Carolina Jiménez-Rivera1, Simon C Ling2, Najma Ahmed3, Jason Yap4, Mary Aglipay5, Nick Barrowman5, Samantha Graitson5, Jeff Critch6, Mohsin Rashid7, Vicky L Ng2, Eve A Roberts2, Herbert Brill8, Jenna K Dowhaniuk8, Garth Bruce9, Kevin Bax10, Mark Deneau11, Orlee R Guttman12, Richard A Schreiber12, Steven Martin13, Fernando Alvarez14. 1. University of Ottawa and Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada; cajimenez@cheo.on.ca. 2. University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada; 3. McGill University and Montreal Children's Hospital, Montreal, Quebec, Canada; 4. University of Alberta and Stollery Children's Hospital, Edmonton, Alberta, Canada; 5. University of Ottawa and Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada; 6. Memorial University of Newfoundland and Janeway Children's Hospital, St. John's, Newfoundland, Canada; 7. Dalhousie University and IWK Health Center, Halifax, Nova Scotia, Canada; 8. McMaster University and McMaster Children's Hospital, Hamilton, Ontario, Canada; 9. University of Saskatchewan and Children's Hospital of Saskatchewan, Saskatoon, Saskatchewan, Canada; 10. University of Western Ontario and London Health Science Center, London, Ontario, Canada; 11. University of Manitoba and The Children's Hospital of Winnipeg, Winnipeg, Manitoba, Canada; 12. University of British Columbia and BC Children's Hospital, Vancouver, British Columbia, Canada; 13. University of Calgary and Alberta Children's Hospital Calgary, Alberta, Canada; and. 14. University of Montreal and Centre Hospitalier Universitaire Sainte-Justine, Montreal, Quebec, Canada.
Abstract
BACKGROUND AND OBJECTIVES: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease of unknown etiology, with limited population-based estimates of pediatric incidence. We reported the incidence of pediatric AIH in Canada and described its clinical characteristics. METHODS: We conducted a retrospective cohort study of patients aged <18 years diagnosed with AIH between 2000-2009 at all pediatric centers in Canada. RESULTS: A total of 159 children with AIH (60.3% female, 13.2% type 2 AIH) were identified. Annual incidence was 0.23 per 100000 children. Median age at presentation for type 1 was 12 years (interquartile range: 11-14) versus 10 years for type 2 (interquartile range: 4.5-13) (P = .03). Fatigue (58%), jaundice (54%), and abdominal pain (49%) were the most common presenting symptoms. Serum albumin (33 vs 38 g/L; P = .03) and platelet count (187 000 vs 249 000; P <.001) were significantly lower and the international normalized ratio (1.4 vs 1.2; P <.001) was higher in cirrhotic versus noncirrhotic patients. Initial treatment included corticosteroids (80%), azathioprine (32%), and/or cyclosporine (13%). Response to treatment at 1 year was complete in 90%, and partial in 3%. 3% of patients had no response, and 3% responded and later relapsed. Nine patients underwent liver transplantation, and 4 patients died at a mean follow-up of 4 years. CONCLUSIONS: AIH is uncommon in children and adolescents in Canada. Type 1 AIH was diagnosed 5.5 times more frequently than type 2 AIH. Most patients respond well to conventional therapy, diminishing the need for liver transplantation.
BACKGROUND AND OBJECTIVES:Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease of unknown etiology, with limited population-based estimates of pediatric incidence. We reported the incidence of pediatric AIH in Canada and described its clinical characteristics. METHODS: We conducted a retrospective cohort study of patients aged <18 years diagnosed with AIH between 2000-2009 at all pediatric centers in Canada. RESULTS: A total of 159 children with AIH (60.3% female, 13.2% type 2 AIH) were identified. Annual incidence was 0.23 per 100000 children. Median age at presentation for type 1 was 12 years (interquartile range: 11-14) versus 10 years for type 2 (interquartile range: 4.5-13) (P = .03). Fatigue (58%), jaundice (54%), and abdominal pain (49%) were the most common presenting symptoms. Serum albumin (33 vs 38 g/L; P = .03) and platelet count (187 000 vs 249 000; P <.001) were significantly lower and the international normalized ratio (1.4 vs 1.2; P <.001) was higher in cirrhotic versus noncirrhoticpatients. Initial treatment included corticosteroids (80%), azathioprine (32%), and/or cyclosporine (13%). Response to treatment at 1 year was complete in 90%, and partial in 3%. 3% of patients had no response, and 3% responded and later relapsed. Nine patients underwent liver transplantation, and 4 patients died at a mean follow-up of 4 years. CONCLUSIONS: AIH is uncommon in children and adolescents in Canada. Type 1 AIH was diagnosed 5.5 times more frequently than type 2 AIH. Most patients respond well to conventional therapy, diminishing the need for liver transplantation.
Authors: Melissa A Sheiko; Shikha S Sundaram; Kelley E Capocelli; Zhaoxing Pan; Annette M McCoy; Cara L Mack Journal: J Pediatr Gastroenterol Nutr Date: 2017-07 Impact factor: 2.839
Authors: Aldo J Montano-Loza; Jessica R Allegretti; Angela Cheung; Maryam Ebadi; David Jones; Nanda Kerkar; Cynthia Levy; Sumera Rizvi; John M Vierling; Fernando Alvarez; Wayne Bai; Susan Gilmour; Aliya Gulamhusein; Orlee Guttman; Bettina E Hansen; Sonya MacParland; Andrew Mason; Fernanda Onofrio; Pere Santamaria; Ashley Stueck; Mark Swain; Catherine Vincent; Amanda Ricciuto; Gideon Hirschfield Journal: Can Liver J Date: 2021-11-11
Authors: Angelo Di Giorgio; A D'Adda; A Marseglia; A Sonzogni; L Licini; E Nicastro; L D'Antiga Journal: Hepatol Int Date: 2019-05-08 Impact factor: 6.047