SURGICAL BILIARY DIVERSION IS ASSOCIATED WITH AN INCREASED RISK OF LIVER TRANSPLANTATION OR DEATH IN ALAGILLE SYNDROME

SURGICAL BILIARY DIVERSION IS ASSOCIATED WITH AN INCREASED RISK OF LIVER TRANSPLANTATION OR DEATH INALAGILLE SYNDROME★ Shannon M. Vandriel1, Kathleen M. Loomes2, David A.Piccoli3, Elizabeth Rand3, Li-Ting Li4, Huiyu She4, JianShe Wang4, Rima L. Fawaz5, Silvia Nastasio6, Henkjan J.Verkade7, M. Kyle Jensen8, Catalina Jaramillo8, Nathalie Rock9, Irena Jankowska10, Piotr Czubkowski10, Dorota Gliwicz-Miedzińska10, Henry C. Lin11, Deirdre A. Kelly12, Catherine Larson-Nath13, Florence Lacaille14, Dominique Debray15, Saul Karpen16, Rene Romero17, Cristina Molera Busoms18, Étienne M. Sokal19, Tanguy Demaret19, Nehal M. El-Koofy20, Mohamed A. Elmonem20, Shikha S. Sundaram21, Alexander Chaidez21, Palaniswamy Karthikeyan22, Wikrom Karnsakul23, Winita Hardikar24, Sahana Shankar25, Ruben E. Quiros-Tejeira26, Seema Alam27, Pinar Bulut28, Christina Hajinicolaou29, Victorien M. Wolters30, Zerrin Önal31, Emmanuel M. Gonzales32, Emmanuel Jacquemin32, Jérôme Bouligand33, Lorenzo D'Antiga34,Emanuele Nicastro35, Noelle H. Ebel36, Jeffrey A. Feinstein37, Björn Fischler38, Henrik Arnell38, Susan Siew39, Michael O. Stormon39, Kyung Mo Kim40, Seak Hee Oh40, Amin J. Roberts41, Helen M. Evans41, Maria Camila Sanchez42, Maria Lorena Cavalieri42, Way Seah Lee43, Chatmanee Lertudomphonwanit44, Ryan T. Fischer45, Orith Waisbourd-Zinman46, James E.Squires47, Cigdem Arikan48, Jesus Quintero Bernabeu49,50, Mureo Kasahara51, Elisa Carvalho52, Cristina Targa Ferreira53, Pamela L. Valentino54,Giuseppe Indolfi55, John Eshun56, Pier Luigi Calvo57, Dev M. Desai58, Aglaia Zellos59, Antal Dezsőfi60, Sabina Wiecek61, Gabriella Nebbia62, Raquel Borges Pinto63, Maria Rogalidou64, María Legarda Tamara65, Andreanne N. Zizzo66, Jennifer Garcia67, Kathleen B. Schwarz68,Niviann Blondet69, Marisa Beretta70, Thomas Damgaard Sandahl71, Jernej Brecelj72, Cristina Gonçalves73,74,Eberhard Lurz75, Ermelinda Santos-Silva76, Nanda Kerkar77, Quais Mujawar78, Christos Tzivinikos79, Uzma Shah80, Carolina Jimenez-Rivera81, Jesus M. Banales82, Richard J. Thompson83, Bettina E. E. Hansen84,85, Binita M. Kamath86 and The Global ALagille Alliance (GALA) Study Group, (1)The Hospital for Sick Children and the University of Toronto, (2)The Children's Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, (3)The Children's Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine, (4)Children's Hospital of Fudan University, the Center for Pediatric Liver Diseases, (5)Yale University School of Medicine, New Haven, CT, (6)Boston Children's Hospital and Harvard Medical School, Boston, MA, (7)University Medical Center Groningen, (8)University of Utah, Primary Children’s Hospital, (9)Swiss Pediatric Liver Center, University Hospitals Geneva and University of Geneva,(10)The Children's Memorial Health Institute, (11)Oregon Health and Science University, (12)Birmingham Women’s & Children's Hospital NHS Trust and University of Birmingham, (13)University of Minnesota, (14)Necker‐ Enfants Malades Hospital, University of Paris, (15)National Reference Centre for Rare Pediatric Liver Diseases (Biliary Atresia and Genetic Cholestasis),Filfoie, ERN RARE LIVER, Necker‐Enfants Malades Hospital, University of Paris, (16)Children’s Healthcare ofAtlanta, (17)Children’s Healthcare of Atlanta & Emory University School of Medicine, Atlanta, GA, (18)Hospital Sant Joan De Déu, (19)Cliniques Universitaires SaintLuc, (20)Cairo University, (21)Children’s Hospital of Colorado and University of Colorado School of Medicine, (22)Leeds Teaching Hospitals NHS Trust, Leeds Children's Hospital, (23)Johns Hopkins University School of Medicine, (24)Royal Children’s Hospital, (25) Mazumdar Shaw Medical Center, Narayana Health, (26) Children's Hospital & Medical Center and University of Nebraska Medical Center, (27)Institute of Liver and Biliary Sciences, (28)Phoenix Children’s Hospital, (29)Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, (30)University Medical Center Utrecht, (31)Istanbul University Istanbul Medical Faculty, (32)Centre De Référence De l’Atrésie Des Voies Biliaires Et Des Cholestases Génétiques (AVB-CG), Fsmr Filfoie, ERN RARE LIVER, Hôpital Bicêtre, AP-HP, Faculté De Médecine Paris-Saclay, Le Kremlin-Bicêtre, and Inserm U1193, Hépatinov Université Paris-Saclay, (33)Hôpitaux Universitaires Paris-Saclay, Assistance PubliqueHôpitaux De Paris, Centre Hospitalier Universitaire De Bicêtre, (34)Ospedale Papa Giovanni XXIII, Bergamo, Italy, (35)Ospedale Papa Giovanni XXIII, (36)Stanford University School of Medicine, (37)Stanford University School of Medicine, Lucile Packard Children’s Hospital, (38)Astrid Lindgren Children's Hospital, Karolinska University Hospital and Department of Women's and Children's Health, (39)The Children’s Hospital at Westmead, (40)University of Ulsan College of Medicine, Asan Medical Center Children’s Hospital, (41)Starship Child Health, (42)Hospital Italiano Buenos Aires, (43) University of Malaya, (44)Ramathibodi Hospital Mahidol University, (45)Children's Mercy Kansas City, (46) Schneider Children's Medical Center of Israel, Institute of Gastroenterology, Nutrition and Liver Diseases, (47)UPMC Children's Hospital of Pittsburgh, (48)Koc University School of Medicine, (49)Biodonostia Health Research Institute – Donostia University Hospital –, University of the Basque Country (UPV/EHU), (50) Hospital Universiatri Vall D'hebron, (51)Organ Transplantation Center, National Center for Child Health and Development, (52)Hospital De Base Do Distrito Federal, Hospital Da Criança De Brasília, Centro Universitário De Brasília, (53)Hospital Da Criança Santo Antônio, Universidade Federal De Ciências Da Saúde De Porto Alegre, Complexo Hospitalar Santa Casa, (54) Seattle Children’s Hospital, (55)University of Florence and Meyer Children's University Hospital, (56)Le Bonheur Children's Hospital and the University of Tennessee Health Science Center, (57)Regina Margherita Children’s Hospital, Azienda Ospedaliera-Universitaria Citta' Della Salute e Della Scienza, (58)Children's Health – Children's Medical Center, (59)Aghia Sophia Children's Hospital, National and Kapodistrian University of Athens, Athens, Greece, (60)Semmelweis University, (61)Medical University of Silesia in Katowice, (62)Fondazione Irccs Ca’ Granda Ospedale Maggiore Policlinico, (63)Hospital Da Criança Conceição Do Grupo Hospitalar Conceição, (64)Agia Sofia Children's Hospital, University of Athens, (65)Cruces University Hospital, (66)Children's Hospital, London Health Sciences Centre, Western University, (67)Miami Transplant Institute, University of Miami, (68) University of California San Diego, Rady Children’s Hospital San Diego, (69)Seattle Children’s Hospital, Seattle, (70)Wits Donald Gordon Medical Centre, University of the Witwatersrand, (71)Aarhus University, (72)University Medical Center Ljubljana, (73)European Reference Network on Hepatological Diseases (ERN RARE-LIVER), (74)Pediatric Gastroenterology/ Hepatology Center Lisbon, (75)Von Hauner Children's Hospital, University Hospital, Lmu Munich, (76)Centro Hospitalar Universitário De Santo António, (77)University of Rochester Medical Center, (78)University of Manitoba, (79)Al Jalila Children’s Specialty Hospital, Mohammed Bin Rashid University of Medicine and Health Sciences, (80)Harvard Medical School, Massachusetts General Hospital for Children, Royal Oak, MI, (81)Children's Hospital of Eastern Ontario, (82)Biodonostia Health Research Institute - Donostia University Hospital, Universidad Del País Vasco (UPV/EHU), Centro De Investigación Biomédica En Red De Enfermedades Hepáticas y Digestivas (CIBERehd), (83)Institute of Liver Studies, King's College London, London, United Kingdom, (84)Toronto General Hospital University Health Network, (85)Institute of Health Policy, Management and Evaluation, (86)Division of Gastroenterology, Hepatology and Nutrition, the Hospital for Sick Children, Toronto, ON, Canada Background: Alagille syndrome (ALGS) is an inherited liver disorder dominated by high γ-glutamyltransferase (GGT) cholestasis. Previous studies have demonstrated limited efficacy of surgical interruption of the enterohepatic circulation in ALGS, with varying degrees of improvement in pruritus and xanthomas. Utilizing theGALA database, we sought to evaluate whether surgicalbiliary diversion (SBD) alters the natural history of liver disease. Methods: Multicenter retrospective analysis of children with clinically and/or genetically confirmed ALGS. Laboratory data were collected preoperatively (−6 to 0 mo) and postoperatively (3 to 12 mo). Paired sample t-tests were used to compare continuous variables, and McNemar’s tests were used to compare binominal variables pre-and postoperatively. Receiver operating characteristic (ROC) curves were used to determine the optimal laboratory threshold for predicting native liver survival (NLS) following SBD. Cox proportional hazards models were constructed to determine NLS in ALGS-SBD patients. Results: Of 1673 ALGS patients, 3.7% (n=62; 54.8% male) underwent SBD from 26 centers. The median age of SBD was 2.5 years (IQR 1.8 – 4.4). Most ALGS patients underwent a partial external biliary diversion (54.8%, n =34), followed by a partial internal biliary diversion in 19.4% (n =12) and ileal exclusion in 12.9% (n=8). 100% (n=62) of patients reported pruritus at the time of SBD, and 51.4% (n =18/35) reported xanthomas. ALGS-SBD patients had a 2.5-fold greater risk of liver transplantation (LT) or death (95%CI 1.6 – 3.9; p<0.01). Following SBD, there were no significant differences in total bilirubin (TB) (8.8 vs. 9.1 mg/dL, p=0.51), ALT (159.5 vs. 189.7 U/L, p=0.38),GGT (495.0 vs. 459.5 U/L, p=0.21) or cholesterol (493.0 vs. 414.6 mg/dL, p=0.21). Availability of serum bile acids (SBA) was limited; however, in 10 patients with SBAs preand postoperatively, there was a significant reduction following SBD (257.2 vs. 97.7 μmol, p=0.05). Amon

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