Evidence map›Paper›PMID 39741383›Full record

GuidelineJournal of pediatric gastroenterology and nutrition2025

Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.

Patrick F van Rheenen, Kaija-Leena Kolho, Richard K Russell, Marina Aloi, Annamaria Deganello, Séamus Hussey, Norman Junge, Jan De Laffolie, Mark R Deneau, Emer Fitzpatrick and 11 more

Abstract readPractice Guideline
PubMed Full text at PMC Publisher
In one paragraph

Guideline 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 8 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
8citing papers in PubMed, 2 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

8 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Guideline
  2. Guideline
  3. Observational
  4. Review
  5. Article
  6. Review
  7. Article
  8. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

21 authors.

Patrick F van RheenenDepartment of Paediatric Gastroenterology, Hepatology, and Nutrition, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.ORCID 0000-0003-3867-2665
Kaija-Leena KolhoChildren's Hospital, University of Helsinki and HUS, Helsinki, Finland.
Richard K RussellDepartment of Paediatric Gastroenterology, and Nutrition, Royal Hospital for Children and Young People, Edinburgh, UK.
Marina AloiSapienza University of Rome - Umberto I Hospital, Rome, Italy.
Annamaria DeganelloDepartment of Radiology, King's College Hospital, School of Biomedical Engineering and Imaging Sciences, King's College London, London, UK.
Séamus HusseyChildren's Health Ireland and University College Dublin, Dublin, Ireland.
Norman JungeDivision for Pediatric Gastroenterology and Hepatology, Department of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School, Hannover, Germany.
Jan De LaffolieGeneral Paediatrics and Neonatology, Gastroenterology, Justus Liebig University Giessen, Giessen, Germany.
Mark R DeneauUniversity of Utah and Intermountain Healthcare Primary Children's Hospital, Salt Lake City, Utah, USA.
Emer FitzpatrickChildren's Health Ireland and University College Dublin, Dublin, Ireland.
Anne M GriffithsFaculty of Medicine, IBD Centre, SickKids Hospital, University of Toronto, Toronto, Ontario, Canada.
Iva HojsakChildren's Hospital Zagreb, University of Zagreb Medical School, Zagreb, Croatia.
Emanuele NicastroPediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy.
Andreia NitaDepartment of Paediatric Gastroenterology, Great Ormond Street Hospital, London, UK.
Mikko PakarinenDepartment of Pediatric Surgery, The New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Amanda RicciutoFaculty of Medicine, IBD Centre, SickKids Hospital, University of Toronto, Toronto, Ontario, Canada.
Lissy de RidderDepartment of Paediatric Gastroenterology, Erasmus University Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.
Aurelio SonzogniDepartment of Pathology, ASST Bergamo Est, Bergamo, Italy.
Andrea TencaHelsinki University and Helsinki University Hospital HUS, Abdominal Center, Helsinki, Finland.
Marianne SamynPaediatric Liver, GI and Nutrition Service, King's College Hospital, London, UK.
Giuseppe IndolfiMeyer Children's Hospital IRCCS, Florence, Italy.

Funding

European Society for Paediatric Gastroenterology Hepatology and Nutrition
6 · The paper itself

Abstract

objectiveWe aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).

methodsThe core group formulated seven PICO-structured clinical questions. A systematic literature search from inception to December 2022 was conducted by a medical librarian using MEDLINE and EMBASE. Core messages from the literature were phrased as position statements and then circulated to a sounding board composed of international experts in pediatric gastroenterology and hepatology, histopathology, adult gastroenterology and hepatology, radiology, and surgery. Statements reaching at least 80% agreement were considered as final. The other statements were refined and then subjected to a second online vote or rejection.

resultsRegular screening for gamma-glutamyltransferase (GGT) is essential for detecting possible biliary disease in children with IBD. MR cholangiopancreatography is the radiological modality of choice for establishing the diagnosis of PSC. Liver biopsy is relevant in the evaluation of small duct PSC or autoimmune hepatitis. Children who do not have known IBD at the time of PSC diagnosis should undergo initial screening with fecal calprotectin for asymptomatic colitis, and then at least once yearly thereafter. Children with a cholestatic liver enzyme profile can be considered for treatment with ursodeoxycholic acid and can continue if there is a meaningful reduction or normalization in GGT. Oral vancomycin may have a beneficial effect on GGT and intestinal inflammation, but judicious use is recommended due to the lack of long-term studies. Children with PSC-IBD combined with convincing features of autoimmune hepatitis may benefit from corticosteroids and antimetabolites.

conclusionsWe present state-of-the-art guidance on the diagnostic criteria, follow-up strategies, and therapeutic strategies and point out research gaps in children and adolescents with PSC-IBD.

Indexed as

Cholangitis, SclerosingInflammatory Bowel DiseasesAdolescentChildCholangiopancreatography, Magnetic ResonanceGastroenterologyHumanscalprotectincolorectal carcinomaMRCPsurveillance colonoscopyursodeoxycholic acid

Identifiers

PMID39741383
PMCPMC11788976

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.