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Alagille Syndrome
Progressive Familial Intrahepatic Cholestasis

Partial external biliary diversion shows promise in treating severe pruritus in pediatric liver disorders

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Partial external biliary diversion (PEBD) procedure shows promise as an effective and safe treatment option for severe pruritus in children diagnosed with progressive familial intrahepatic cholestasis (PFIC) or Alagille syndrome (ALGS), according to a study.

The study conducted on a cohort of patients (N = 7) demonstrated significant improvements in pruritus severity, overall quality of life, and liver function markers following the PEBD procedure. The procedure also appeared to positively impact patient growth and ameliorate growth failure.

The authors note that further research is needed to understand its long-term effects and broader applicability, but suggests that PEBD could be a valuable intervention for managing pruritus that has been difficult to address through traditional medical treatments.

Reference
Nasr El-Din HM, Yassin NA, El Koofy NM, et al. Evaluation of Clinical Outcomes in Children with Intrahepatic Cholestasis Postpartial External Biliary Diversion: A Single-Center Experience. J Indian Assoc Pediatr Surg. 2023;28(4):293-299. doi: 10.4103/jiaps.jiaps_49_23. Epub 2023 Jul 11. PMID: 37635886; PMCID: PMC10455711.

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