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Patients with this condition develop jaundice, kernicterus, and bilirubin deposition in the basal ganglia because of high levels of unconjugated bilirubin. The disease is caused by a lack of uridine diphosphate glucuronyl transferase, the enzyme normally responsible for conjugating bilirubin. Crigler-Najjar syndrome type I manifests early in life, as bilirubin quickly builds up in the complete absence of conjugation. It is fatal if phototherapy and plasmapheresis are not quickly initiated to prevent the development of kernicterus.

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