NEONATAL

HYPERBILIRUBINEMIA
= NEONATAL JAUNDICE.
Dr. David Eseli Simiyu
 DEFINITION
 Increased neonatal serum bilirubin levels
sufficient enough to result in jaundice.

This can be physiological or

pathological.
 BILIRUBIN METABOLISM
 Bilirubin is made from breakdown of
RBCs. Myoglobin,and other heame
containing substances in the body.The
heame is metabolised to bilirubin:-

 HEAME→BILIVERDIN→BILIRUBIN
↑ ↑
heame biliverdin
oxygenase. Reductase.
 METABOLISM contd
 Uncojugated energy depen conjugat
Bilirubin → liver metab →bilirubin
transferase enz

In serum, unconjugated bilirubin is bound

to albumin.↓serum albumin leads to ↑free
bilirubin.This is fat soluble and crosses the
blood / brain barrier to cause kernicterus if
the levels are high.
 METABOLISM contd
 SOME FACTORS DISPLACE BILIRUBIN
FROM THE ALBUMIN BINDING SITES.

Acidosis, drugs esp. sulphonamides

 METABOLISM contd
Conjugated water excreted via
Bilirubin → soluble → urine/stool

Conjugation is an energy dependant activity

that can be disrupted by various factors to
be mentioned later.
 PHYSIOLOGIC JAUNDICE
 Serum bili increases after birth to a peak
at around day 3-4 of life.
 This is due to breakdown of fetal RBCs in
the face of inadequate liver metabolism.
 The level starts to reduce around day 6.
 The peak may result in jaundice.
 6-7% of full term infants are affected.
 PHYSIOLOGIC JAUNDICE
 DEFINITION;- that jaundice that occurs in
a full term infant around day 3-4 of life and
no pathological cause can be found.

 A DIAGNOSIS OF EXCLUSION
JAUNDICE OF PREMATURITY
 Jaundice occurring in a preterm infant.
 Usually caused by;- ↑RBC mass.
immature liver metab
prone to hypo-
glycemia, anoxia,
acidosis.
p/s premature infants can also have
pathological jaundice described ahead
 PATHOLOGICAL JAUNDICE
 DUE TO:- (a) overproduction of bilirubin
Such as increased RBC destruction.

(b) reduced metab/excretion.

Such as enzymatic defficiencies
 Overproduction of bilirubin
 HEMOLYTIC DISEASE OF THE
NEWBORN (HDN);-
ABO incompatibility…..mother O+ve
baby is either A,B or AB.
Can occur with 1st baby
Can be very severe.
Common in our country.
 HDN
 Rhesus incompatibility..
mother rh-ve while baby is rh+ve
occurs in subsequent deliveries after the
1st because the mother has to be
sensitised by prior exposure to rh+ve
stimuli.
Less common than ABO in our setup but is
frequent.
 HDN
 Minor blood group incompatibilities.
Not routinely tested for e.g Kell, Duffy
and others that may be restricted to
regions

RBC membrane defects e.g congenital

spherocytosis
RBC enzymatic defects e.g G6PD
defficiency
 OVERPRODUCTION
 POLYCYTHEMIA…infants of diabetic
mothers, intra-uterine growth
retardation(SGA),etc
 Collections of free blood e.g
cephalohematoma.
 Traumatic birth that destroys myoglobin
and injures the cytochrome system
 REDUCED EXCRETION
 Either by interfering with metabolism, excretion
or both.

 Asphyxia,hypoglycemia,infections….all interfere
with liver cell function hence its ability to
metabolise bilirubin.

 Enzyme deffeciencies….lack of the transeferase

enzyme---Criggler Najar type 1 and 2, …..lack of
ligand proteins x and y---roto syndrome.
 REDUCED EXCRETION
 Biliary atresia…..intra or extra hepatic.
Conjugated bilirubin cant be excreted via
bile as the ducts are atretic within or
outside the liver.

 Intestinal obstruction….conjugated
bilirubin gets to the gut but due to
obstruction,there is stasis allowing the
enzyme –glucuronidase--to deconjugate it
with re-absorbtion of unconjugated
bilirubin (enterohepatic circulation)
 BREASTMILK JAUNDICE
 Juandice associated with breastfeeding.
 For some mothers ,their breastmilk
contains factors that interfere with bilirubin
metabolism.(glucuronidase,NEFFA)
Exclusively breastfed infants have a higher
peak of bilirubin that results in jaundice.
May be due to dehydration resulting from
initial low milk output.=breastfeeding
jaundice.
 RX stoppage of breastfeeding for 24hrs
resolves the jaundice!
 HYPERBILI clinical features
 Jaundice on the skin,sclera of eyes,under
the tongue , and palms and soles of feet.
 Generally jaundice occurring on 1st or 2nd
day of life is pathological.
 Jaundice on the 3rd or 4th day may be
physiological and if physiological it should
last less than 2 weeks.
 Jaundice appearing after day 4 is also
most likely pathological.
 CLINICAL FEATURES
 HISTORY----any history of previously
affected sibling.
--mothers blood group if known.
--suggestive pre-disposing
factors to sepsis.
 CLINICAL FEATURES
 EXAMINATION…jaundice,pallor,hepatome
gally,vomiting,lethargy,poor feeding.
With onset of kernicterus,there is high
pitched cry,hypertonicity,jitteriness
convulsions, bulging fontanelles,
ophisthotonus posturing.
 DIAGNOSTIC EVALUATION
 HISTORY and CLINICAL FINDINGS
 LABORATORY WORKUP
(a)Total bili and differential..serially to
monitor effectiveness of treatment.
(b) Heamogram…HB and reticulocyte
count.
(c) Blood group….mother and baby
 LABORATORY INV
 (d) Coombs test to identify case of HDN
that may not involve ABO or RH factors.

 (e) Peripheral blood film….may show

features of infection or hemolysis.

 (f) Total protein and albumin to determine

albumin binding capacity.
 MANAGEMENT
 PREVENTION:-
use of anti-d to prevent maternal rhesus
sensitization if she is rh-ve and has:-
(a) blood tx of inco. Blood

(b) fetal loss i.e abortion

(c) delivers rh+ve baby

 MANAGEMENT
 SUPPORTIVE:- (a) maintain hydration
(b) ensure nutrition or
adequate caloric
intake to avoid hypo
glycemia.
(c) correct acidosis and
hypoxia.
 MANAGEMENT

 SPECIFIC:-(1) Phototherapy
Works by a process of
photo-isomerisation so
that isomers (cis) are
produced
which are water soluble and
do not require liver metab
for excretion.
 PHOTOTHERAPY
 Light of 420-470 nm wavelength is most
effective. Blue light is most appropriate.
 Distance from light source to the baby
should be not more than 15-20 cm
 Turn baby frequently or use photo-optic
blanket.
 May cause loose stools,skin
rashes,dehydration,retinal
damage,overheating.
 MANAGEMENT
 EXCHANGE TRANSFUSION.(EX TX)

(a) Removes xs bilirubin.

(b) Removes sensitised RBCs.

(c) Improves HB level.

 EXCHANGE TRANSFUSION
 INDICATIONS:
(1) Very rapid rise of bilirubin
8-17 umol/dl/hr.
(2) Rapid fall in HB.(hemolysis)
(3) Sick child—sepsis/meningitis
(4) Prematurity
(5) Previously severe dx in sibs
(6) Reticulocyte count > 15 %
 EXCHANGE TRANSFUSION
 PROCEDURE:
-- use fresh blood preferably
heparinised.
-- blood group O-ve most suitable
-- can use babies ABO type but RH-ve
cross matched against mothers serum.
-- warm blood to 35-37°c
-- continuosely mix the blood to avoid
sedimentation.
 PROCEDURE
 Empty infants stomach with NGT and
leave it in situ.
 Aseptically cannulate infants umbilical vein
(up to 7cm for full term baby)
 Take pre exchange sample for HB and
Bilirubin baseline.
 Exchange over 45-60 minutes using 5-
20ml aliquots depending on baby size and
condition.
 PROCEDURE
 Monitor HR, RR by continuous monitor if
possible otherwise use observation and
stethoscope strapped on infants
precordium.
 Take post exchange sample for HB and
Bilirubin at end of procedure.
 Continue phototherapy and serial bilirubin
monitoring at end of procedure.
 EXCHANGE TRANSFUSION

 DOUBLE VOLUME TRANSFUSION

Assumes baby blood vol. of 85 mls /kg body

weight.

(2 x 85) wt in kg= vol of blood required.

 HYPERBILIRUBINEMIA
 COMPLICATIONS.

KERNICTERUS

Unconjugated bilirubin crosses the

blood/brain barrier and if very high can
lead to kernicterus.
 KERNICTERUS
 High unconjugated bilirubin crosses the
blood brain barrier and interferes with
oxygen uptake of the brain cells.
 It is deposited in the basal ganglia giving
them a yellow coloration.
 Some pre-disposing factors increase the
chance of kernicterus occurring.
 KERNICTERUS
 Pre-disposing factors:
(a) Low serum albumin.
(b) Prematurity.
(c) Sepsis esp. with meningitis.
(d) Asphyxia.
(e) Acidosis.
(f) Intra-ventricular hemorrhage
 KERNICTERUS
 Lethargy, poor suck, loss of moros
response,reduced tendon reflexes.
 Respiratory distress, bulging fontannelles,
shrill high pitched cry,convulsions and
spasms eventually develops ophisthotonic
posturing.
 75% mortality while survivors have various
degrees of brain injury including athetotic
cerebral palsy.
HYPERBILIRUBINEMIA

END

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