THE “HOMEMADE” REVOLUTIONARY RESEARCH
Lorenza Matarazzo1, Giuseppe Indolfi2, Raffaele Iorio3, Fabiola Di Dato3, Andrea Pietrobattista4, Marco Sciveres5, Mara Cananzi6, Giusy Ranucci5, Gabriella Nebbia7, Federica Nuti8, Pier LuigiCalvo9, Fernanda Cristofori10, Antonio Moschetta10, Matteo Motta1, Angela Marino1, Angelo Di Giorgio1, Lorenzo D’Antiga1, Emanuele Nicastro1
1.Pediatric Hepatology, Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, Bergamo
2.Department Neurofarba, University of Florence and Meyer Children’s University Hospital, Florence
3.Department of Translational Medical Sciences, Section of Pediatrics, University Federico II, Naples
4.Hepatology Gastroenterology and Nutrition, Bambino Gesù Children’s Hospital, Rome
5.Pediatric Departmeny, IRCCS ISMETT-UPMC Research Institute, Palermo
6.Pediatric Hepatology and Gastroenterology, University Hospital of Padova, Padua
7.Health Protection Agency ATS Milan, Milan
8.Intermediate Pediatric Care Unit, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan
9.Pediatric Gastroenterology Unit, Regina Margherita Children’s Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Turin
10. Interdisciplinary Department of Medicine, Pediatric Section, Pediatric Hospital Giovanni XXIII, Bari
In Alagille syndrome (ALGS) chronic cholestasis leads to pruritus and liver fibrosis by bile acid (BA) retention. The inhibitor of the ileal bile acid transporter (IBAT) Maralixibat reduces the serum BA pool improving pruritus. We report the Italian experience of Maralixibat in ALGS.