Crigler Najjar (CN) disease and liver transplantation
| Journal | Volume 68 - 2005 |
| Issue | Fasc.4 - Symposium |
| Author(s) | M. Sinaasappel |
| Full article |
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| Erasmus MC, Rotterdam, The Netherlands |
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CN disease is caused by an inherited defect of biliru- bin conjugation and biliary secretion resulting in an increase of plasma unconjugated bilirubin (UCB) levels. The prognosis is determined by the occurrence of ker- nicterus caused by toxic levels of free UCB in the brain. In case of complete absence of bilirubin conjugation this condition start at birth, while residual capacity is associ- ated with milder course and later presentation of the dis- ease. |
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