Neonatal jaundice, a common condition in newborns, is caused by elevated bilirubin levels that can lead to permanent neurological damage if left untreated. There are four main reasons why newborns are prone to jaundice: 1) increased bilirubin synthesis, 2) decreased binding and transport capacity, 3) limited conjugation and excretion capacity, and 4) increased enterohepatic circulation of bilirubin. Treatment may involve phototherapy or exchange transfusion to prevent kernicterus, a condition where bilirubin causes neuronal damage or death. Proper assessment and treatment of jaundiced newborns is important to avoid potential long-term complications.

1. NEONATAL
HYPERBILIRUBINEMIA

2. HYPERBILI-
RUBINEMIA
• State wherein there is elevated
bilirubin level in the blood

3. NEONATAL
JAUNDICE
• Manifestation on the skin and sclera
of elevated bilirubin concentrations
– Neonates appear jaundiced when
serum total bilirubin (TsB) levels
reach 5-7 mg/dL

4. • Common in newborn
– Fullterm 60%
– Preterm 80%
• Requiring 6-10% phototherapy
and/or therapeutic options
• Left untreated may lead to permanent
neuronal damage

5. B I L I R U B I N M E T A B O L I S M

6. WHAT MAKES
NEONATES PRONE TO
DEVELOP JAUNDICE

7. REASONS
I. Increased Bilirubin Synthesis
II. Decreased Binding and Transport
Capacity
III. Limited Conjugation and Excretion
Capacity
IV. Increased Enterohepatic Circulation
of Bilirubin

8. 1ST REASON
• Increased bilirubin synthesis
– Increased rate of degradation
– Shortened circulating RBC life span
(70-90 days versus 120 days)
– A very large pool of hematopoietic
tissue that ceases to function shortly
after birth resulting in heme
degradation
– An increase in enterohepatic
circulation

10. 2ND
REASON
• Decreased binding and transport
capacity
– Newborns have reduced albumin
concentrations
– Albumin have lower plasma binding
capacity for bilirubin

12. 3RD
REASON
• Limited Conjugation and Excretion
Capacity
– In newborns, bilirubin excretion
requires conversion of the nonpolar
unconjugated bilirubin into a more
polar water-soluble substance,
conjugated bilirubin
– Ductus venosus allows blood to
bypass the liver completely
– Conjugation depends on the maturity
of the liver cell

14. 4TH
REASON
• Increased enterohepatic circulation
– High mucosal beta-glucuronidase
activity and alkaline environment
increases hydrolysis of conjugated
bilirubin
– In newborn, the relative lack of
intestinal bacterial flora to reduce
bilirubin to urobilinogen

16. PHYSIOLOGIC
HYPERBILIRUBINEMIA
• Onset: later than 24 hours
• Unconjugated bilirubin peak at 72 hours of life
• Rapid decline in TsB by the 5th or 7-10th day respectively

17. PATHOLOGIC
HYPERBILIRUBINEMIA
• TSB levels have been arbitrarily defined as pathological, if it exceeds
5 mg/ dL on first day, 10 mg/ dL on second day, or 15 mg/ dL
thereafter in term babies.
Presence of any of the following signs indicates pathological jaundice:
• Clinical jaundice detected before 24 hours of age .
• Rise in serum bilirubin by more than 5 mg/ dL/ day
• Serum bilirubin more than 15 mg/dL
• Clinical jaundice persisting beyond 14 days of life
• Clay-/white-colored stool and/or dark urine staining the clothes
yellow
• Direct bilirubin >2 mg/ dL at any time

18. Important causes : .
• Hemolytic: Rh incompatibility, ABO incompatibility, G6PD
deficiency, thalassemias, hereditary spherocytosis
• Non-hemolytic: Prematurity, extravasated blood, inadequate
feeding, polycythemia, idiopathic, breast milk jaundice
Appearing within 24 hours of age:
• Hemolytic disease of newborn: Rh, ABO and minor group
incompatibility
• Infections: Intrauterine viral, bacterial; malaria G6PD deficiency
Appearing between 24 and 72 hours of life:
• Physiological
• Sepsis
• Polycythemia
• Concealed hemorrhages: Cephalohematoma, subarachnoid bleed,
IVH
• Increased enterohepatic circulation

19. Appearing after 72 hours:
• Sepsis
• Neonatal hepatitis
• Extrahepatic biliary atresia
• Breast milk jaundice
• Metabolic disorders
Can also be due to disorders of
– Production
– Hepatic Uptake
– Conjugation
– Enterohepatic Circulation

20. DISORDERS OF
PRODUCTION
• Isoimmunization
• Erythrocyte Enzymatic Defects
• Erythrocyte Structural Defects
• Infection
• Sequestration
• Polycythemia

21. RH INCOMPATIBILITY

22. ABO INCOMPATIBILITY
• Hemolytic reaction of maternal anti-A or anti-B antibodies with
fetal A or B antigens
– Usually milder than Rh
– Almost exclusively in type O mothers
– Jaundice appears at 24-72 hours
– Half of infants with a positive Coombs show hemolysis and
some with a negative Coombs have hemolysis

23. GLUCOSE-6-PHOSPHATE
DEHYDROGENASE DEFICIENCY
• G6PD associated with pentose phosphate metabolic pathway
• G6PD deficiency is common, especially in people of
Mediterranean, African, and Asian decent
• X-linked
• Jaundice occurs between 24 and 72 hours of life

24. HEREDITARY
SPHEROCYTOSIS
• Incidence of 1:5000
• Autosomal dominant
• 50% with hemolytic
anemia,
hyperbilirubinemia,
reticulocytosis

25. INFECTION
• Hyperbilirubinemia is believed to be secondary to hemolysis
• Sepsis may impair conjugation also leading to increased
bilirubin levels

26. SEQUESTRATION
• Sequestration of blood in body cavities may lead to
hyperbilirubinemia as the body metabolizes hemoglobin
– Cephalohematomas, subdural hematomas, subgaleal
hematomas
– Excessive bruising

27. POLYCYTHEMIA
• The increase in RBC mass has the potential to overload the
newborn hemoglobin metabolism capacities

28. GILBERTS SYNDROME
– Benign disorder producing persistent unconjugated
hyperbilirubinemia
• There is defective hepatic uptake and decreased UDPGT
activity
• It usually occurs in the second decade of life, but can
present in neonates

29. CRIGLER-NAJJAR
SYNDROME
• Type I
– There is absence of UDPGT activity
– Autosomal recessive
– 1:1,000,000
– Severe unconjugated hyperbilirubinemia develops and
persists beyond the first week of life
– No hemolysis
– Lifelong risk of kernicterus
– Lifelong phototherapy is needed

30. CRIGLER-NAJJAR
SYNDROME
• Type II
– There is various degree of decrease of UDPGT activity
– Typically benign
– There is unconjugated hyperbilirubinemia in the first few
days of life that does not exceed 20 mg/dL
– Hyperbilirubinemia persists into adulthood
– The treatment is phenobarbital

31. DISORDERS OF
ENTEROHEPATIC
CIRCULATION
• Breast Feeding Jaundice
• Breast Milk Jaundice

32. BREAST FEEDING
JAUNDICE
• Unconjugated hyperbilirubinemia is secondary to a suboptimal
establishment of breastfeeding
• Newborns are under-hydrated, starved
• Delayed passage of meconium
• Enterohepatic reuptake of bilirubin is consequently increased,
leading to hyperbilirubinemia
• Treatment and prevention include frequent feedings (8-12/day)

33. BREAST MILK
JAUNDICE
• Occurs after 3-5 days of life, typically at 2-3 weeks of life
• The etiology is unknown, but believed to be a factor in breast
milk or an altered chemistry in breast milk that enhances
intestinal reabsorption of bilirubin
• No need to stop breastfeeding unless bilirubin levels are
dangerously high

34. W H Y W O R R Y A B O U T
H Y P E R B I L I R U B I N E M I A ?

35. KERNICTERUS
• AKA Bilirubin encaphalopathy
• Penetration of bilirubin into
the brain cell and cause
neuronal dysfunction or
death
• 50% mortality

36. POST MORTEM
EXAMINATION OF BRAIN
Ameer Hamza. Kernicterus. Autopsy Case Reports. 2019

37. KERNICTERUS
• Phase 1 (early) - poor suck, hypotonia, and depressed
sensorium
• Phase 2 (middle) - fever and hypertonia or opisthotonos
• Phase 3 (late) - seizures, less hypertonia, high pitched cry, coma,
poor feeding, athetosis

38. KERNICTERUS
• Long term sequelae:
– Chorioathetoid cerebral palsy
– Sensorineural hearing loss
– Upward gaze palsy
– Dental-enamel dysplasia
– Mental retardation

39. ASSESSMENT OF RISK
• Major
– Predischarge TSB or TcB in the high-risk zone
– Jaundice in the first 24h
– Hemolytic disease
– Gestational age 35-36 weeks
– Sibling received phototherapy
– Cephalohematoma or bruising
– Poor breastfeeding
– East Asian descent

40. DIAGNOSIS
• All neonates are entitled to a thorough physical examination
and evaluation to determine which neonates are at an increased
risk for becoming abnormally jaundiced and developing
sequelae
• Every newborn should be assessed, especially if discharged
before 48-72 hours of life
• TSB or TcB before discharge

41. PHYSICAL
EXAMINATION
• Detection of clinical
jaundice requires digital
pressure and under
adequate/proper lighting,
preferably daylight
• If clinical jaundice is
detected, a total and
direct serum bilirubin or
transcutaneous bilirubin
(TcB) should be measured

42. K
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Serum levels of total bilirubin are approximately
4-6 mg/dL (zone l), 6-8 mg/dL (zone 2), 8-12 mg/dL
(z?ne 3), 12-14 mg/dL (zone 4) and >15 mg/dL
(zone 5)

43. LABORATORY
EXAMINATIONS
• The aim of performing investigations is to confirm the
level of jaundice, identify the cause and follow response to
treatment
• Serum bilirubin
• Determination of maternal blood group and Rh type
• Determination of newborns blood type and Rh type
• Direct Coombs test
• Hemoglobin and Hematocrit
• Peripheral blood smear
• Reticulocyte count
• G6PD level
• Others - . Sepsis screen;,thyroid function test..
On first sample: Hb, Serum bilirubin., Blood group and Rh type ( mother and
baby )
if there is incompatibility, send repeat bilirubin, peripheral smear, Direct
coombs test in next prick.

47. …………………………………………….

50. PHOTOTHERAPY
• Make sure that ambient room temperature is optimum 25° to 28°C to
prevent hypothermia or hyperthermia in the baby.
• Remove all clothes.of the baby except the diaper.
• Cover the baby's eyes with an eye patch, ensuring that it does not block
baby's nostrils.
• Place the naked baby under the lights in a cot or bassinet, if weight is
more than 2 kg or in an incubator or radiant warmer, if the baby is small (
<2 kg). Keep the distance between baby and light 30 to 45 cm
• Ensure optimum breastfeeding
• Monitor temperature of the baby every 2 to 4 hours.
• Measure TSB level every 12 to 24 hours. Discontinue phototherapy once
two TSB values 12 hours apart fall below current age-specific cut offs

52. EXCHANGE TRANSFUSION
• exchange transfusion should be performed, if the TSB levels
reach to age specific cut-off for exchange transfusion or the
infant shows signs of bilirubin encephalopathy irrespective of
TSB levels.
• Indications for ET at birth in infants with Rh isoimmunization
include: i. Cord bilirubin is 5 mg/ dL or more ii. Cord Hb is 10
g/dL or less
• The ET should be performed by pull and push technique using
umbilical venous route. Umbilical catheter should be inserted
just enough to get free flow of blood.

53. PREVENTION
• Antenatal investigation should include maternal blood
grouping. Rh positive baby born to an Rh negative mother is at
higher risk. Anti-D (RhoGam) injectton after first obstetrical
event ensures decreased risk of sensitization in future
pregnancies.
• Ensuring adequate breastfeeding
• Parent education -yellowish .discoloration below knees and
elbows or persistent Jaundice beyond 15 days as reason for
immediate check-up by health personnel.
• High-risk babies, such. as ones with large cephalhematoma or
family history of Jaundice, should be followed up after 2-3 days
of discharge.

54. THANK YOU