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- Title
Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients.
- Authors
Foroutan, Hamid Reza; Bahador, Ali; Ghanim, Sultan Mohsin; Dehghani, Seyed Mohsen; Anbardar, Mohammad Hossein; Fattahi, Mohammad Reza; Forooghi, Mehdi; Azh, Omidreza; Tadayon, Ali; Sherafat, Alireza; Yaghoobi, Amir Arsalan; Ashraf, Mohammad Ali
- Abstract
<bold>Purpose: </bold>Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease characterized by cholestasis, which may cause jaundice, severe pruritus, and cirrhosis in the later stages. By the invention of biliary diversion methods, these patients were prevented from undergoing liver transplant. Using biliary diversion techniques, the entero-hepatic cycle was interrupted. This lowers the bile acid pool and resolves the pruritus. Herein, we report 44 cases of PFIC who underwent partial internal biliary diversion (PIBD) and long-term follow-up of these children. This comprises the largest case series of PIBD.<bold>Methods: </bold>All patients were diagnosed by liver biopsy as PFIC before the operation. All underwent cholecysto colic bypass by jejunal interposition due to severe pruritus unresponsive to medication. Laboratory blood tests, sonography, and physical exam were done before and after the operation once every 3 months. Besides, a questionnaire was designed to ask the patients about the symptoms after the operation, and a pruritus score was measured using the 5D-itch scale.<bold>Results: </bold>44 children (25 boys, 19 girls), between 1.75 and 27.5 years (at the time of this study) were followed for a median period of 54 months. Age at operation ranged from 2 months to 18 years, with a median of 29 months. Of these children, 14 were lost to follow up. Results showed a significant decrease in pruritus and sleep disturbance after the surgery (p < 0.001). Also, jaundice decreased from 82.1 before to 7.1% following the surgery. 50% of the patients became medication-free at follow-up.<bold>Conclusion: </bold>PIBD is a safe procedure which helps non-cirrhotic children preserve their liver function. Therefore, PIBD prevents them from undergoing liver transplant. Effective results were achieved in terms of severe pruritus and jaundice, and children were able to regain their sleep patterns. It also avoided external stoma, which is more convenient from the patient's point of view.
- Subjects
CHOLESTASIS; GENETIC disorders; LIVER transplantation; LIVER biopsy; JEJUNOILEAL bypass; BLOOD testing; BILIARY atresia; BILIARY liver cirrhosis; BIOPSY; ULTRASONIC imaging; SURGICAL anastomosis; OPERATIVE surgery; TIME; TREATMENT effectiveness; GALLBLADDER; BILIARY tract surgery; LONGITUDINAL method
- Publication
Pediatric Surgery International, 2020, Vol 36, Issue 5, p603
- ISSN
0179-0358
- Publication type
journal article
- DOI
10.1007/s00383-020-04641-z