Kernicterus

1. PEDIATRICS
Faten Danoun

2. KERNICTERUS
•
Kernicterus, or bilirubin
encephalopathy, is a neurologic
syndrome resulting from the
deposition of unconjugated
(indirect) bilirubin in the basal
ganglia and brainstem nuclei.

3. RISK
FACTORS
•
Jaundice within first 24 hrs of birth
•
A sibling who was jaundiced as neonate
•
Unrecognised hemolysis (ABO- or Rh-
incompatibility)
•
Non optimal sucking nursing
•
Deficiency of G6PD
•
Infection
•
Cephalhematomas bruising
•
East Asian or Mediterranean descent

4. PREVENTABLE CAUSES OF KERNICTERUS
•
The American Academy of Pediatrics has identified potentially preventable causes of kernicterus, as follows:
(1)
early discharge (<48 hr) with no early follow-up (within 48 hr of discharge); this problem is particularly
important in near-term infants (35-37 wk of gestation);
(2)
failure to check the bilirubin level in an infant noted to be jaundiced in the 1st 24 hr;
(3)
failure to recognize the presence of risk factors for hyperbilirubinemia;
(4)
underestimation of the severity of jaundice by clinical (visual) assessment;
(5)
lack of concern regarding the presence of jaundice;
(6)
delay in measuring the serum bilirubin level despite marked jaundice or delay in initiating phototherapy in
the presence of elevated bilirubin levels; and
(7)
failure to respond to parental concern regarding jaundice, poor feeding, or lethargy.

5. PATHOGENESIS •
MULTIFACTORIAL
1.
Unconjugated bilirubin levels,
.2
Albumin binding and unbound bilirubin levels,
.3
Passage across the blood-brain barrier, and
.4
Neuronal susceptibility to injury.

6. CLINICAL
FEATURES
•
INITIAL SIGNS
•
LETHARGY
•
POOR FEEDING
•
LOSS OF MORO REFLEX
•
LATER SIGNS
•
INFANT APPEARS GRAVELY ILL
•
PROSTRATIONS
•
DIMINISHED TENDON REFLEXES
•
RESPIRATORY DISTRESS
•
OPISTHOTONOS WITH BULDGING FONTANELLE
•
TWITCHING OF FACE OR LIMBS
•
SHRILL HIGH PITCHED CRY

7. CLINICAL
FEATURES
•
ADVANCED STAGE
•
CONVULSIONS AND SPASM OCCUR
•
AFFECTED INFANTS STIFFLY EXTENDING THEIR ARMS IN AN
INWARD ROTATION WITH THE FISTS CLENCHED.
•
RIGIDITY IS RARE AT THIS LATE STAGE.

8. PREVENTION
• Universal screening for hyperbilirubinemia in the 1st 24-48 hr
after birth to detect infants at high risk for severe jaundice and
bilirubin induced neurologic dysfunction.
• Protocols
• Hour-specific bilirubin nomogram
• Physical examination, and
• Clinical risk factors

9. MANAGEMENT
•
The following approach is further recommended:
(1)
any infant who is jaundiced before 24 hr requires measurement of total and
direct serum bilirubin levels and, if it is elevated, evaluation for possible hemolytic
disease and
(2)
follow-up should be provided within 2-3 days of discharge to all neonates
discharged earlier than 48 hr after birth.
•
Early follow-up is particularly important for infants younger than 38 wk of
gestation.
•
Parental communication with regard to concerns about infant’s skin color and
behavioral activities should be addressed early.
•
Mothers should be advised to nurse their infants every 2-3 hr and to avoid
routine supplementation with water or glucose water in order to ensure
adequate hydration and caloric intake.

10. REFERENCES
I.
NELSON TEXTBOOK OF
PEDIATRICS, 20TH EDITION

11. THANK YOU