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Treatment, Prognosis and Complications of Crigler-Najjar Syndrome

Last Updated on Aug 04, 2014
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Treatment, Prognosis and Complications of Crigler-Najjar Syndrome

Phototherapy converts bilirubin to soluble forms that can be excreted in the urine. Type 2 disease is less severe, and is treatable using phenobarbital.

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Type 1 Crigler-Najjar Syndrome

Before the advent of phototherapy most patients with Crigler-Najjar syndrome type 1 died of kernicterus in infancy or early childhood. Phototherapy or light therapy is a technique in which the patient is exposed to particular wavelengths of light. Phototherapy converts bilirubin to soluble forms that can be excreted in the urine. Phototherapy is sometimes supplemented by a technique called plasma exchange. In this procedure, the excessive bilirubin from the blood is filtered out. Early liver transplantation (before the onset of brain damage) may save lives.

Calcium compounds are sometimes used to bind with and remove bilirubin. Chemicals like tin protoporphyrin or tin-mesoporphyrin may help to reduce bilirubin levels as an emergency, but the effect is transient.

Type 2 Crigler-Najjar Syndrome

Type 2 disease is less severe, and is treatable using phenobarbital. This drug belongs to the group called barbiturates. A single bedtime dose of phenobarbital usually maintains clinically - safe plasma bilirubin concentrations.

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Prognosis of Crigler-Najjar Syndrome

Type 1 Crigler-Najjar syndrome is severe and can lead to death in childhood if left untreated. Infants who survive to adulthood may need daily treatment since the jaundice tends to persist. Life expectancy for this type is 30 years. Brain damage (kernicterus) is certain in most cases.

Type 2 disease is less severe, has fewer symptoms and less organ damage.

Complications of Crigler-Najjar Syndrome

Crigler-Najjar disease may cause brain damage (kernicterus) and chronic jaundice.

References:

  1. Harrison's Principles of Internal Medicine 18th Edition
  2. The Merck Manual
  3. Nelson Textbook of Pediatrics. 18th ed.
  4. Cecil Medicine 24th Edition

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