P0281 Comparison of disease characteristics and disease course between paediatric and adult onset Primary Sclerosing Cholangitis and Inflammatory Bowel Disease
Notice bibliographique
Résumé
Abstract Background Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease that frequently coexists with inflammatory bowel disease (IBD). Data comparing the paediatric- and adult-onset PSC-IBD are lacking. We compared disease characteristics and the clinical course of PSC-IBD between Canadian children and adults. Methods This was a multi-center retrospective cohort study including eight paediatric centers from different provinces across Canada including (Ontario, Alberta, Nova Scotia, Manitoba) and four adult centers from the province of Ontario, including patients diagnosed with PSC-IBD from 1985-2023. Patient and disease characteristics and outcome data were extracted from the clinical charts. We compared patient demographics, disease course, laboratory and imaging results, and complications including liver transplant among paediatric- and adult-onset PSC-IBD. Continuous data were reported as median (Q1-Q3) and compared with the Wilcoxon Rank Sum test, and categorical data were reported as number (%) and compared with chi-square or Fisher exact tests. We compared the time to transplant with the log-rank test. Results We included 521 patients (228 children, median follow-up 3.3 (Q1-Q3 1.5-5.8) years; 293 adults, median follow-up 9.8 (Q1-Q3 4.3-16.0) years (Table-1). The interval between PSC and IBD diagnosis was longer in adults. Small duct disease and PSC with autoimmune hepatitis (AIH) features were more common in children, with corresponding higher ALT and more frequently positive autoantibodies. Rates of portal hypertension at PSC diagnosis were similar in both groups. Ulcerative colitis predominated in both groups with more IBD-unclassified in children. Colitis was most severe in the right colon in a third of children and adults. Children were more often treated with ursodeoxycholic acid, vancomycin, corticosteroids and immunomodulators. Adult patients experienced a higher proportion of adverse liver events over the entire follow-up duration, but time to event analysis showed similar rates of progression to liver transplant (log-rank p=0.075; 2- and 5-year survival with native liver in paediatric vs. adult: 97% and 90% vs. 94% and 84%). (Figure-1) Conclusion In this large Canadian multi-center cohort of PSC-IBD, paediatric-onset disease was characterized by more frequent small duct disease and features of AIH. The interval between IBD and PSC diagnosis was far longer in adults. Although a greater proportion of adult-onset patients experienced adverse liver outcomes overall, rates of transplant at 2 and 5 years were similar between groups, suggesting that PSC progression may in fact be similar.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,001 | 0,002 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,001 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,003 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».