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Mattsson 21 and Buket Dalgic 22 , (1)University Childrens Hospital Tbingen, (2)Akdeniz University, (3)Hannover Medical School, Hannover, Germany, (4)The Childrens Memorial Health Institute, (5)Birmingham Womens and Childrens NHS Foundation Trust, (6)Istanbul University, Istanbul Faculty of Medicine, (7)Universit Paris-Saclay, Hpatinov, Inserm U 1193, (8)Kings College London, (9)Kings College Hospital NHS Trust, (10)The Hospital for Sick Children, Toronto, ON, Canada, (11)Hospices Civils De Lyon, (12)Hacettepe University Faculty of Medicine, (13)Azienda Ospedaliera Papa Giovanni XXIII, (14)Wilhelmina Childrens Hospital, University Medical Centre Utrecht, (15)Hpital Universitaire Necker-Enfants Malades, (16)Leeds Childrens Hospital, (17)CHU, Hospital De La Timone, (18)Children's Hospital Los Angeles, (19)Shaare Zedek Medical Center, (20)Baylor College of Medicine, Texas Childrens Hospital, Houston, TX, (21)Albireo Pharma, Inc., (22)Gazi University Faculty of Medicine Background: Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited liver diseases with clinical features that may include elevated serum bile acids and severe pruritus

The arginine salt acts as a protective carrier and stabilizer