Management of Crigler-Najjar syndrome type I

Citation
Ha. Al-shurafa et al., Management of Crigler-Najjar syndrome type I, SAUDI MED J, 22(6), 2001, pp. 486-489
Citations number
22
Categorie Soggetti
General & Internal Medicine
Journal title
SAUDI MEDICAL JOURNAL
ISSN journal
03795284 → ACNP
Volume
22
Issue
6
Year of publication
2001
Pages
486 - 489
Database
ISI
SICI code
0379-5284(200106)22:6<486:MOCSTI>2.0.ZU;2-3
Abstract
Crigler-Najjar Syndrome type I is a rare congenital disease with high morta lity and morbidity rates due to brain complications. It has been treated by life-long phototherapy until the era of liver transplantation. Liver trans plantation is currently the only curative treatment for this syndrome. Live r transplantation prevents the severe neurological complications that are t he main cause of life-long disability in Crigler-Najjar Syndrome type I. Th e ideal age for transplantation is 3-5 years. Despite the advent of auxilia ry transplantation we believe that orthotopic liver transplantation is the optimal treatment and the ideal method of liver transplantation for Crigler -Najjar Syndrome type I.