Kernicterus

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdulkerim Yassin, M.B.B.S[2]

Synonyms and keywords: Chronic bilirubin encephalopathy

Overview

Kernicterus is irreversible brain damage due to chronic high levels of unconjugated bilirubin in the baby`s blood which is not treated early. Hyperbilirubinemia frequently occurs in majority of newborn infants but mostly it is benign and in severe cases can progress to kernicterus and developmental abnormalities. The risk of bilirubin induced neurologic damage and kernicterus are more in preterm than term neonates and the former suffer adverse effects at lower total bilirubin levels with worse long-term outcomes. Liver metabolizes and excretes bilirubin. During pregnancy, the mother`s liver does it for the baby. After birth, some of the baby`s liver enzyme not well developed specially in preterm, bilirubin raises in the baby`s blood and accumulates in the skin and sclera of eyes and cause jaundice. When the jaundice gets severe, The tissues protecting the brain (the blood-brain barrier) are immature in newborns. Bilirubin penetrates the brain and is deposited in the basal ganglia,hippocampus, geniculate bodies and cranial nerve nuclei causing irreversible damage. Depending on the level of exposure, the effects range from unnoticeable to severe brain damage. When the jaundice occurs within (24 hours) of life is always pathological, whereas it happens after 24 hours of life, it can be physiological. Several underlying pathologic processes responsible for hyperbilirubinemia are G6PD deficiency, Crigler-Najjar syndrome, Gilbert syndrome, hemolytic disorders, and a decreased ability to conjugate bilirubin in neonates and infants. Newborn babies are often polycythemic, meaning they have too many red blood cells. When they break down the cells, one of the byproducts is bilirubin, which circulates in the blood and causes jaundice. When hyperbilirubinemia occurs in adult and older children, it is frequently due to liver abnormalities. Some medications, such as the antibiotic co-trimoxazole (a combination of trimethoprim/sulfamethoxazole) may induce this disorder in the baby, either when taken by the mother or given directly to the baby, due to displacement of bilirubin from binding sites on serum albumin. The bilirubin is then free to pass into the Central Nervous System, because the baby's blood-brain barrier is not fully developed. In the (first 48 hrs of life), A baby should be checked for jaundice and if it is discharged before 72 hrs, the baby should be seen after 2 days. The treatment is phototherapy and exchange transfusion.

Historical Perspective

• Kernicterus was first discovered by Christrian Georg Schmorl, a German Pathologist, in 1904 .

• The association between hyperbilirubinemia and kernicterus was made in 1875.

In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Classification

• Based on the duration of symptoms, bilirubin encephalopathy may be classified as either acute, subtle or chronic.
  • Acute bilirubin encephalopathy: comprises the cute illness caused by severe hyperbilirubinemia. The sign and symptom includes decreased feeding, lethargy, hypotonia and/or hypertonia, high-pitched cry, retrocollis and opisthotonus, setting-sun sign, fever, seizures, and may be death.
  • Subtle bilirubin encephalopathy/Bilirubin induced neurologic dysfunction: defined by the presence of insidious developmental disorders without the classical findings of kernicterus. This may presents with neurodevelopmental disorders such as sensory and sensorimotor integration disorders, hypotonia, ataxia or clumsiness, aphasia, and auditory neuropathy which is impaired auditory brainstem reflexes with normal otoacoustic emissions or cochlear microphonic responses.
  • Chronic bilirubin encephalopathy: the long-term outcome of acute bilirubin encephalopathy and composed of a tetrad of clinical characteristics that are typically appear after one year of age:
    • Abnormal motor control, movements, and muscle tone;
      • An auditory processing disturbance with or without hearing loss;
        • Oculomotor impairments, especially impairment of the upward vertical gaze; and
          • Dental enamel hypoplasia/dysplasia of the primary(milk) teeth.

Pathophysiology

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Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

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Causes

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Common causes of [disease] include [cause1], [cause2], and [cause3].

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The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].

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The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

Differentiating ((Page name)) from other Diseases

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