Liver transplantation in Crigler-Najjar syndrome type I disease.
Hepatobiliary Pancreat Dis Int
; 11(5): 545-8, 2012 Oct.
Article
en En
| MEDLINE
| ID: mdl-23060403
ABSTRACT
BACKGROUND:
Crigler-Najjar syndrome type I (CNS I) is a very rare autosomal recessive inherited disease that liver transplantation can properly deal with.METHODS:
We present one case of an 18-month-old child with CNS I diagnosed by clinical findings and genetic detecting. LTx was performed 5 days after kernicterus broke out and neurological symptoms were successfully reversed.RESULT:
Magnetic resonance imaging and magnetic resonance spectroscopy showed encouraging results that brain pathology had a trend to return to normal in 1-year follow-up, combined with electroencephalogram and motor development estimate studies.CONCLUSIONS:
Liver transplantation can cure CNS I with reversible neurological symptoms to some extent in time. Magnetic resonance spectroscopy may be a future option of predicting brain conditions and selecting suitable patients with CNS I for transplantation.
Buscar en Google
Banco de datos:
MEDLINE
Asunto principal:
Trasplante de Hígado
/
Síndrome de Crigler-Najjar
Tipo de estudio:
Prognostic_studies
Límite:
Humans
/
Male
/
Newborn
Idioma:
En
Revista:
Hepatobiliary Pancreat Dis Int
Asunto de la revista:
GASTROENTEROLOGIA
Año:
2012
Tipo del documento:
Article
País de afiliación:
China