Elevated Serum Bile Acids Predict Poor Liver Outcomes in Children With Alagille Syndrome: Results From the GALA Study Group

Por: Perez CFM, Vandriel SM, Gonzales EM, Wang JS, Li LT, She H, Jankowska I, Czubkowski P, Gliwicz-Miedzinska D, Jacquemin E, Bouligand J, D'Antiga L, Nicastro E, Fischler B, Arnell H, Siew SM, Stormon M, Loomes KM, Piccoli DA, Rand EB, Squires JE, Karpen SJ, Romero R, Kasahara M, Önal Z, Sokal É, Demaret T, Wiecek S, Lacaille F, Debray D, Hardikar W, Shankar S, Valentino PL, Sundaram SS, Ebel NH, Feinstein JA, Waisbourd-Zinman O, Lin HC, Rock N, Verkade HJ, Jensen MK, Jaramillo C, Kim KM, Oh SH, Brecemiclj J, Alam S, Indolfi G, Blondet N, Fawaz R, Nastasio S, Calvo PL, Nebbia G, Arikan C, Larson-Nath C, Zizzo AN, Sandahl TD, Tzivinikos C, El-Koofy NM, Elmonem MA, Aqul AA, Karnsakul W, Bulut P, Kerkar N, Wolters VM, Roberts AJ, Evans HM, Sanchez MC, Cavalieri ML, Kelly DA, Hartley J, Lee WS, Hajinicolaou C, Lertudomphonwanit C, Fischer RT, Bernabeu JQ, Quiros-Tejeira RE, Ferreira CT, Carvalho E, Eshun J, Zellos A, Dezsofi A, Pinto RB, Schwarz K, Rogalidou M, Garcia J, Tamara ML, Beretta M, Mujawar Q, Santos-Silva E, Molera C, Lurz E, Gonçalves C, Jimenez-Rivera C, Banales JM, Thompson RJ, Hansen BE and Kamath BM

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Publicada: 18 nov 2025

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Filiaciones:
Perez CFM:
 Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children and the University of Toronto, Toronto, Canada

Vandriel SM:
 Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children and the University of Toronto, Toronto, Canada

Gonzales EM:
 Pediatric Hepatology and Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases, FILFOIE, ERN RARE LIVER, Bicêtre Hospital, AP-HP, Université Paris-Saclay, Le Kremlin-Bicêtre, and Inserm U1193, Hepatinov, University of Paris-Saclay, Orsay, France

Wang JS:
 Children's Hospital of Fudan University, The Center for Pediatric Liver Diseases, Shanghai, China

Li LT:
 Children's Hospital of Fudan University, The Center for Pediatric Liver Diseases, Shanghai, China

She H:
 Children's Hospital of Fudan University, The Center for Pediatric Liver Diseases, Shanghai, China

Jankowska I:
 Department of Gastroenterology, Hepatology, Nutrition Disturbances and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland

Czubkowski P:
 Department of Gastroenterology, Hepatology, Nutrition Disturbances and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland

Gliwicz-Miedzinska D:
 Department of Gastroenterology, Hepatology, Nutrition Disturbances and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland

Jacquemin E:
 Department of Gastroenterology, Hepatology, Nutrition Disturbances and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland

Bouligand J:
 Service de Génétique Moléculaire, Pharmacogénétique et Hormonologie, Hôpitaux Universitaires Paris-Saclay, Assistance Publique-Hôpitaux de Paris, Centre Hospitalier Universitaire de Bicêtre, Le Kremlin-Bicêtre, France

D'Antiga L:
 Ospedale Papa Giovanni XXIII, Pediatric Hepatology, Gastroenterology and Transplantation, Bergamo, Italy

 Department of Medicine and Surgery, University of Milano, Milan, Italy

Nicastro E:
 Ospedale Papa Giovanni XXIII, Pediatric Hepatology, Gastroenterology and Transplantation, Bergamo, Italy

Fischler B:
 Department of Paediatric Gastroenterology, Hepatology and Nutrition, Astrid Lindgren Children's Hospital, Karolinska University Hospital and CLINTEC, Karolinska Institutet, Stockholm, Sweden

Arnell H:
 Department of Paediatric Gastroenterology, Hepatology and Nutrition, Astrid Lindgren Children's Hospital, Karolinska University Hospital and Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden

Siew SM:
 Department of Gastroenterology, The Children's Hospital at Westmead, Sydney, Australia

Stormon M:
 Department of Gastroenterology, The Children's Hospital at Westmead, Sydney, Australia

Loomes KM:
 Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA

Piccoli DA:
 Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA

Rand EB:
 Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA

Squires JE:
 Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA

Karpen SJ:
 Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, Georgia, USA

 Stravitz-Sanyal Institute for Liver Disease and Metabolic Health at Virginia Commonwealth University, Richmond, Virginia, USA

Romero R:
 Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, Georgia, USA

Kasahara M:
 Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan

Önal Z:
 Pediatric Gastroenterology, Hepatology and Nutrition Department, Istanbul University Istanbul Medical Faculty, Istanbul, Turkey

Sokal É:
 Cliniques Universitaires Saint-Luc, Service De Gastroentérologie and Hépatologie Pédiatrique, Brussels, Belgium

Demaret T:
 Cliniques Universitaires Saint-Luc, Service De Gastroentérologie and Hépatologie Pédiatrique, Brussels, Belgium

Wiecek S:
 Department of Pediatrics, Medical University of Silesia, Katowice, Poland

Lacaille F:
 Pediatric Gastroenterology-Nutrition and Hepatology Units, Necker-Enfants Malades Hospital, University of Paris, Paris, France

Debray D:
 Pediatric Liver Unit, National Reference Centre for Rare Pediatric Liver Diseases (Biliary Atresia and Genetic Cholestasis), FILFOIE, ERN RARE LIVER, Necker-Enfants Malades Hospital, University of Paris, Paris, France

Hardikar W:
 Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Australia

Shankar S:
 Mazumdar Shaw Medical Center, Narayana Health, Bangalore, India

Valentino PL:
 Gastroenterology and Hepatology Division, Department of Pediatrics, University of Washington, Seattle Children's Hospital, Seattle, Washington, USA

Sundaram SS:
 Section of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics and the Digestive Health Institute, Children's Hospital of Colorado and University of Colorado School of Medicine, Aurora, Colorado, USA

Ebel NH:
 Division of Gastroenterology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California, USA

Feinstein JA:
 Department of Pediatrics (Cardiology), Stanford University School of Medicine, Lucile Packard Children's Hospital, Palo Alto, California, USA

Waisbourd-Zinman O:
 Schneider Children's Medical Center of Israel, Institute of Gastroenterology, Nutrition and Liver Diseases, Petah Tikva, Israel

Lin HC:
 Division of Pediatric Gastroenterology, Department of Pediatrics, Oregon Health and Science University, Portland, Oregon, USA

Rock N:
 Division of Pediatric Specialties, Department of Pediatrics, Gynecology, and Obstetrics, Swiss Pediatric Liver Center, University Hospitals Geneva and University of Geneva, Geneva, Switzerland

Verkade HJ:
 Department of Pediatrics, Center for Liver, Digestive, and Metabolic Diseases, University Medical Center Groningen, Groningen, the Netherlands

Jensen MK:
 Division of Pediatric Gastroenterology, Hepatology and Nutrition, Primary Children's Hospital, University of Utah, Salt Lake City, Utah, USA

Jaramillo C:
 Division of Pediatric Gastroenterology, Hepatology and Nutrition, Primary Children's Hospital, University of Utah, Salt Lake City, Utah, USA

Kim KM:
 Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea

Oh SH:
 Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea

Brecemiclj J:
 Pediatric Gastroenterology, Hepatology and Nutrition, and Department of Pediatrics, Faculty of Medicine, University Medical Center Ljubljana, Ljubljana, Slovenia

Alam S:
 Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India

Indolfi G:
 Meyer Children's University Hospital IRCCS, Florence, Italy

Blondet N:
 Gastroenterology and Hepatology Division, Department of Pediatrics, University of Washington, Seattle Children's Hospital, Seattle, Washington, USA

Fawaz R:
 Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut, USA

Nastasio S:
 Division of Gastroenterology, Hepatology, and Nutritions, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA

Calvo PL:
 Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Citta' della Salute e della Scienza, Turin, Italy

Nebbia G:
 Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Servizio di Epatologia Pediatrica, Milan, Italy

Arikan C:
 Department of Pediatric Gastroenterology and Organ Transplant, Koc University School of Medicine, Istanbul, Turkey

Larson-Nath C:
 Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of Minnesota, Minneapolis, Minnesota, USA

Zizzo AN:
 Division of Paediatric Gastroenterology and Hepatology, Children's Hospital, London Health Sciences Centre, Western University, London, Ontario, Canada

Sandahl TD:
 Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark

Tzivinikos C:
 Department of Paediatric Gastroenterology, Al Jalila Children's Specialty Hospital, Mohammed Bin Rashid University of Medicine and Health Sciences, Dubai, UAE

El-Koofy NM:
 Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt

Elmonem MA:
 Department of Clinical and Chemical Pathology, Faculty of Medicine, Cairo University, Cairo, Egypt

Aqul AA:
 University of Texas Southwestern Medical Center in Dallas and Children's Health, Dallas, Texas, USA

Karnsakul W:
 Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

Bulut P:
 Division of Pediatric Gastroenterology and Hepatology, Phoenix Children's Hospital, Phoenix, Arizona, USA

Kerkar N:
 Massachusetts General Brigham for Children, Boston, Massachusetts, USA

 University of Rochester Medical Center, Rochester, New York, USA

Wolters VM:
 Department of Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, the Netherlands

Roberts AJ:
 Department of Paediatric Gastroenterology, Starship Child Health, Auckland, New Zealand

Evans HM:
 Department of Paediatric Gastroenterology, Starship Child Health, Auckland, New Zealand

Sanchez MC:
 Hospital Italiano Buenos Aires, Pediatric Gastroenterology and Hepatology Division, Buenos Aires, Argentina

Cavalieri ML:
 Hospital Italiano Buenos Aires, Pediatric Gastroenterology and Hepatology Division, Buenos Aires, Argentina

Kelly DA:
 Liver Unit, Birmingham Women's and Children's Hospital NHS Trust and University of Birmingham, Birmingham, UK

Hartley J:
 Liver Unit, Birmingham Women's and Children's Hospital NHS Trust and University of Birmingham, Birmingham, UK

Lee WS:
 Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia

Hajinicolaou C:
 Division of Paediatric Gastroenterology, Department of Paediatrics and Child Health, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa

Lertudomphonwanit C:
 Division of Gastroenterology, Department of Pediatrics, Ramathibodi Hospital Mahidol University, Bangkok, Thailand

Fischer RT:
 Section of Hepatology, Department of Gastroenterology, Children's Mercy Kansas City, Kansas City, Kansas, USA

Bernabeu JQ:
 Pediatric Hepatology and Liver Transplant Department, Hospital Universiatri Vall d'Hebron, Barcelona, Spain

 Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital - University of the Basque Country (UPV/EHU), San Sebastian, Spain

Quiros-Tejeira RE:
 Department of Pediatrics, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha, Nebraska, USA

Ferreira CT:
 Pediatric Gastroenterology Service, Hospital da Criança Santo Antônio, Universidade Federal de Ciências da Saúde de Porto Alegre, Complexo Hospitalar Santa Casa, Porto Alegre, Rio Grande do Sul, Brazil

Carvalho E:
 Pediatric Gastroenterology Department, Hospital de Base do Distrito Federal, Hospital da Criança de Brasília, Centro Universitário de Brasília, Brasília, Distrito Federal, Brazil

Eshun J:
 Department of Pediatric Gastroenterology, Le Bonheur Children's Hospital and The University of Tennessee Health Science Center, Memphis, Tennessee, USA

Zellos A:
 Mitera Children's Hospital, Athens, Greece

Dezsofi A:
 First Department of Paediatrics, Semmelweis University, Budapest, Hungary

Pinto RB:
 Division of Pediatric Gastroenterology, Hospital Criança Conceição, Grupo Hospitalar Conceição, Porto Alegre, Rio Grande do Sul, Brazil

Schwarz K:
 Division of Pediatric Gastroenterology, Rady Children's Hospital San Diego, University of California San Diego, San Diego, California, USA

Rogalidou M:
 Division of Gastroenterology and Hepatology, First Department of Paediatrics, University of Athens, "Aghia Sofia" Children's Hospital, Athens, Greece

Garcia J:
 Division of Pediatric Gastroenterology, Hepatology and Nutrition, Miami Transplant Institute, University of Miami, Miami, Florida, USA

Tamara ML:
 Paediatric Gastroenterology Unit, Cruces University Hospital, Bilbao, Spain

Beretta M:
 Faculty of Health Sciences, Wits Donald Gordon Medical Centre, University of the Witwatersrand, Johannesburg, South Africa

Mujawar Q:
 Section of Pediatric Gastroenterology, Department of Pediatrics, University of Manitoba, Winnipeg, Canada

Santos-Silva E:
 Centro Hospitalar Universitário de Santo António, Centro Materno-Infantil do Norte Albino Aroso, Porto, Portugal

 UCIBIO - Applied Molecular Biosciences Unit, Biochemistry Laboratory, Department of Biological Sciences, Faculdade de Farmácia, Universidade do Porto, Porto, Portugal

Molera C:
 Pediatric Gastroenterology Hepatology and Nutrition Unit, Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain

Lurz E:
 Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, LMU Munich, Munich, Germany

Gonçalves C:
 European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany

 Pediatric Gastroenterology/Hepatology Center Lisbon, Lisbon, Portugal

Jimenez-Rivera C:
 Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, Canada

Banales JM:
 Department of Hepatology and Gastroenterology, 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), San Sebastián, Spain

Thompson RJ:
 Institute of Liver Studies, King's College London, London, UK

Hansen BE:
 Toronto General Hospital University Health Network, Toronto, Canada

 Institute of Health Policy, Management and Evaluation, Toronto, Canada

Kamath BM:
 Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children and the University of Toronto, Toronto, Canada

 Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA