Kernicterus Definition, Reason, Symptoms, Treatment & Prevention
Understanding
Kernicterus or bilirubin encephalopathy is a rare brain (neurological) disorder caused by high levels of bilirubin in the blood (hyperbilirubinemia) in infants.
This condition can occur when jaundice in infants is not handled properly. The level of bilirubin in the blood continues to increase until it causes brain damage.
In the condition kernicterus, toxic bilirubin collects in certain areas of the brain (basal ganglia and brainstem).
If not treated immediately, this has the potential to cause serious complications.
Kernicterus
| Medical specialist | Children Specialist |
| Symptom | Jaundice, lack of energy, poor eating habits, fever, vomiting |
| risk factor | Family history, premature birth, blood type O or rhesus negative |
| How to diagnose | Physical examination, blood tests, blood group test, Coomb test, reticulocyte count |
| Treatment | Phototherapy, exchange transfusion, plasmapheresis, Intravenous Immunoglobulins (IVIG) |
| Complications | Hearing loss, Athetoid cerebral palsy, visual disturbances, dental abnormalities, muscle spasms, difficulty speaking |
| When to see a doctor? | Found symptoms of kernicterus |
Symptom
In some cases, kernicterus symptoms appear 2-5 days after the baby is born. Initial findings may vary from case to case, including:
- Jaundice
- Lack of energy (lethargy)
- Bad eating habits
- Fever
- Vomit
- Squeaky cries
- Loss of certain reflexes (such as the Moro reflex or the startle reflex)
In addition, mild to severe muscle spasms, uncontrollable muscle movements, breathing problems, and decreased muscle tone (hypotonia) can also be found.
As you get older, other symptoms can be found, such as:
- Delayed and/or abnormal movement or motor development
- seizures
- Impaired ability to coordinate voluntary movements (ataxia)
- Abnormal muscle stiffness resulting in muscle spasms (dystonia)
- Problems with sensory perception
- Lack of upward gaze
- Hearing disorders
In some cases, babies affected by kernicterus may show mental retardation and difficulty speaking (dysarthria).
In most cases, the characteristic kernicterus syndrome develops by the age of 3-4 years.
Reason
The cause of kernicterus is not known. Some cases of kernicterus occur randomly, for no apparent reason (sporadic).
According to the medical literature, hyperbilirubinemia alone is not sufficient to cause kernicterus. It is suspected that Rh disease and/or unknown factors play a role in causing this condition.
In rare cases, kernicterus can be caused by a rare disorder, namely Crigler-Najjar Syndrome Type I.
Risk Factors
Although the cause has not been ascertained, several things are thought to increase the risk of kernicterus, namely:
1. Family History
Having a family history of anemia or jaundice (jaundice) from birth or as a teenager can increase the risk of kernicterus.
2. Premature birth
Premature babies have a liver that is not yet fully developed so that the excretion of bilirubin is more inhibited.
3. Blood Type O or Rhesus Negative
Mothers with blood type O or rhesus negative are more at risk of experiencing jaundice.
Diagnosis
Kernicterus in infants can be suspected in the first days of life. Diagnosis can be established from the symptoms and signs obtained from a thorough examination.
For example, persistent jaundice (yellow skin, mucous membranes, and whites of the eyes), abnormal crying, loss of reflexes in infants (especially the Moro reflex).
On blood examination, unconjugated bilirubin levels were found to be more than 25 mg/dL. Other tests may also be suggested by the doctor, for example blood type tests, Coomb tests, and reticulocyte counts.
Non-routine radiological examinations can be carried out unless the results of laboratory examinations are not supportive.
Treatment
Therapy and treatment of kernicterus aims to reduce the amount of unconjugated bilirubin in the blood. Treatment usually includes:
1. Phototherapy
Intense fluorescent light is focused on baby’s bare skin, while eyes are protected. This aims to accelerate the expenditure (excretion) of bilirubin from the skin and help decompose it.
2. Exchange transfusion
If the bilirubin level does not decrease after phototherapy, an exchange transfusion can be done.
In this method, a small amount of blood is repeatedly drawn and replaced with blood from the donor, until most of the blood has been exchanged.
3. Plasmapheresis
Unwanted substances (such as toxins, metabolic substances, and plasma parts) are removed from the blood. The plasma is then replaced with other human plasma and blood is transfused to the affected individual.
4. Intravenous Immunoglobulins (IVIG)
This method is used when related to immunological conditions or the presence of Rh, or ABO incompatibility.
In addition, in more serious conditions, liver transfusion may be performed. Other treatments can be done as symptomatic and supportive.
Prevention
Early detection can help prevent the condition of Kernicterus. Newborns will generally be observed every 8-12 hours in the first 2 days of life.
Observations will be made again before the baby is five days old. If the baby looks yellow, the doctor can check the blood bilirubin. If the level is high, further therapy will be carried out.
Immediate treatment is carried out if jaundice in infants does not improve.
Complications
Complications of kernicterus disease can include:
- Hearing disorders
- Athetoid cerebral palsy, a type of movement disorder due to brain damage
- Visual disturbances, including difficulty looking up
- Abnormalities in teeth
- muscle spasms
- Speech difficulties
When to See a Doctor?
If you find the symptoms of kernicterus above, immediately take the baby to the doctor. Or, you find physical symptoms such as, baby’s body is yellow, see a doctor immediately.