Impact of paediatric onset primary sclerosing cholangitis on clinical course and outcome of inflammatory bowel disease : a case-control population-based study in Finland

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Tenca , A , Jaakkola , T , Färkkilä , M , Arola , J & Kolho , K-L 2019 , ' Impact of paediatric onset primary sclerosing cholangitis on clinical course and outcome of inflammatory bowel disease : a case-control population-based study in Finland ' , Scandinavian Journal of Gastroenterology , vol. 54 , no. 8 , pp. 984-990 . https://doi.org/10.1080/00365521.2019.1648547

Title: Impact of paediatric onset primary sclerosing cholangitis on clinical course and outcome of inflammatory bowel disease : a case-control population-based study in Finland
Author: Tenca, Andrea; Jaakkola, Tytti; Färkkilä, Martti; Arola, Johanna; Kolho, Kaija-Leena
Contributor: University of Helsinki, HUS Abdominal Center
University of Helsinki, HUS Children and Adolescents
University of Helsinki, Centre of Excellence in Complex Disease Genetics
University of Helsinki, HUSLAB
University of Helsinki, Children's Hospital
Date: 2019-08-03
Language: eng
Number of pages: 7
Belongs to series: Scandinavian Journal of Gastroenterology
ISSN: 0036-5521
URI: http://hdl.handle.net/10138/326222
Abstract: Introduction and aim: The aim of this study was to investigate the outcome of a paediatric onset of inflammatory bowel disease (IBD) in a cohort of subjects with primary sclerosing cholangitis (PSC) and in a matched-age population-based control group without PSC. Methods: We identified 28 IBD-PSC cases (median age at IBD diagnosis 12.5 years, 25-75th: 10-16 years) and selected three IBD controls for each case matched for age and year of IBD diagnosis. All data regarding the gastrointestinal tract and liver were collected at diagnosis and at last follow-up (median 15 years). Results: At diagnosis the prevalence of pancolitis was similar between the groups (78% and 79%, respectively p = -.30), but histologic inflammation was milder in IBD-PSC (61% vs 30%, p = .06). At last follow-up (median age 29 years) pancolitis was less frequent (6% and 33%, respectively p = .04) and the remission higher (76% and 47%, respectively p = .08) in IBD-PSC patients than in IBD patients. Panproctolectomy (32% in IBD-PSC and 34% in IBD, p = 1.0) and the rate of pouchitis (62% and 70%, respectively p = .8) were similar. Conclusions: The outcome of paediatric onset IBD in patients with PSC in adulthood seems to be comparable to those with IBD only.
Subject: Primary sclerosing cholangitis
autoimmune liver disease
dysplasia
colorectal cancer
pouchitis
ulcerative colitis
WIDE ASSOCIATION ANALYSIS
URSODEOXYCHOLIC ACID
ULCERATIVE-COLITIS
AUTOIMMUNE HEPATITIS
NATURAL-HISTORY
PSC-IBD
CHILDREN
RISK
EPIDEMIOLOGY
PHENOTYPE
3121 General medicine, internal medicine and other clinical medicine
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