ArticleJournal of pediatric gastroenterology and nutrition2025
Intestinal histopathology in pediatric PSC-IBD: Characterization of phenotype and assessment of the Nancy Index.
Article in Journal of pediatric gastroenterology and nutrition, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
What it found
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Who cites it
3 citing papers in PubMed.
- Distinct phenotype of primary sclerosing cholangitis-associated inflammatory bowel disease.Journal of gastroenterology · 2026Review
- Special Considerations in Pediatric Inflammatory Bowel Disease Pathology.Diagnostics (Basel, Switzerland) · 2025Review
- Intestinal histopathology in pediatric PSC-IBD: Characterization of phenotype and assessment of the Nancy Index.Journal of pediatric gastroenterology and nutrition · 2025Article
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Authors and funding
6 authors.
Funding
Abstract
objectivesWe aimed to characterize the histologic gut phenotype of pediatric primary sclerosing cholangitis (PSC)-associated inflammatory bowel disease (IBD) against non-PSC colitis, and to assess Nancy Index (NI) performance in pediatric PSC-IBD.
methodsSingle-center retrospective cohort study including children diagnosed with PSC-IBD or non-PSC colitis (ulcerative colitis [UC] or IBD-unclassified) from 2000 to 2018, with diagnostic intestinal biopsies. Biopsies were re-reviewed by two independent pathologists who assessed microscopic disease distribution, NI scores, and specific histological features in the right and left colons, overall and stratified by endoscopic severity (moderate-severe vs. no more than mild). We examined NI inter-rater reliability with Fleiss' weighted (quadratic) kappa and NI construct validity against global endoscopic severity (Spearman correlation) and clinical outcomes (logistic regression).
resultsFifty children with PSC-IBD and 81 colitis controls were included. Histologically, pancolitis (84% vs. 55%), right colon-predominant colitis (48% vs. 3%), and backwash ileitis (53% vs. 12%) (all p < 0.01) were significantly more common in PSC-IBD; histologic rectal sparing occurred at similar rates (6% vs. 10%, p = 0.54). Lamina propria-predominant neutrophils, prominent eosinophilic infiltration (left colon), and surface villiform change (right colon) were more common in PSC-IBD than colitis controls (p < 0.01). NI showed excellent inter-rater reliability (kappa > 0.9) and correlated moderately with global endoscopic severity but poorly with clinical activity in PSC-IBD.
conclusionsPediatric PSC-IBD has a distinct histologic phenotype that largely mirrors the endoscopic phenotype in distribution and includes a greater frequency of features not included in conventional UC histologic activity indices. Future work should investigate whether a PSC-IBD-specific index incorporating these features is warranted.
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