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Successful pregnancy after ileal exclusion in progressive familial intrahepatic cholestasis type 2.
- Source :
-
Annals of hepatology [Ann Hepatol] 2015 Jul-Aug; Vol. 14 (4), pp. 550-2. - Publication Year :
- 2015
-
Abstract
- Progressive familial intrahepatic cholestasis type 2 (PFIC 2) results from mutations in ABCB11 gene coding bile salt export pump (BSEP). Medical treatment is usually unsuccessful and surgery intervention is necessary. Partial external biliary diversion (PEBD) is regarded as the first choice of surgical treatment. Ileal exclusion (IE) is an alternative operation if external stoma is not tolerated; however, a favorable outcome is uncertain. In chronic liver diseases pregnancy brings additional risk of deterioration of liver function and generally is not recommended. We present the first case report of successful pregnancy in a genetically confirmed PFIC 2 patient after surgical conversion from PEBD to IE.
- Subjects :
- ATP Binding Cassette Transporter, Subfamily B, Member 11
ATP-Binding Cassette Transporters genetics
Adult
Cholestasis, Intrahepatic diagnosis
Cholestasis, Intrahepatic genetics
Female
Genetic Predisposition to Disease
Humans
Liver Function Tests
Mutation
Phenotype
Predictive Value of Tests
Pregnancy
Cholestasis, Intrahepatic surgery
Ileum surgery
Live Birth
Subjects
Details
- Language :
- English
- ISSN :
- 1665-2681
- Volume :
- 14
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Annals of hepatology
- Publication Type :
- Academic Journal
- Accession number :
- 26019043