Quality of Life in Patients with Progressive Familial Intrahepatic Cholestasis: No Difference between Post Liver Transplantation and Post Partial External Biliary Diversion

Objectives: In patients with progressive familial intrahepatic cholestasis (PFIC), partial external biliary diversion (PEBD), which is associated with a permanent stoma, is recommended as first-line therapy, whereas primary liver transplantation (LTx) is restricted to those with cirrhosis. Our aim was to quantify the health-related quality of life (HRQOL) in PFIC patients and to evaluate whether there is a difference in their HRQOL depending on the surgical approach. Methods: A prospective HRQOL study on a consecutive series of PFIC was conducted using Pediatric Quality of Life Inventory 4.0 child-self and parent-proxy reports. PFIC patients after PEBD who still lived with their native livers were compared to those after LTx. Both groups were compared to healthy children. Results: 32 patients (53% female) patients with a mean age of 17.7 ± 7.3 years were studied. 22 had undergone LTx at a mean age of 7.8 ± 3.8 years and 10 had undergone PEBD at at mean age of 4.1 ± 3.9 years. At the time of HRQOL assessment, the mean age was 18.9 ± 7.5 years in the LTx group and 15.3 ± 6.5 years in the PEBD group. Child-self and parent-proxy reports showed no significant difference in HRQOL between PFIC patients after LTx and those after PEBD except for marginal difference in physical functioning/health (p = 0.07). Except for a lower score in patient school functioning of patients after LTx (p = 0.01), HRQOL-results showed no difference from healthy children in any group. Conclusions: The HRQOL of PFIC patients after PEBD was similar to those after LTx. The HRQOL in both groups was also similar to that of healthy children. Thus, our data supports the current policy of PEBD as primary surgical treatment for PFIC patients without cirrhosis. Address correspondence and reprint requests to Nagoud Schukfeh, MD, Department of Pediatric Surgery, Hannover Medical School, 30625 Hannover, Germany (e-mail: schukfeh.nagoud@mh-hannover.de). Received 4 January, 2018 Accepted 9 July, 2018 Author contribution Sonja Wasman: Conception, data acquisition and analysis, drafting manuscript Eva D. Pfister: Data acquisition and analysis and drafting and revising manuscript Benno M. Ure: Conception, interpretation, drafting and revising manuscript Imeke Goldschmidt: Data interpretation, analysis, drafting manuscript Jens Dingemann: Data interpretation, analysis, drafting and revising manuscript Ulrich Baumann: Data interpretation, analysis, drafting manuscript Joachim F. Kuebler: Conception and design, interpretation, revising manuscript Nagoud Schukfeh: Conception, design, data acquisition and analysis, drafting and revising manuscript Sonja Wassman and Eva-Doreen Pfister equally contributed to this manuscript and shared first authorship. Conflicts of interest and source of funding: All authors state that they have no financial support or grant and no conflict of interest to declare. Trial identification number (Ethical committee): 3683-2017 © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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