KERNICTERUS HISTORY DIAGNOSIS
Category: Child Health
Abstract : kernicterus history: A history of risk for hemolytic disease can be an
important clue to a neonate's increased risk of pathologic hyperbilirubinemia,
particularly Rh antigen incompatibility between mother and baby. ABO
incompatibility and a family history of red blood cell (RBC) abnormalities (ie,
G6PD deficiency, hereditary spherocytosis) are also
concerning. Conversely, if th
kernicterus history:
A history of risk for hemolytic disease can be an
important clue to a neonate's increased risk of pathologic hyperbilirubinemia,
particularly Rh antigen incompatibility between mother and baby. ABO
incompatibility and a family history of red blood cell (RBC) abnormalities (ie,
G6PD deficiency, hereditary spherocytosis) are also
concerning.
Conversely, if the baby is breastfeeding well and appears
healthy and vigorous, this can be reassuring. The mother may have breastfed
previous babies who also developed significant jaundice. If so, she may be one
of the approximately 20-40% of women who have above-average levels of
beta-glucuronidase in their breast milk, which potentiates and prolongs
hyperbilirubinemia in their breastfed babies.
kernicterus physical:
•
Acute bilirubin encephalopathy:
The clinical features of this diagnosis have
been well described and can be divided into 3 stages. Of babies with
kernicterus, approximately 55-65% manifest these features, 20-30% may display
some neurologic abnormalities, and approximately 15% have no neurologic
signs.
o Phase 1 (first few days of life): Decreased alertness, hypotonia,
and poor feeding are the typical signs. Obviously, these are quite nonspecific
and could easily be indicative of a multitude of neonatal abnormalities. A high
index of suspicion of possible bilirubin encephalopathy at this stage that leads
to prompt intervention can halt the progression of the illness, significantly
minimizing long-term sequelae. Of note, seizure is not typically associated with
acute bilirubin encephalopathy.
o Phase 2 (variable onset and duration):
Hypertonia of the extensor muscles is a typical sign. Patients present
clinically with retrocollis (backward arching of the neck), opisthotonus
(backward arching of the back), or both. Infants who progress to this phase
develop long-term neurologic deficits.
o Phase 3 (infants aged >1 wk):
Hypotonia is a typical sign.
• Chronic bilirubin encephalopathy:
The
clinical features of chronic bilirubin encephalopathy evolve slowly over the
first several years of life in the affected infant. The clinical features can be
divided into phases; the first phase occurs in the first year of life and
consists of hypotonia, hyperreflexia, and delayed acquisition of motor
milestones. The tonic neck reflex can also be observed. In children older than 1
year, the more familiar clinical features develop, which include abnormalities
in the extrapyramidal, visual, and auditory systems. Minor intellectual deficits
can also occur.
o Extrapyramidal abnormalities: Athetosis is the most common
movement disorder associated with chronic bilirubin encephalopathy, although
chorea can also occur. The upper extremities are usually more affected than the
lower ones; bulbar functions can also be impacted. The abnormalities result from
damage to the basal ganglia, the characteristic feature of chronic bilirubin
encephalopathy.
o Visual abnormalities: Ocular movements are affected, most
commonly resulting in upward gaze, although horizontal gaze abnormalities and
gaze palsies can also be observed. These deficits result from damage to the
corresponding cranial nerve nuclei in the brain stem.
o Auditory
abnormalities: Hearing abnormalities are the most consistent feature of chronic
bilirubin encephalopathy and can develop in patients who show none of the other
characteristic features. The most common abnormality is high-frequency hearing
loss, which can range from mild to severe. These deficits can result from damage
both to the cochlear nuclei in the brain stem and to the auditory nerve, which
appear to be exquisitely sensitive to the toxic effects of bilirubin, even at
relatively low levels. Clinically, this deficit can manifest as delayed language
acquisition. Hence, auditory function must be assessed early in any baby at risk
for chronic bilirubin encephalopathy
o Cognitive deficits: Cognitive function
is relatively spared in chronic bilirubin encephalopathy. However, individuals
with chronic bilirubin encephalopathy are often mistakenly considered mentally
retarded because of their choreoathetoid movement disorders and hearing
deficits. The clinician must emphasize that intellectual functioning is not
typically severely affected.
• Abnormalities of dentition: Some degree of
dental enamel hypoplasia can be observed in about three quarters of patients
with chronic bilirubin encephalopathy. A smaller number of individuals develop
green-stained teeth.
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