI in kernicterus
bilateral basal ganglia hyperintensity
 Management

1. Phototherapy

2. intravenous immune globulin (IVIG)

3. Exchange transfusion

4. Drugs
 Baby under triple unit intense
phototherapy

Baby under conventional phototherapy Baby under triple unit intense phototherapy
 Principle of phototherapy
Native bilirubin ( water Insoluble)

450-460nm of light

`Photo isomers of bilirubin (water Soluble)

Urine
 Guidelines for phototherapy in hospitalized infants of 35 or more weeks' gestation

Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316

μmol X0.055 = mg/dl

Copyright ©2004 American Academy of
In the left panel, the solid line refers to the current recommendation of the American Academy of
Pediatrics (AAP) for low-risk infants, the line consisting of long dashes (- - - - -) for infants at intermediate
risk, and the line with short dashes (-----) for infants at high risk.
In the right panel, the dotted line (......) represents the AAP suggested intervention level for exchange
transfusion in infants considered at low risk, the line consisting of dash-dot-dash (-.-.-.-.) for intermediate
risk, and the line consisting of dash-dot-dot-dash (-..-..-..-) for infants at high risk. ( AAP,2004)
 Phototherapy Technique
• Perform hand wash
• Place baby naked in cradle or incubator
• Fix eye shades
• Keep baby at least 45 cm from lights
• Start phototherapy
• Frequent extra breast feeding every 2 hourly
• Turn baby after each feed
• Temperature record 2 to 4 hourly
• Weight record- daily
• Monitor urine frequency
• Monitor bilirubin level
A common mistake while managing hyperbilirubinemia is the
false believes in the efficacy of the ordinary florescent lamps
(not Day-light), and D5% orally.
 Side effects of phototherapy
• Increased insensible water loss
• Loose stools
• Skin rash
• Bronze baby syndrome
• Hyperthermia
• May result in hypocalcemia
 Intravenous immune globulin
• IVIG in infants with Rh or ABO isoimmunization can
significantly reduce the need for exchange transfusions.

• Now IVIG has replaced exchange transfusion as the

second-line treatment in infants with isoimmune
jaundice.

• 1 gm/kg/dose IV
 Exchange transfusion
• Exchange transfusion is indicated
for avoiding bilirubin neurotoxicity
when other therapeutic modalities
have failed or are not sufficient.

• the procedure may be indicated in

infants with erythroblastosis who
present with severe anemia,
hydrops, or both, even in the
absence of high serum bilirubin
 Phenobarbital (Luminal)
• Hyperbilirubinemia: 3-8 mg/kg/d PO/IV
initially; may increase up to 12 mg/kg/d
Not to exceed IV administration rate of 1
mg/kg/min or 30 mg/min for infants
 Prolonged indirect jaundice
Causes
• Breast milk jaundice
• Hypothyroidism
• Pyloric stenosis
• Ongoing hemolysis
• Crigler Najjar syndrome
 Conjugated hyperbilirubinemia
Suspect
• High colored urine
• White or clay colored stool

Caution
• Always refer to hospital for investigations so
that biliary atresia or metabolic disorders can
be diagnosed and managed early
 Remember that:
• Jaundice is the most frequent cause of admission after
early discharge from nursery.

• It is not physiological if: appear in first 24 hrs, increases

by > 0.5 mg/dL/hr , evidence of hemolysis, abnormal
examination, direct bilirubin is > 20% of total, or persists
> 3 weeks.

• Jaundice present in the first 24 hrs of life must be

investigated and treated as an emergency.