Crigler Najjar Syndrome

Below you will find more information about Crigler Najjar Syndrome from Medigest. If you believe that you are suffering from any of the symptoms of Crigler Najjar Syndrome it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Crigler Najjar Syndrome and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Crigler Najjar Syndrome comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Crigler Najjar Syndrome (CNS) is a serious disorder that interferes with the metabolism of bilirubin, a chemical formed from the breakdown of blood, usually afflicting new borns. This causes an inherited form of non-hemolytic jaundice, which may often result to brain damage in infants.

Diagnosis

Babies suspected with CNS should be tested with bile analysis or genetic testing.

Treatment

The only treatment that cures CNS is total or partial liver transplant, an operation that needs to be proceeded with long-term use of drugs that weakens the immune system, allowing the patient to be more susceptible to other illnesses.

Symptoms and Signs

The high levels of blood and skin biliburin levels causes CNS patients to have persistent orange or yellow skin. The whites of the eyes also appear yellow. Dangerous complications such as brain damage may also arise when the condition is left untreated.

Causes

CNS is caused by possible defects on copies of a gene that produces the liver enzyme glucuronyl transferase essential for the normal method of removing bilirubin from the blood. The normal method of taking out bilirubin will fail if both both copies are defective, causing the blood bilirubin levels to increase at rates related to the level of active enzyme present. It may also be genetic.

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