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Most neonates with neonatal unconjugated hyperbilirubinemia do well
after phototherapy and/or exchange transfusion. Kernicterus should be
preventable if recommendations for hyperbilirubinemia management
are carried out in a timely manner. For neonates with hemolytic anemia
secondary to blood group incompatibility, hemolysis should not present
as a problem once the maternal antibodies are gone. Babies with
polycythemia and extravasation of blood should not have any problem
once the extra hemoglobin breakdown is taken care of. Surgical causes
of increased enterohepatic circulation should resolve once the specific
condition is treated. Babies with partial specific enzymatic conjugation
defects can usually have their bilirubin levels kept under control with
night-time phototherapy.
For babies with conjugated hyperbilirubinemia, the outlook depends on
the etiology of the condition. The clinical course is variable in babies
with alpha 1-antitrypsin deficiency and cystic fibrosis. In babies with
Zellweger's syndrome, the prognosis is poor, with most neonates dying
during the first year or surviving with severe mental retardation and
seizures. Patients with Dublin-Johnson and Rotor's syndromes
(inherited as autosomal recessive) have an excellent prognosis.
Prognosis in babies with other metabolic/genetic defects is dependent
on early recognition and management of the specific enzyme
deficiencies and accumulation of metabolites. Parenteral-induced
cholestasis should improve if enteral feeding can be established. Some
of the infectious etiologies (such as congenital syphilis, bacterial) of
hepatitis improve with specific treatments; others resolve over time.
Supportive treatment is done for the survivors of kernicterus.
Rehabilitative treatment is recommended for the specific neurological
The prognosis for physiological neonatal jaundice is generally very
good. Very few infants ever have bilirubin levels greater than 20 mg/dL,
which is the level that is correlated with kernicterus (an abnormal
accumulation of bile pigment in the brain and other nerve tissue that
causes yellow staining and tissue damage). It rarely occurs with
bilirubin levels lower than 20 mg/dL but typically occurs when levels
exceed 30 mg/dL. Levels between 20 and 30 mg/dL associated with
prematurity and hemolytic disease may increase the risk of kernicterus.
There are long-term neurological problems when this occurs. Affected
children have marked developmental and motor delays in the form of
cerebral palsy and mental retardation may also be present.
Prognosis is excellent if the patient receives treatment according to
accepted guidelines. Brain damage due to kernicterus remains a true
risk, and the apparent increased incidence of kernicterus in recent
years may be due to the misconception that jaundice in the healthy full-
term infant is not dangerous and can be disregarded. If your newborns
jaundice has escalated to Kernicterus, there are a number of severe
long-term consequences ranging from learning disabilities and ADHD to
more severe intellectual disabilities, cerebral palsy, or death. However,
most normal newborn jaundice goes away at a gradual rate, leaving no
lifetime long term effects on the child.
Kernicterus is a complication of neonatal jaundice. The incidence of
kernicterus in North America and Europe ranges from 0.4-2.7 cases per
100,000 births. [20] Death from physiologic neonatal jaundice per se
should not occur. Death from kernicterus may occur, particularly in
countries with less developed medical care systems. In one small study
from rural Nigeria, 31% of infants with clinical jaundice tested had G-6-
PD deficiency, and 36% of the infants with G-6-PD deficiency died with
presumed kernicterus compared with only 3% of the infants with a
normal G-6-PD screening test result.
Neonatal jaundice unspecified is a cause of death certain conditions
originating in the perinatal period and hemorrhagic, hematological
disorders of newborn. In 2015, it killed 2 people as the underlying cause
of death in the United States. Additionally, it was involved in 4 other
deaths as a contributing cause. The sex, race, and age group killed at
the highest rate were men, Black people, and ages <1.