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Evaluation of Clinical Outcomes in Children with Intrahepatic Cholestasis Postpartial External Biliary Diversion: A
Hadeer Mohamed Nasr El-Din1, Noha Adel Yassin2, Nehal M El Koofy2
1Pediatric Surgery Department, Cairo University Specialized Pediatric Hospital, Cairo University, Giza, Egypt.
Insights
Partial external biliary diversion (PEBD) effectively reduces severe itching in children with progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS). This safe surgical option improves quality of life and growth while preserving liver function.
Area of Science:
- Hepatology
- Pediatric Surgery
- Gastroenterology
Background:
- Severe pruritus in progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is often refractory to medical management.
- Surgical interruption of the enterohepatic circulation is a key strategy for symptom relief and delaying cirrhosis.
Purpose of the Study:
- To evaluate the short-term efficacy of partial external biliary diversion (PEBD) in pediatric patients with PFIC and AGS.
- Assess the impact of PEBD on pruritus, liver disease progression, growth, and quality of life.
Main Methods:
- Prospective cohort study of children with PFIC and AGS undergoing PEBD.
- Outcomes measured using pruritus scores, quality of life scales (PedsQL), growth parameters, bile acid levels, and liver function tests.
- Follow-up duration ranged from 6 to 12 months.
Main Results:
- Significant improvement in pruritus (5-D itching score, p<0.001) and quality of life (PedsQL, p<0.001).
- Marked reduction in bile acid levels (p=0.013) and amelioration of growth failure.
- No significant changes in bilirubin or other liver function tests; no intra-operative complications.
Conclusions:
- PEBD is an effective and safe treatment for intractable pruritus in PFIC and AGS.
- The procedure helps preserve synthetic liver functions.
- PEBD offers a viable surgical option for improving patient outcomes.
Background:
Severe pruritus caused by progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is refractory to medical treatment. Surgical interruption of the enterohepatic circulation is considered the mainstay of alleviating distressing symptoms and delaying cirrhosis.
Aim And Objectives:
This study aims to evaluate the short-term effect of partial external biliary diversion (PEBD) on pruritus, liver disease progression, patient's growth, and quality of life.
Material And Methods:
This prospective cohort study enrolled children with PFIC and AGS from July 2019 to July 2021, whose guardians consented to the PEBD procedure. A standard surgical approach was performed by a single surgeon. Outcomes were measured subjectively and objectively pre- and post-procedure using the pruritus 5-D itching score, Paediatric Quality of Life Inventory scale (PedsQL), growth parameters, bile acids level, and liver function tests. Patients' follow-up period ranged from 6 to 12 months.
Results:
Seven patients had PEBD procedure; five with PFIC and two with AGS. A significant improvement was detected in the 5-D itching score (p-value < 0.001), PedsQL (p-value < 0.001), and bile acids level (p-value 0.013). The preexisting growth failure was ameliorated. The downward trend in the bilirubin level was not significant. No influential difference in the other liver function tests occurred. No intra-operative complications encountered. Only one case had a post-operative stoma prolapse which was managed surgically.
Conclusion:
PEBD procedure could be considered as an effective and safe treatment options for intractable pruritus in patients with PFIC or AGS, providing preserved synthetic liver functions.
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