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Sahisnuta Basnet
Neurological syndrome resulting from the
depostion of unconjugated bilirubin in
brain cells
etiology
1. prematurity
2. hemolytic disease of the newborn
3. congenital familial non hemolytic
jaundice
4. neonatal hepatitis
5. congenital spherocytosis
Pathophysiology
Precise level above which indirect
reacting bilirubin will be toxic for an
individual infant is unpredictable but
kernicterus is rare in healthy term
infants if serum level is under 25mg/dl
Less mature the infant the greater the
susceptibility to kernicterus
Asphyxia, acidosis, infection, and
hyperosmolality increases the
susceptibility- in these situations levels
as low as 8-12mg/dl may cause
kernicterus BBB is damaged by
asphyxia, acidosis, hyperosmolality
KERNICTERUS
Yellow staining of brain
assc with neuronal
injury
Affects basal ganglia,
cranial nerve nuclei,
brain stem nuclei,
hippocampus and AHC
of spinal cord
Necrosis, neuronal loss
and gliosis …
pathological findings
Clinical manifestation
Early signs: may be subtle. Lethargy,
poor feeding, loss of moro reflex
Acute bilirubin encephalopathy:
Stage 1: hypotonia, lethargy, high pitched
cry, poor suck, poor Moro
Stage 2: hypertonia of extensor muscles,
ophisthotonus, rigidity, retrocollis, fever,
twitching of face or limbs, bulging fontanel,
seizures
Stage 3: hypotonia replaces hypertonia
after about 1 wk of age
Many die, survivors develop chronic
bilirubin encephalopathy
Chronic bilirubin encephalopathy:
Chreoathetosis with involuntary muscle spasms
Extrapyramidal signs
Seizures
Mental deficiency
Dysarthric speech
Hearing loss
Squints and defective upward gaze
Treatment
C/F suggestive of bilirubin toxicity is an
indication for EXCHANGE TRANSFUSION
Once established kernicterus is
irreversible and management is only
symptomatic