{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T18:13:11Z","timestamp":1790100791594,"version":"4.0.1"},"reference-count":78,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,12,31]],"date-time":"2024-12-31T00:00:00Z","timestamp":1735603200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>We aimed to provide an evidence\u2010supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The core group formulated seven PICO\u2010structured 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Regular screening for gamma\u2010glutamyltransferase (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\u2010term studies. Children with PSC\u2013IBD combined with convincing features of autoimmune hepatitis may benefit from corticosteroids and antimetabolites.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We present state\u2010of\u2010the\u2010art guidance on the diagnostic criteria, follow\u2010up strategies, and therapeutic strategies and point out research gaps in children and adolescents with PSC\u2013IBD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.12378","type":"journal-article","created":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:17Z","timestamp":1735689617000},"page":"374-393","source":"Crossref","is-referenced-by-count":14,"title":["Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group"],"prefix":"10.1002","volume":"80","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3867-2665","authenticated-orcid":false,"given":"Patrick F.","family":"van Rheenen","sequence":"first","affiliation":[{"name":"Department of Paediatric Gastroenterology, Hepatology, and Nutrition University Medical Center Groningen, University of Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaija\u2010Leena","family":"Kolho","sequence":"additional","affiliation":[{"name":"Children's Hospital, University of Helsinki and HUS Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard K.","family":"Russell","sequence":"additional","affiliation":[{"name":"Department of Paediatric Gastroenterology, and Nutrition Royal Hospital for Children and Young People Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Aloi","sequence":"additional","affiliation":[{"name":"Sapienza University of Rome \u2010 Umberto I Hospital Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Deganello","sequence":"additional","affiliation":[{"name":"Department of Radiology King's College Hospital, School of Biomedical Engineering and Imaging Sciences, King's College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9amus","family":"Hussey","sequence":"additional","affiliation":[{"name":"Children's Health Ireland and University College Dublin Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norman","family":"Junge","sequence":"additional","affiliation":[{"name":"Division for Pediatric Gastroenterology and Hepatology, Department of Pediatric Kidney, Liver, and Metabolic Diseases Hannover Medical School Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"De Laffolie","sequence":"additional","affiliation":[{"name":"General Paediatrics and Neonatology, Gastroenterology Justus Liebig University Giessen Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark R.","family":"Deneau","sequence":"additional","affiliation":[{"name":"University of Utah and Intermountain Healthcare Primary Children's Hospital Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emer","family":"Fitzpatrick","sequence":"additional","affiliation":[{"name":"Children's Health Ireland and University College Dublin Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne M.","family":"Griffiths","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, IBD Centre, SickKids Hospital University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iva","family":"Hojsak","sequence":"additional","affiliation":[{"name":"Children's Hospital Zagreb University of Zagreb Medical School Zagreb Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Nicastro","sequence":"additional","affiliation":[{"name":"Pediatric Hepatology Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreia","family":"Nita","sequence":"additional","affiliation":[{"name":"Department of Paediatric Gastroenterology Great Ormond Street Hospital London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikko","family":"Pakarinen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery The New Children's Hospital University of Helsinki and Helsinki University Hospital Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Ricciuto","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, IBD Centre, SickKids Hospital University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lissy","family":"de Ridder","sequence":"additional","affiliation":[{"name":"Department of Paediatric Gastroenterology Erasmus University Medical Center Sophia Children's Hospital Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurelio","family":"Sonzogni","sequence":"additional","affiliation":[{"name":"Department of Pathology ASST Bergamo Est Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Tenca","sequence":"additional","affiliation":[{"name":"Helsinki University and Helsinki University Hospital HUS, Abdominal Center Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"Samyn","sequence":"additional","affiliation":[{"name":"Paediatric Liver, GI and Nutrition Service King's College Hospital London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Indolfi","sequence":"additional","affiliation":[{"name":"Meyer Children's Hospital IRCCS Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,12,31]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000672"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm10225359"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.07.065"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.14159"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.29204"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2018-317993"},{"issue":"2","key":"e_1_2_11_8_1","first-page":"1","article-title":"AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma","volume":"77","author":"Bowlus CL","year":"2022","journal-title":"Hepatology"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2022.05.011"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000409"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ibd.22864"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/MIB.0000000000000558"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2021.07.046"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000001045"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/ecco-jcc\/jjad108"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2021.07.047"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/MCG.0000000000001084"},{"key":"e_1_2_11_18_1","volume-title":"Clinical and Research Information on Drug\u2010Induced Liver Injury","author":"LiverTox","year":"2012"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1911063"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41390-019-0702-3"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.11.028"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001801"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2004.08.002"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep4.1454"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2008.10.019"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.crohns.2013.01.016"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.3109\/15513815.2014.898721"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-021-08333-7"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2559.2009.03391.x"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11894-018-0620-2"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v21.i6.1956"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0042-109264"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2019-2235"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2019.08.048"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2015.08.041"},{"key":"e_1_2_11_36_1","doi-asserted-by":"publisher","DOI":"10.1136\/flgastro-2021-102053"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2018.01.020"},{"key":"e_1_2_11_38_1","doi-asserted-by":"publisher","DOI":"10.1093\/ecco-jcc\/jjy113"},{"key":"e_1_2_11_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2023.02.030"},{"key":"e_1_2_11_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep4.1251"},{"key":"e_1_2_11_41_1","doi-asserted-by":"publisher","DOI":"10.1002\/hsr2.1086"},{"key":"e_1_2_11_42_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.31393"},{"key":"e_1_2_11_43_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.j3951"},{"key":"e_1_2_11_44_1","doi-asserted-by":"publisher","DOI":"10.1097\/PG9.0000000000000341"},{"key":"e_1_2_11_45_1","doi-asserted-by":"publisher","DOI":"10.1093\/ecco-jcc\/jjac187"},{"key":"e_1_2_11_46_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2021.05.041"},{"key":"e_1_2_11_47_1","doi-asserted-by":"publisher","DOI":"10.1055\/a-1671-6336"},{"key":"e_1_2_11_48_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26565"},{"key":"e_1_2_11_49_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2017.02.038"},{"key":"e_1_2_11_50_1","doi-asserted-by":"publisher","DOI":"10.3389\/ti.2023.11729"},{"key":"e_1_2_11_51_1","doi-asserted-by":"publisher","DOI":"10.1111\/tri.13814"},{"key":"e_1_2_11_52_1","doi-asserted-by":"publisher","DOI":"10.1111\/tri.13097"},{"key":"e_1_2_11_53_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.31911"},{"key":"e_1_2_11_54_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2022.100599"},{"key":"e_1_2_11_55_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v24.i28.3171"},{"key":"e_1_2_11_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.01.039"},{"key":"e_1_2_11_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coph.2021.11.003"},{"key":"e_1_2_11_58_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.23082"},{"key":"e_1_2_11_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2018.08.006"},{"key":"e_1_2_11_60_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep4.1421"},{"key":"e_1_2_11_61_1","doi-asserted-by":"publisher","DOI":"10.4254\/wjh.v11.i1.19"},{"key":"e_1_2_11_62_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2024.01.049"},{"key":"e_1_2_11_63_1","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2018-316599"},{"key":"e_1_2_11_64_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.17936"},{"key":"e_1_2_11_65_1","doi-asserted-by":"publisher","DOI":"10.1080\/00365521.2020.1787501"},{"key":"e_1_2_11_66_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e31816fee95"},{"key":"e_1_2_11_67_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.31560"},{"key":"e_1_2_11_68_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-230X-14-51"},{"key":"e_1_2_11_69_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000003513"},{"key":"e_1_2_11_70_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2023.11.032"},{"key":"e_1_2_11_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2020.02.014"},{"key":"e_1_2_11_72_1","doi-asserted-by":"publisher","DOI":"10.1097\/MCG.0b013e3181662426"},{"key":"e_1_2_11_73_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002855"},{"key":"e_1_2_11_74_1","doi-asserted-by":"publisher","DOI":"10.1093\/ecco-jcc\/jjaa161"},{"key":"e_1_2_11_75_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002035"},{"key":"e_1_2_11_76_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2018.05.034"},{"key":"e_1_2_11_77_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dld.2023.05.024"},{"key":"e_1_2_11_78_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.14393"},{"key":"e_1_2_11_79_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2021.08.039"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpn3.12378","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T00:47:48Z","timestamp":1748566068000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpn3.12378"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,31]]},"references-count":78,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2025,2]]}},"alternative-id":["10.1002\/jpn3.12378"],"URL":"https:\/\/doi.org\/10.1002\/jpn3.12378","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,12,31]]}}}