Kernicterus

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1. pediatrics Raafi ul basheer zargar

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 J aundice within first 24 hrs of birth A sibling who was jaundiced as neonate U nrecognised hemolysis (ABO- or Rh-incompatibility) N on optimal sucking\ nursing D eficiency of G6PD I nfection C ephalhematomas \ bruising E ast 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 1 st 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 Unconjugated bilirubin levels, Albumin binding and unbound bilirubin levels, Passage across the blood-brain barrier, and Neuronal susceptibility to injury.

6. ETIOPATHOGENESIS The precise blood level above which indirect-reacting bilirubin or free bilirubin will be toxic for an individual infant is unpredictable, but in a large series, kernicterus occurred only in infants with a bilirubin >20 mg/dL. Ninety percent of the infants in whom kernicterus developed were in previously healthy, predominantly breastfed term and near-term infants. The duration of exposure to high bilirubin levels needed to produce toxic effects are unknown. The more immature the infant is, the greater the susceptibility to kernicterus.

7. 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

8. 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.

9. RECOVERY Many infants who progress to these severe neurologic signs die; the survivors are usually seriously damaged but may appear to recover and for 2-3 months show few abnormalities. Later in the 1st yr , opisthotonos, muscle rigidity, irregular movements, and convulsions tend to recur. In the 2nd yr , the opisthotonos and seizures abate, but irregular, involuntary movements, muscle rigidity, or, in some infants, hypotonia increase steadily. By 3 yr of age , the complete neurologic syndrome is often apparent; it consists of bilateral choreoathetosis with involuntary muscle spasms, extrapyramidal signs, seizures, mental deficiency, dysarthric speech, high-frequency hearing loss, squinting, and defective upward eye movements. Pyramidal signs, hypotonia, and ataxia occur in a few infants. In mildly affected infants, the syndrome may be characterized only by mild to moderate neuromuscular incoordination, partial deafness, or “minimal brain dysfunction,” occurring singly or in combination; these problems may be unapparent until the child enters school.

10. INCIDENCE AND PROGNOSIS

11. 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

12. 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.

13. references NELSON TEXTBOOK OF PEDIATRICS, 20 TH EDITION

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