Partial Internal Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: Introduction of a New Approach
نویسندگان
چکیده
INTRODUCTION Facilitation of biliary salts secretion represents the mainstay of treatment for progressive familial intrahepatic cholestasis (PFIC). The purpose of this study was to introduce a new approach for the treatment of progressive familial intrahepatic cholestasis (PFIC) to avoid ostoma. CASE PRESENTATION An 11-year-old girl with the diagnosis of PFIC underwent cholecystoappendicostomy with myotomy operation. Because of anastomosis stricture, she was reoperated with cholecystojejunocolic anastomosis and intussuscepted valve surgery. She was followed for 9 months. Despite disappointing outcomes of internal drainage with cholecystoappendicostomy, results of cholecystojejunocolic anastomosis with intussuscepted valve surgery were promising. DISCUSSION The cholecystojejunocolic anastomosis with intussuscepted valve surgery could be considered as a forthcoming approach in the treatment of intrahepatic cholestasis.
منابع مشابه
Current and future therapies for inherited cholestatic liver diseases
Familial intrahepatic cholestasis (FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phenotypes range from benign recurrent intrahepatic cholestasis (BRIC), associated with recurrent cholestatic attacks, to progressive FIC (PFIC). Patients often suffer from severe pruritus a...
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