Jaundice PEDIATRICS

1. G.ERIC™ MD4

2. Jaundice is an important problem in the first week of life.
High bilirubin levels may be toxic to the developing
central nervous system and may cause neurological
impairment even in term newborns. Nearly 60% of term
newborn becomes visibly jaundiced in the first week of
life. In most cases, it is benign and no intervention is
required. Approximately 5-10% of them have clinically
significant jaundice requiring use of phototherapy or
other
therapeutic options

3. Physiological jaundice represents physiological
immaturity
of the neonates to handle increased bilirubin production.
Visible jaundice usually appears between 24-72 hr of age.
Total serum bilirubin (TSB) level usually peaks by 3 days
of age and then falls in term neonates. TSB levels are
below
the designated cut-offs for phototherapy. It does not
require any treatment.

4. Pathological jaundice is referred to as an elevation of
TSB levels to the extent where treatment of jaundice is
more likely to result into benefit than harm. There is no
clear cut demarcation between pathological and physiological
jaundice. 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.
Such jaundice warrants investigation for the cause and
therapeutic intervention such as phototherapy. Appearance
of jaundice within 24 hr, TSB levels above the expected
normal range, presence of clinical jaundice beyond
3 weeks and conjugated bilirubin (dark urine staining the
nappy) would be categorized under this category.

5. Exclusively breastfed infants have a different pattern of
physiological jaundice as compared to artificially-fed
babies. Jaundice in breastfed babies usually appears
between 24-72 hr of age, peaks by 5-15 days of life and
disappears by the third week of life. One-third of all
breastfed babies are detected to have mild clinical jaundice
in the third week of life, which may persist into the 2nd to
3rd month of life in a few babies. This increased frequency
of jaundice in breastfed babies is not related to characteristics
of breast milk but rather to inadequate breastfeeding
(breastfeeding jaundice). Ensuring optimum breastfeeding
would help decrease this kind of jaundice

6. Approximately 2-4% of exclusively breastfed term babies
have jaundice in excess of 10 mg/ dl beyond third-fourth
weeks of life. These babies should be investigated for
prolonged jaundice. A diagnosis of breast milk jaundice
should be considered if this is unconjugated (not staining
nappies); and other causes for prolongation such as
inadequate feeding, continuing hemolysis, extravasated
blood, G6PD deficiency and hypothyroidism have been
ruled out. Mothers should be advised to continue
breastfeeding at frequent intervals and TSB levels usually
decline over a period of time. Some babies may require
phototherapy. Breastfeeding should not be stopped either
for diagnosis or treatment of breast milk jaundice

7. Originally described by Kramer, dermal staining of
bilirubin may be used as a clinical guide to the level of
jaundice. Dermal staining in newborn progresses in a
cephalocaudal direction. The newborn should be
examined in good daylight. The skin of forehead, chest,
abdomen, thighs, legs, palms and soles should be blanched
with digital pressure and the underlying color of skin and
subcutaneous tissue should be noted.
Serum levels of total bilirubin are approximately
4-6 mg/dl (zone 1), 6-8 mg/dl (zone 2), 8-12 mg/dl
(zone 3), 12-14 mg/dl (zone 4) and >15 mg/dl (zone 5)

9. Yellow staining of palms and soles is a danger
sign and requires urgent serum bilirubin estimation and
further management. In general, the estimation of
bilirubin
levels by dermal zones is unreliable particularly at higher
TSB levels, after phototherapy and when it is carried out
by an inexperienced observer. Total serum bilirubin can
be assessed non invasively by a transcutaneous handheld
device.

10. Measurement of Billrubin Levels
Newborns detected to have yellow discoloration of the
skin beyond the legs, or when their clinically assessed
TSB
levels approach phototherapy range, should have lab
confirmation of total serum bilirubin. TSB assessment
has
a marked interlaboratory variability.

11. Important causes of jaundice in neonates include:
i. Hemolytic: Rh incompatibility, ABO incompatability,
G6PD deficiency, thalassemias, hereditary spherocytosis
ii. Non-hemolytic: prematurity, extravasated blood,
inadequate feeding, polycythernia, idiopathic, breast
milk jaundice
Risk factors for development of severe hyper bilirubinernia
include:
i. Jaundice observed in the first 24 hr
ii. Blood group incompatibility with positive direct
antiglobulin test, other known hemolytic disease (e.g.
G6PD deficiency).
iii. Gestational age 35-36 weeks.
iv. Previous sibling received phototherapy.
v. Cephalohematoma or significant bruising.
vi. If breastfeeding is inadequate with excessive weight loss

12. All the neonates should be visually inspected for jaundice
every 12 hr during initial 3 to 5 days of lifeTranscutaneous
bilirubin (TcB) can be used as an aid for
initial screening of infants. Visual assessment (when
performed properly) and TcB have reasonable sensitivity
for initial assessment of jaundice.
As a first step, serious jaundice should be ruled out.
Phototherapy should be initiated if the infant meets the
criteria for serious jaundice. Total serum bilirubin should
be determined subsequently in these infants to determine
further course of action.

13. InvestigationsThe aim of performing investigations is to confirm the
level
of jaundice, identify the cause and follow response to
treatment.
First line
• Total serum bilirubin (and its fractions, if jaundice is
prolonged or there is yellow staining of nappies): All
cases with suspected pathological levels either clinically
or by trancutaneous measurements need confirmation
by blood examination of serum bilirubin levels.
• Blood groups of mother and baby (if the mother is 'O'
or Rh negative): detects any incompatibility
• Peripheral smear: evidence of hemolysis

14. Second line
• Direct Coombs test: detects presence of antibody
coating on fetal RBC
• Hematocrit: decreased in hemolysis
• Reticulocyte count: increased in hemolysis
• G6PD levels in RBC
• Others: sepsis screen; thyroid function test; urine for
reducing substances to rule out galactosernia; specific
enzyme/ genetic studies for Crigler-Najjar, Gilbert and
other genetic enzyme deficiencies

15. Physiological Jaundice
The parents should be explained about the benign nature
of jaundice. The mother should be encouraged to
breastfeed frequently and exclusively. Mother should be
told to bring the baby to the hospital if the baby looks
deep yellow or palms and soles have yellow staining.
There is no use to expose the baby to direct sunlight to
reduce hyperbilirubinemia.
Any newborn discharged prior to 72 hr of life should
be evaluated again in the next 48 hr for assessment of
adequacy of breastfeeding and progression of jaundice.

16. Prolonged Jaundice Beyond 3 Weeks
This is defined as persistence of significant jaundice
(10 mg/dl) beyond three weeks in a term baby. The
common causes include inadequate feeding, breast milk
jaundice, extravasated blood (cephalohematoma), ongoing
hemolytic disease, G6PD deficiency and hypothyroidism.
One should rule out cholestasis by noting the urine and
stool color and checking the level of direct bilirubin. If the
baby has dark urine or significant jaundice, investigations
should be initiated to rule out:
i. Cholestasis (stool color, urine color, direct and indirect
bilirubin levels)
ii. Ongoing hemolysis, G6PD screen
iii. Hypothyroidism
iv. Urinary tract infectionA

17. Phototherapy Phototherapy remains the mainstay of
treating hyperbilirubinemia in neonates. Photocopy is
highly effective and carries an excellent safety track record
of over 50 yr. It acts by converting insoluble bilirubin
(unconjugated) into soluble isomers that can be excreted
in urine and feces. Many review articles have provided
detailed discussion on phototherapy related issues. The
bilirubin molecule isomerizes to harmless forms under
blue-green light (460-490 nm); and the light sources
having high irradiance in this particular wavelength range
are more effective than the others.

18. For phototherapy to be effective, bilirubin needs to be
present in skin so there is no role for prophylactic
phototherapy. Phototherapy acts by several ways:
Configurational isomerization: Here the Z-isomers of
bilirubin are converted into E-isomers. The reaction is
instantaneous upon exposure to light but reversible as
bilirubin reaches into the bile duct. After exposure of
8-12 hr of phototherapy, this constitutes about 25% of
TSB, which is nontoxic. Since this is excreted slowly
from body this is not a major mechanism for decrease
in TSB.
• Structural isomerization: This is an irreversible reaction
where the bilirubin is converted into lumirubin. The
reaction is directly proportional to dose of phototherapy.
This product forms 2-6% of TSB which is
rapidly excreted from body thus is mainly responsible
for phototherapy induced decline in TSB.
• Photo oxidation: This is a minor reaction, where
photo-products are excreted in urine.

20. Monitoring and stopping phototherapy. Monitor
temperature
of the baby every 2 to 4 hr. Measure TSB level every 12
to
24 hr.
Discontinue phototherapy once two TSB values 12 hr
apart fall below current age specific cut offs. The infant
should be monitored clinically for rebound bilirubin rise
within 24 hr after stopping phototherapy for babies with
hemolytic disorders.

21. Exchange Transfusion
Double volume exchange transfusion (DVET) should be
performed if the TSB levels reach to age specific cut-off
for exchange transfusion (Fig. 8.52 and Table 8.25) or the
infant shows signs of bilirubin encephalopathy irrespective
of TSB levels.
Indications for DVET 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
At birth, if a baby shows signs of hydrops or cardiac
decompensation in presence of low PCV (<35%), partial
exchange transfusion with 50 ml/kg of packed red blood
cells should be done to quickly restore oxygen carrying
capacity of blood. 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.

22. Followup
Babies with serum bilirubin ;?.20 mg/dl and those who
require exchange transfusion should be kept under
followup in the high-risk clinic for neurodevelopmental
outcome. Hearing assessment (BERA) should be done at
3 months of age. With prompt treatment, even very
elevated serum bilirubin levels within the range of 25 to
29 mg/ dl are not likely to result in longterm adverse
effects
on neurodevelopment.

23. Prevention
• Antenatal investigation should include maternal blood
grouping. Rh positive baby born to a Rh negative
mother is at higher risk for hyperbilirubinemia and requires greater
monitoring. Anti D (RhoGam) injection
after first obstetrical event ensures decreased risk of
sensitization in future pregnancies.
• Ensuring adequate breastfeeding
• Parent education regarding danger signs should
include yellowish discoloration below knees and
elbows or persistent jaundice beyond 15 days as reason
for immediate checkup by health personnel.
• High risk babies such as ones with large cephalohematoma
or family history of jaundice should be
followed up after 2-3 days of discharge