A complex case of low-phospholipid-associated cholelithiasis syndrome.
Rev Esp Enferm Dig
; 112(7): 573-574, 2020 Jul.
Article
em En
| MEDLINE
| ID: mdl-32543870
ABSTRACT
The low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic and recurring cholelithiasis occurring in young adults, associated with mutations in the ABCB4 gene. It is a clinical syndrome characterized by at least two of the following criteria age at onset of biliary symptoms below 40 years, intrahepatic echogenic foci or microlithiasis and recurrence of biliary symptoms after cholecystectomy. In the rare cases progressing to end-stage liver disease, a liver transplant may be indicated. We report a case of a 40-year-old female patient with clinical criteria for LPAC syndrome and with ABCB4 gene mutation. She had a complex history of choledocholithiasis recurrence despite treatment with ursodeoxycholic acid and multiple therapeutic endoscopic retrograde cholangiopancreatography, and she developed portal vein thrombosis.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Ácido Ursodesoxicólico
/
Coledocolitíase
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Portugal