ArticleJournal of pediatric gastroenterology and nutrition2017
Unique Inflammatory Bowel Disease Phenotype of Pediatric Primary Sclerosing Cholangitis: A Single-Center Study.
Article in Journal of pediatric gastroenterology and nutrition, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 36 citations in OpenAlex.
- Association of extraintestinal manifestations and disease severity in paediatric inflammatory bowel disease: a multicentre cohort study.BMJ open gastroenterology · 2026Observational
- Prognosis of pediatric-onset inflammatory bowel disease associated with primary sclerosing cholangitis: A population-based study.Journal of pediatric gastroenterology and nutrition · 2025Article
- Should all pediatric patients with type 1 autoimmune hepatitis be screened for inflammatory bowel disease?JHEP reports : innovation in hepatology · 2025Article
- Outcomes of oral vancomycin therapy in children with atypical ulcerative colitis with or without confirmed primary sclerosing cholangitis: a real-world observational study.BMJ open gastroenterology · 2025Observational
- A Single-Center Study of Long-Term Effectiveness of Vedolizumab in Anti-TNF Refractory Pediatric Inflammatory Bowel Disease.JPGN reports · 2023Article
- Long-Term Follow-Up into Adulthood of Pediatric-Onset Primary Sclerosing Cholangitis and Autoimmune Sclerosing Cholangitis.JPGN reports · 2022Article
- Microbiome Responses to Vancomycin Treatment in a Child With Primary Sclerosing Cholangitis and Ulcerative Colitis.ACG case reports journal · 2021Article
- Progression to colectomy in the era of biologics: A single center experience with pediatric ulcerative colitis.Journal of pediatric surgery · 2020Article
- Gut Microbiome of Children and Adolescents With Primary Sclerosing Cholangitis in Association With Ulcerative Colitis.Frontiers in immunology · 2020Article
- Specific Features of Patients With Inflammatory Bowel Disease and Primary Sclerosing Cholangitis.Journal of clinical medicine research · 2019Review
- Treatment of primary sclerosing cholangitis in children.World journal of hepatology · 2019Review
- The IBD and PSC Phenotypes of PSC-IBD.Current gastroenterology reports · 2018Review
- Primary sclerosing cholangitis and inflammatory bowel disease comorbidity: an update of the evidence.Annals of gastroenterologyReview
Corrections and comments
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Authors and funding
6 authors at 4 institutions in 2 countries.
Funding
Abstract
objectivesIn adults, primary sclerosing cholangitis (PSC), a cholestatic liver disease characterized by inflammation/fibrosis of intra/extrahepatic bile ducts, associates with a milder form of inflammatory bowel disease (IBD), particularly ulcerative colitis (UC). The pediatric PSC-IBD phenotype is less well characterized.
methodsWe performed a retrospective, single-center study examining patients with PSC-IBD at Texas Children's Hospital between 2000 and 2015. IBD-phenotype (Modified Montreal Classification), medications, laboratory values, endoscopic records, and IBD-based hospital admissions were collected. PSC-UC phenotype was compared to UC, non-PSC patients (n = 95) from Texas Children's Hospital. Elevated gamma-glutamyl transpeptidase levels were compared to calprotectin levels and IBD-flare activity, that is, gastrointestinal symptoms resulting in office/emergency department visits or hospital admission.
resultsOf 39 patients with PSC-IBD, 34 (87.2%) had UC (PSC-UC) and 5 (12.8%) had Crohn disease. Pancolitis was more common in PSC-UC than UC, non-PSC (96.3%, 64%, P = 0.0009). Patients with PSC-UC required less treatment with steroids (76.5%, 91.6%, P = 0.0326) or infliximab (8.8%, 37.9%, P = 0.0011), and fewer had at least 1 IBD-related hospital admission (32.4%, 63.2%, P = 0.0025) than UC, non-PSC. Progression to colectomy was significantly less (5.8%, 24.2%, P = 0.0223) in PSC-UC. Median diagnosis-to-colectomy time tended to be longer in PSC-UC (6.37, 2.5 years, P = 0.0792). In 2 smaller subsets, gamma-glutamyl transpeptidase did not correlate with calprotectin in PSC-UC (n = 11, P = 0.7922) and less strongly associated with IBD-flares in PSC-UC than UC, non-PSC (n = 33, n = 67; 15.2%, 41.8%, P = 0.0120).
conclusionsPediatric PSC appears to associate with milder pancolitic-UC. PSC and IBD activity do not appear to correlate. Our findings may provide useful information toward etiology and management of pediatric PSC-IBD.
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