Síndrome de Alagille y trasplante hepático / Alagille's syndrome and hepatic transplantation
Bol. pediatr
; 48(205): 276-278, 2008. ilus, tab
Article
in Es
| IBECS
| ID: ibc-68027
Responsible library:
ES15.1
Localization: ES15.1 - BNCS
RESUMEN
El síndrome de Alagille es una entidad infrecuente. Es un trastorno autosómico dominante, con gran penetrancia y expresividad variable. Se caracteriza por una hipoplasiade vías biliares intrahepáticas que puede manifestarse en el primer trimestre de vida. Se asocia a diversas manifestaciones clínicas (cardíacas, oftalmológicas, vertebrales, faciespeculiar
). Presentamos el caso de un paciente que manifestó ictericia durante los primeros días de vida, siendo posteriormente diagnosticado del síndrome. Al año de vida se realizó trasplante, con buena respuesta clínica tras ser retrasplantado por rechazo agudo. Tres años después presentó rechazo crónico, siendo sometido a tratamiento inmunosupresor, tras el cual precisó varios ingresos por neutropenia (AU)
ABSTRACT
Alagille's syndrome in an uncommon disease. It is adominant autosomal disorder with great penetrance and variable expressivity. It is characterized by intrahepatic biliaryhypoplasia that may be seen in the first three months of life. It is associated to diverse clinical manifestations (cardiac, ophthalmological, vertebral, characteristic facies, etc.).We present the case of a male patient who presented with jaundice during the first days of life, subsequently being diagnosed of the syndrome. At one year of life, a transplant was performed, with good clinical response after being retransplanted due to acute rejection. Three years later, he had chronic rejection, being subjected to immunosuppressant treatment, after which he required several admissions due to neutropenia (AU)
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Collection:
National databases
/
Spain
Database:
IBECS
Main subject:
Tonsillectomy
/
Adenoidectomy
/
Immunosuppression Therapy
/
Liver Transplantation
/
Alagille Syndrome
/
Jaundice
Limits:
Humans
/
Male
/
Infant, Newborn
Language:
Spanish
Journal:
Bol. pediatr
Year:
2008
Document type:
Article
Institution/Affiliation country:
Complejo asistencial de León/España