Pediatric Crigler-Najjar Syndrome

Pediatric Crigler-Najjar Syndrome


What is Crigler-Najjar syndrome?

Crigler-Najjar syndrome occurs when a child has high levels of bilirubin in the blood.

Expanded overview

Crigler-Najjar syndrome is a very rare disorder. It occurs when a child has high levels of bilirubin (a toxic byproduct of the normal breakdown of red blood cells) in the blood. Over time, excess bilirubin in the blood can lead to excess bilirubin in the brain and nerve tissues, which causes a form of brain damage known as kernicterus.

If untreated, kernicterus can cause a variety of neurological problems, such as involuntary muscle spasms of the body, hearing problems or intellectual disability.

What causes Crigler-Najjar syndrome?

A gene mutation that is hereditary (passed down in families) causes Crigler-Najjar syndrome.

What are the types of Crigler-Najjar syndrome?

There are two types of Crigler-Najjar syndrome:

  • Type 1 (CN1): highly severe and can lead to death
  • Type 2 (CN2): more mild form of the condition

What are the symptoms of Crigler-Najjar syndrome?

Signs and symptoms of this condition include:

  • Extreme lethargy (tiredness)
  • Jaundice
  • Low muscle tone (hypotonia)
  • Periodic arching of the back

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