Ileal exclusion in children with progressive familial intrahepatic cholestasis.
J Pediatr Gastroenterol Nutr
; 58(1): 92-5, 2014 Jan.
Article
em En
| MEDLINE
| ID: mdl-24385022
ABSTRACT
OBJECTIVES:
Children with progressive familial intrahepatic cholestasis (PFIC) rarely benefit from medical treatment and most patients require surgical intervention. Partial external biliary diversion (PEBD) is presently the treatment of choice but for those who cannot benefit from PEBD, an alternative surgical procedure--ileal exclusion (IE)--was introduced. The aim of this study was to analyze our experience with IE in children with PFIC.METHODS:
This procedure was performed in 9 patients (6 girls, 3 boys) at the median age of 11 years (range 8-21). In 4 children, it was the primary operation (group 1), and in 5, IE was performed after PEBD (group 2). All of the patients were screened for ABCB11 and ATP8B1 mutations, and in 3 cases, PFIC type 2 was confirmed.RESULTS:
Median follow-up after IE surgery was 8.5 years (range 3-14). In group 1, 1 patient had to be converted to PEBD and the remaining 3 children experienced alleviation in pruritus and decrease in bilirubin and bile acids concentrations 2 and 5 years after IE. After 10 years, only 2 children were still accessible for follow-up. In both, pruritus varied and elevated serum bile acids were observed. Of the 5 patients who underwent IE after PEBD, 1 eventually required liver transplantation, 1 developed varying degree of pruritus, and 3 female patients, operated on because of aesthetic reasons, had excellent outcomes.CONCLUSIONS:
IE is an alternative rescue option to PEBD and should be offered cautiously, only to patients who cannot benefit from PEBD.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Prurido
/
Procedimentos Cirúrgicos do Sistema Digestório
/
Bilirrubina
/
Ácidos e Sais Biliares
/
Colestase Intra-Hepática
/
Íleo
/
Fígado
Tipo de estudo:
Etiology_studies
/
Evaluation_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
J Pediatr Gastroenterol Nutr
Ano de publicação:
2014
Tipo de documento:
Article
País de afiliação:
Polônia