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.
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.
Authors: P Ramachandran; N P Shanmugam; S Al Sinani; V Shanmugam; S Srinivas; M Sathiyasekaran; V Tamilvanan; M Rela Journal: Pediatr Surg Int Date: 2014-07-27 Impact factor: 1.827
Authors: Kasper S Wang; Greg Tiao; Lee M Bass; Paula M Hertel; Douglas Mogul; Nanda Kerkar; Matthew Clifton; Colleen Azen; Laura Bull; Philip Rosenthal; Dylan Stewart; Riccardo Superina; Ronen Arnon; Molly Bozic; Mary L Brandt; Patrick A Dillon; Annie Fecteau; Kishore Iyer; Binita Kamath; Saul Karpen; Frederick Karrer; Kathleen M Loomes; Cara Mack; Peter Mattei; Alexander Miethke; Kyle Soltys; Yumirle P Turmelle; Karen West; Jessica Zagory; Cat Goodhue; Benjamin L Shneider Journal: Hepatology Date: 2017-03-22 Impact factor: 17.425
Authors: Irena Jankowska; Joanna Pawłowska; Marek Szymczak; Hor Ismail; Dorota Broniszczak; Joanna Cielecka-Kuszyk; Piotr Socha; Dorota Jarzębicka; Piotr Czubkowski Journal: Am J Case Rep Date: 2021-07-20
Authors: Laura N Bull; Ludmila Pawlikowska; Sandra Strautnieks; Irena Jankowska; Piotr Czubkowski; Jennifer L Dodge; Karan Emerick; Catherine Wanty; Sami Wali; Samra Blanchard; Florence Lacaille; Jane A Byrne; Albertien M van Eerde; Kaija-Leena Kolho; Roderick Houwen; Steven Lobritto; Vera Hupertz; Patricia McClean; Giorgina Mieli-Vergani; Etienne Sokal; Philip Rosenthal; Peter F Whitington; Joanna Pawlowska; Richard J Thompson Journal: Hepatol Commun Date: 2018-03-30