Crigler Najjar Syndrome
Crigler Najjar Syndrome 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.
Crigler Najjar Syndrome 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.
Crigler Najjar Syndrome Diagnosis
Babies suspected with CNS should be tested with bile analysis or genetic testing.
Crigler Najjar Syndrome 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.
Crigler Najjar Syndrome 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.