The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan

Background: Primary sclerosing cholangitis (PSC) is often associated with ulcerative colitis (UC). We investigated the clinical characteristics of pediatric UC patients with and without PSC. Methods: We retrospectively recruited children with UC, with and without PSC, from 2006 to 2017 in a tertiary...

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Main Authors: Mu-Ming Chien, Mei-Hwei Chang, Kai-Chi Chang, Yu-Chun Chiu, Huey-Ling Chen, Hong-Yuan Hsu, Jia-Feng Wu
Format: Article
Language:English
Published: Elsevier 2021-09-01
Series:Pediatrics and Neonatology
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Online Access:http://www.sciencedirect.com/science/article/pii/S1875957221000802
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author Mu-Ming Chien
Mei-Hwei Chang
Kai-Chi Chang
Yu-Chun Chiu
Huey-Ling Chen
Hong-Yuan Hsu
Jia-Feng Wu
author_facet Mu-Ming Chien
Mei-Hwei Chang
Kai-Chi Chang
Yu-Chun Chiu
Huey-Ling Chen
Hong-Yuan Hsu
Jia-Feng Wu
author_sort Mu-Ming Chien
collection DOAJ
description Background: Primary sclerosing cholangitis (PSC) is often associated with ulcerative colitis (UC). We investigated the clinical characteristics of pediatric UC patients with and without PSC. Methods: We retrospectively recruited children with UC, with and without PSC, from 2006 to 2017 in a tertiary center in Taiwan. The clinical data of the patients, including clinical and endoscopic UC severity scores, medications, and laboratory parameters, were analyzed. Results: We recruited five children with PSC-UC (PSC-UC group), and 26 with UC alone (non-PSC UC group) in this retrospective analysis. Among the patients with PSC-UC, four (80%) were compatible with definite or probable autoimmune sclerosing cholangitis (ASC). The UC Endoscopic Index of Severity (5.00 vs. 9.00, P = 0.003) and Mayo score (4.00 vs. 8.00, P = 0.014) were significantly lower in the PSC-UC group than the non-PSC UC group. The prevalence of immunomodulator use was significantly higher in the PSC-UC than the non-PSC UC group (100% vs. 42.3%, P = 0.043), but there was no difference regarding steroids, mesalamine, or biologics. At the end of the study, significantly fewer patients were steroid-free in the PSC-UC than the non-PSC UC group (20.0% vs. 84.6%, P = 0.010). Conclusions: Pediatric patients with PSC-UC had less severe colitis than those with UC alone in terms of the clinical activity index and endoscopic severity index, but they were more likely to need an immunomodulator and less likely to be steroid-free in the long term, for the control of liver disease.
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spelling doaj-art-fac5f3edda044bc1b605676e2a1d57bc2025-08-20T02:28:10ZengElsevierPediatrics and Neonatology1875-95722021-09-0162548349010.1016/j.pedneo.2021.05.001The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in TaiwanMu-Ming Chien0Mei-Hwei Chang1Kai-Chi Chang2Yu-Chun Chiu3Huey-Ling Chen4Hong-Yuan Hsu5Jia-Feng Wu6Department of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Department of Pediatrics, Taipei Medical University Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Hepatitis Research Center, National Taiwan University Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Department of Medical Education, National Taiwan University Hospital, National Taiwan University, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Hepatitis Research Center, National Taiwan University Hospital, Taipei, Taiwan; Department of Medical Education and Bioethics, National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Department of Medical Education and Bioethics, National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Corresponding author. Department of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, Taiwan.Background: Primary sclerosing cholangitis (PSC) is often associated with ulcerative colitis (UC). We investigated the clinical characteristics of pediatric UC patients with and without PSC. Methods: We retrospectively recruited children with UC, with and without PSC, from 2006 to 2017 in a tertiary center in Taiwan. The clinical data of the patients, including clinical and endoscopic UC severity scores, medications, and laboratory parameters, were analyzed. Results: We recruited five children with PSC-UC (PSC-UC group), and 26 with UC alone (non-PSC UC group) in this retrospective analysis. Among the patients with PSC-UC, four (80%) were compatible with definite or probable autoimmune sclerosing cholangitis (ASC). The UC Endoscopic Index of Severity (5.00 vs. 9.00, P = 0.003) and Mayo score (4.00 vs. 8.00, P = 0.014) were significantly lower in the PSC-UC group than the non-PSC UC group. The prevalence of immunomodulator use was significantly higher in the PSC-UC than the non-PSC UC group (100% vs. 42.3%, P = 0.043), but there was no difference regarding steroids, mesalamine, or biologics. At the end of the study, significantly fewer patients were steroid-free in the PSC-UC than the non-PSC UC group (20.0% vs. 84.6%, P = 0.010). Conclusions: Pediatric patients with PSC-UC had less severe colitis than those with UC alone in terms of the clinical activity index and endoscopic severity index, but they were more likely to need an immunomodulator and less likely to be steroid-free in the long term, for the control of liver disease.http://www.sciencedirect.com/science/article/pii/S1875957221000802Autoimmune liver diseasePediatricPrimary sclerosingcholangitisUlcerative colitis
spellingShingle Mu-Ming Chien
Mei-Hwei Chang
Kai-Chi Chang
Yu-Chun Chiu
Huey-Ling Chen
Hong-Yuan Hsu
Jia-Feng Wu
The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan
Pediatrics and Neonatology
Autoimmune liver disease
Pediatric
Primary sclerosingcholangitis
Ulcerative colitis
title The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan
title_full The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan
title_fullStr The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan
title_full_unstemmed The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan
title_short The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in Taiwan
title_sort characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis a single center study in taiwan
topic Autoimmune liver disease
Pediatric
Primary sclerosingcholangitis
Ulcerative colitis
url http://www.sciencedirect.com/science/article/pii/S1875957221000802
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