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Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
Ileal exclusion in children with progressive familial intrahepatic cholestasis
Irena Jankowska1, Piotr Czubkowski, Piotr Kaliciński
1*Department of Gastroenterology, Hepatology, and Eating Disorders †Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland.
Insights
Ileal exclusion (IE) offers a potential alternative surgical option for children with progressive familial intrahepatic cholestasis (PFIC) when other treatments fail. This study suggests IE should be cautiously considered for patients not benefiting from partial external biliary diversion (PEBD).
Area of Science:
- Hepatology
- Pediatric Surgery
- Gastroenterology
Background:
- Progressive familial intrahepatic cholestasis (PFIC) is a group of rare genetic liver diseases in children.
- Medical treatments for PFIC are often ineffective, necessitating surgical interventions.
- Partial external biliary diversion (PEBD) is a primary surgical option, but alternatives are needed for non-responders.
Purpose of the Study:
- To evaluate the efficacy and outcomes of ileal exclusion (IE) as a surgical treatment for pediatric PFIC.
- To analyze the experience with IE in children who either underwent it as a primary procedure or after PEBD.
Main Methods:
- A retrospective analysis of 9 pediatric PFIC patients (median age 11 years) who underwent IE.
- Patients were divided into two groups: primary IE (4 patients) and IE after PEBD (5 patients).
- Genetic screening for ABCB11 and ATP8B1 mutations was performed; PFIC type 2 was confirmed in 3 cases.
Main Results:
- After a median follow-up of 8.5 years, 3 of 4 primary IE patients showed symptom improvement (pruritus, bilirubin, bile acids).
- Long-term follow-up (10 years) in primary IE cases showed variable pruritus and elevated bile acids in the 2 accessible patients.
- In the post-PEBD IE group, 1 patient required liver transplantation, 1 had recurrent pruritus, and 3 had excellent outcomes for aesthetic reasons.
Conclusions:
- Ileal exclusion (IE) serves as a viable alternative surgical option for pediatric PFIC patients.
- IE should be cautiously considered for patients who do not achieve adequate benefit from partial external biliary diversion (PEBD).
- Further research is warranted to optimize patient selection and long-term outcomes for IE in PFIC.
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.
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