Neonatal jaundice

Zilpa,AMO
• Neonatal jaundice is a yellowing of the skin
and other tissues of a newborn infant.
• A bilirubin level of more than 85 umol/l
(5 mg/dL) manifests clinical jaundice in
neonates whereas in the adults 34 umol/l
(2 mg/dL).
• Newborn jaundice is a condition marked by high levels
of bilirubin in the blood.
• The increased bilirubin cause the infant's skin and
whites of the eyes (sclera) to look yellow.
• Before birth, the placenta -- the organ that nourishes
the developing baby -- removes the bilirubin from the
infant so that it can be processed by the mother's liver.
• Immediately after birth, the baby's own liver begins to
take over the job, but this can take time. Therefore,
bilirubin levels in an infant are normally a little higher
after birth.
• Physiological jaundice" usually appears
between day 2 and 3, and clears by 2 weeks.

• Physiological jaundice usually causes no

problems.
• Physiological jaundice
• Most infants develop visible jaundice due to elevation
of unconjugated bilirubin concentration during their
first week. This common condition is called
physiological jaundice. This pattern of
hyperbilirubinemia has been classified into two
functionally distinct periods.
• Phase one
• Term infants - jaundice lasts for about 5 days with a
rapid rise of serum bilirubin up to 204 umol/l
(12 mg/dL).
• Preterm infants: For preterm infants jaundice lasts for
about a week, with a rapid rise of serum bilirubin up to
255 umol/l (15 mg/dL).
• Phase two - bilirubin levels decline about
34 umol/l (2 mg/dL) for 2 weeks, eventually
mimicking adult values.
• Preterm infants - phase two can last more
than 1 month.
• In babies who receive exclusive breast
feedings, phase two can last more than 1
month.
 CAUSES
Possible mechanisms involved in Physiological
jaundice
A.Increase bilirubin load on liver cells
1. Increased red blood cell (RBC) volume
2. Increased circulation of bilirubin in the liver
3. Decreased RBC survival
B. Defective hepatic uptake of bilirubin from
blood plasma
1. Decreased ligadin (Y protein)
2. Increased binding of Y proteins by other anions
3. Decreased liver uptake especially in phase two
C. Defective billirubin conjugation
1. Decreased UDPG activity
2. Defective bilirubin excretion
 Pathophysiology
• Neonatal physiologic jaundice results from
simultaneous occurrence of the following 2
phenomena:
1. Bilirubin production is elevated because of increased
breakdown of fetal erythrocytes. This is the result of
the shortened lifespan of fetal erythrocytes and the
higher erythrocyte mass in neonates.
2. Hepatic excretory capacity is low, because of both
low concentrations of the binding protein ligandin in
the hepatocytes and because of low activity of
glucuronyl transferase, the enzyme responsible for
binding bilirubin to glucuronic acid, thus making
bilirubin water soluble (conjugation).
 Bone marrow

RETICULOENDOTHELIAL
SYSTEM (RES) SHUNT PATHWAY
RBCs
Hgb Hgb Fe
heme Heme precursors
Globin
oxygenase Myoglobulin
Globin
+ Biliverdin Non-hgb heme proteins
Heme Biliverdin
reductase

Fe
Bilirubin Kidney
+
Por phogens
Bilirubin-Albumin Complex

Liver uptake ENTEROHEPATIC CIRC

Cytoplasmic
protein
binding Urine
Smooth urobilinogen
endoplasmic Conj ugated excretion
retic ulum bilirubin
Hydrolysis Intestine
Bilirubin

Urobilinogen

Stercobilin
• Breast milk jaundice is another common,
usually non-harmful form of newborn
jaundice. Breast milk may contain a substance
that increases reuse of bilirubin in the
intestines. Such jaundice appears in some
healthy, breastfed babies after day 7 of life,
and usually peaks during weeks 2 and 3. It
may last at low levels for a month or more.
• Contains a metabolite of progesterone called
3-alpha-20-beta pregnanediol.
• This substance inhibits the action of the
enzyme uridine diphosphoglucuronic acid
(UDPGA) glucuronyl transferase responsible
for conjugation and subsequent excretion of
bilirubin.
• Second substance known to cause breast milk
jaundice is called lipoprotein lipase produces
increased concentration of nonesterified free fatty
acids that inhibit hepatic glucuronyl transferase
which again leads to decreased conjugation and
subsequent excretion of bilirubin.
• Jaundice should be managed either with
phototherapy or with exchange blood transfusion
as is needed.
• Breast feeds however need not be discontinued.
The child should be kept well hydrated and extra
feeds given.
• Breastfeeding jaundice" or "lack of
breastfeeding jaundice," is caused by
insufficient breast milk intake, resulting in
inadequate quantities of bowel movements to
remove bilirubin from the body.
 Pathologic Jaundice of Newborn
• Any of the following features characterizes
pathological jaundice:
1. Clinical jaundice appearing in the first 24 hours.
2. Increased in level of total bilirubin by more than
0.5 mg/dL per hour or 5 mg/dL per 24 hours.
3. Total bilirubin more than 15 mg/dL
(hyperbilirubinemia).
4. Direct bilirubin more than 2.0 mg/dL.
 Causes of Pathological Jaundice of
Neonates
• Increased production
1. Fetomaternal blood group incompatibility: Rh,
ABO
2. Hereditary spherocytosis.
3. Non-spherocytic hemolytic anemia:
G-6-PD deficiency, a-thalassemia,
4. Sepsis.
5. Increased enterohepatic circulation: Pyloris
stenosis, or large bowel obstruction.
• Decreased clearance
1. Inborn errors of metabolism: Criggler-Najjar
syndrome type I and II
2. Drugs and Hormones: Hypothryoidism, breast
milk jaundice.
 Pathological jaundice.
• Abnormal blood cell shapes
– Spherocytic
– Elliptocytosis
• Blood type incompatibilities
– ABO incompatibility (Mother
has type O blood, baby is
blood group A, B or AB)
– Rh incompatibility (Mother is
Rh negative, baby is Rh +ve)
• Cephalohematoma or other birth
injury
• Glucose-6-phosphate
dehydrogenase deficiency
• High levels of red blood cells
(polycythemia)
– More common in small for
gestational age babies
– More common in some twins
• Infection (TORCHES)
• Prematurity
• Transfusions
• Biliary atresia
• Certain medications
• Congenital hypothyroidism
• Crigler-Najjar syndrome
• Cystic fibrosis
• Gaucher's disease
• Gilbert syndrome
• Hypoxia
• Neonatal hepatitis
 Clinical presentation
• The main symptom is a yellow color of the
skin.
• The yellow color is best seen right after gently
pressing a finger onto the skin. The color often
begins on the face and then moves down to
the chest, belly area, legs, and soles of the
feet.
• Sometimes, infants with significant jaundice
have extreme tiredness and poor feeding.
 Clinical Features
• Detectable from skin color on blanching the skin with
digital pressure.
• It starts on the head, spreads to the trunk and then to
the limbs. Jaundice may be underestimated clinically
and is harder to detect in preterm and dark-skinned
infants.
• Other features:
– Pallor
– Evidence of infection
– Bruising, petechiae
– Hepatosplenomegaly (in hemolysis)
– Weight loss- dehydration
 DIFFERENTIAL DIAGNOSIS
• Neonatal sepsis
• Bacterial meningitis
• Head trauma
• Hypothyroidism
• Hypoxic ischemic head injury in the newborn
• Congenital biliary atresia
 DIAGNOSIS AND INVESTIGATIONS
 Total and direct serum bilirubin (>5mg/dL)
 Other tests:
 Complete history, including hx of previous deliveries
 Presentation and duration
 Family hx
 Hx of pregnancy and delivery
 Postnatal hx
 Complete physical examination
 Coloration
 Neurological findings
 Hepatosplenomegaly, petechiae, microcephaly
 Investigation.
• All newborns should be examined for jaundice
at least every 8 to 12 hours for the first day of
life.
• Any infant who appears jaundiced in the first
24 hours should have bilirubin levels
measured immediately.
• This can be done with a skin (Transcutaneous
bilirubinometer ) or blood test.
 Investigation ct..
• Complete blood count
• Coomb's test
• Measurement of levels of specific types of
bilirubin (total and direct bilirubin)
• Reticulocyte count
 INVESTIGATIONS CONT…
 Lab investigations
 Full blood picture
 Blood slide (red blood cell morphology)
 Blood culture
 Blood type and Rhesus determination in mother and
infant
 Glucose- 6- phosphate dehydrogenase
 Serum albumin levels
 Direct Coomb’s test (fetal cord blood)
 Thyroid function tests
 INVESTIGATIONS CONT...
 Liver function tests.
 ASAT and ALAT

 Blood gas measurement

 Ingram icteromete
 Imaging studies
 Ultrasonography of the liver and bile ducts
 Treatment.
• Treatment is usually not necessary.

• Keep the baby well-hydrated with breast milk or

formula. Frequent feedings encourage frequent
bowel movements, which helps remove bilirubin
through the stools. (Bilirubin is what gives stool a
brown color).

• Sometimes special blue lights are used on infants

whose levels are very high. This is called
phototherapy.
• Phototherapy works through a process of
isomerization that changes the bilirubin into water-
soluble isomers that can be passed without getting
stuck in the liver.
• In phototherapy, blue light is typically used because
it is more effective at breaking down bilirubin.
• The infant is placed naked under artificial light. The
eyes are protected from the light.

• In the most severe cases of jaundice, an exchange

transfusion is required. In this procedure, the baby's
blood is replaced with fresh blood.
 THERAPEUTIC EFFECT OF
PHOTOTHERAPY

1. Should be broad spectrum white-blue light energy.

2. The effective range of wavelength of 420-470nm.

3. Distance between the light and the infant is 40cm.

4. Maximum surface area of the skin exposed.

 Side effects of phototherapy

1. Watery diarrhoea and increased fecal water loss.

2. Erythrematous macular- papuric rash.
3. Overheating and dehydration due to increase of
the insensible loss.
4. Retinal damage.
5. Bronze baby syndrome. Is a dark greyish brown
discoloration of the skin due to mixed type of
jaundice i.e Haemolytic and Obstructive.
6. Deprives the maternal - infant interactions /
bonding,
BLUE-light phototherapy machine
 2. EXCHANGE TRANSFUSION.
INDICATION:
1. If intensive phototherapy has failed to reduce bilirubin level and
appearence of clinical signs suggesting kernicterus at any level of
of serum bilirubin.
2. Stop hemolysis and bilirubin production by removing antibody
and sensitized RBC’s.

Potential complications of exchange transfusiON.

– 1. Acidosis.
– 2. Electrolyte abnormalities.
– 3. Hypoglycemia.
– 4. Thrombocytopenia.
 CONT….
5. Volume overload.
6. Arrhythmias.
7. Necrotizing EnteroColitis (NEC).
8. Infection.
9. Graft vs Host disease.
 Cont….

Phenobarbitone.
• It reduces the serum bilirubin effectively by
induces the hepatic enzyme uptake involved
in bilirubin conjugation and increases biliary
excretion.
• It promotes ligandin levels.
 Complication.
Rare, but serious, complications from high
bilirubin levels include:
• Cerebral palsy
• Deafness
• Kernicterus -- brain damage from very high
bilirubin levels
 COMPLICATIONS
KERNICTERUS ( BILIRUBIN ENCEPHALOPATHY ).
• Is a neurological syndrome resulting from the deposition of
unconjugated bilirubin in the basal ganglia, geniculate
bodies, hippocampus, and brain stem nuclei.
• Noticed and developed when bilirubin exceeds 30mg/dl.
• Rare in healthy term infant.

• PREDISPOSING FACTORS TO KERNICTERUS.

• 1. Asphyxia.
• 2. Sepsis.
• 3. Acidosis.
• 4. Hypoglycemia.
• 5. RDS.
 PATHOGENESIS OF KERNICTERUS.
• Is a multifactorial and involves an interaction between
unconjugated bilirubin levels, albumin binding and
unbound bilirubin levels, passage across the BBB and
neuronal susceptibility.
• Acidosis, infection and the amount of unbound bilirubin
play a role in its pathogenesis.
• Neuronal necrosis leading to the clinical findings consistent
with chronic bilirubin encephalopathy is also essential in
pathogenesis.

CLINICAL FEATURES: ACUTE FORM.

Phase 1: Lethargy; poor sucking; stupor; hypotonia;
seizures; and loss of Moro reflex.
 CONT…
Phase 2: Prostration; diminished tendon reflex;
respiratory distress; opisthotonus with bulging
fontanelle; twitching of the face or lips; and high
pitched cry.
Phase 3: Convulsions or spasms; clenched fists and
hypotonia .
CHRONIC FORM
– Opisthotonus; muscle rigidity; hypotonia with complete
neurological syndrome which are: bilateral choreoathetosis
with involutary muscle spasm; extrapyramidal signs;
seizures; mental deficiency; dysarthia speech; sensory
neural hearing loss; squinting and defective upward
movement of the eye.
 PREVENTIVE MEASURES OF KERNICTERUS.

1. Avoid early discharge <48hrs and promote early follow up

at POPNF.
2. Always check the bilirubin level in an infant noted to be
jaundiced.
3. Early detection the presence of risk factors for
hyperbilirubinaemia.
4. Never underestimating the severity of jaundice by clinical
visual assessment.
5. Increase concern regarding the presence of jaundice.
6. Early measuring of the serum bilirubin level despite the
marked jaundice.
 CONT….

7. Early initiation of phototherapy in the presence of

elevated bilirubin level.
8. Early and adequate respond to parental concern
regarding jaundice, poor feeding or lethargy.
 Prevention in general.
• In newborns, some degree of jaundice is normal and probably
not preventable.
• The risk of significant jaundice can often be reduced by
feeding babies at least 8 to 12 times a day for the first several
days and by carefully identifying infants at highest risk.
• All pregnant women should be tested for blood type.
• If the mother is Rh negative, follow-up testing on the infant's
cord is recommended.
• Careful monitoring of all babies during the first 5 days of life
can prevent most complications of jaundice. Ideally, this
includes:
• Considering a baby's risk for jaundice
• Checking bilirubin level in the first day or so
• Scheduling at least one follow-up visit the first week of life
for babies sent home from the hospital in 72 hours
PROGNOSIS OF NEONATAL JAUNDICE.
• Excellent if the baby receives early and
adequate treatment according to the
accepted guidelines.