title: Autoimmune liver diseases and inflammatory bowel diseases in children: current issues and future perspectives.
abstract: Inflammatory bowel diseases (IBDs) represent a group of intestinal disorders with a chronic and relapsing inflammation of the gut, and with a potential risk of systemic involvement of other organs and systems. Over the pediatric age, an incidence higher than 20% of developing extraintestinal manifestation during follow-up has been reported. The liver and the biliary system are frequently involved, and primary sclerosing cholangitis (PSC) is the most predominant entity with an incidence rate of 6.4-7.8% in children. PSC recognizes a multifactorial pathogenesis, and so far a not fully known mechanism for this association. The peculiar phenotype and the distinct clinical course of patients with IBD and PSC-associated make this 'linkage' an attractive study model to better understand mechanisms underlying these diseases. Approaching to these patients is complex and multidisciplinary, and a unique therapeutic strategy has not been standardized yet. New medications are being studied; however, further studies are needed to fully understand the pathogenesis and to improve the care of these patients. The aim of this paper is to review the recent literature regarding hepatobiliary involvement in IBD patients, with particular attention to PSC, and to provide the latest information for a correct diagnosis and appropriate management.
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Cardile
authors.affiliation: b Department of Pediatrics , University of Messina , Messina , Italy.
authors.forename: Tommaso
authors.initials: T
authors.lastname: Alterio
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Manila
authors.initials: M
authors.lastname: Candusso
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Pietrobattista
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Liccardo
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Maria Sole
authors.initials: MS
authors.lastname: Basso
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Bronislava
authors.initials: B
authors.lastname: Papadatou
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Fiammetta
authors.initials: F
authors.lastname: Bracci
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Knafelz
authors.affiliation: a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Giuliano
authors.initials: G
authors.lastname: Torre
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications* / Q000628:therapy; D002648:Child; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000628:therapy; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000628:therapy; D008099:Liver / Q000473:pathology*; D016031:Liver Transplantation; D010372:Pediatrics
keywords: Autoimmune liver diseases; extra-gastrointestinal manifestations; inflammatory bowel diseases; pediatrics; primary sclerosing cholangitis