{"doi":"10.1002/jpn3.70281","title":"Secondary prophylaxis of variceal hemorrhage in biliary atresia—Analysis of prospective multicenter studies","abstract":"Gastroesophageal variceal hemorrhage (VH) can lead to morbidity and occasionally mortality in biliary atresia (BA), including decompensation in hepatic function and accelerated listing for liver transplantation.1-4 We have previously reported that the 5-year incidence of VH in BA is 8%–10%.5 Guidelines, based upon expert opinion for treatment of pediatric portal hypertension (PHT), recommend secondary prophylaxis of esophageal varices following a bleeding episode to prevent further bleeding episodes and decompensation.6 Guidelines for adults recommend secondary prophylaxis by variceal ligation (EVL) and/or propranolol/carvedilol.7 There are few studies that systematically and prospectively evaluated the management and outcome after VH in BA. The Childhood Liver Disease Research Network (ChiLDReN), funded by the National Institute of Diabetes, Digestive and Kidney Diseases (NIDDK)/National Institute of Health, conducts the Prospective Database of Infants with Cholestasis (PROBE; Clinical Trials.gov: NCT00061828) and the Biliary Atresia Study in Infants and Children (BASIC; Clinical Trials.gov: NCT00345553). These are incident and prevalent cohorts, respectively, designed to prospectively acquire clinical data, allowing for analysis of the natural history and interventional approaches after VH in BA. Research was conducted in accordance with both the Declaration of Helsinki and the Istanbul Declaration. All participating ChiLDReN centers had institutional and central review boards and/or research ethics approval. Written informed consent was obtained from parents and/or guardians, and assent was obtained from children aged 7 years or older. Data from PROBE participants enrolled beginning June 2004, with follow-up to July 2023 and BASIC participants enrolled beginning July 2006, with follow-up to March 2023 were analyzed. PROBE participants were dichotomized according to functioning post-hepatoportoenterostomy (HPE) bile drainage as determined by achievement of total bilirubin <2 mg/dL within 3 months of HPE (TB < 2 vs TB > 2).8 The study population consisted of children with BA who met definite or possible clinically evident portal hypertension criteria (dpCEPH)9 and subsequently had gastrointestinal hemorrhage (GIH). The index GIH (GIHi) was defined as the first GIH after a study visit with dpCEPH, presumed to be due to VH. Clinical, laboratory, and endoscopic data were collected at baseline as well as prospectively following GIHi. Additional GIH and/or endoscopic interventions reported within 7 days of GIHi were treated as a single bleeding episode. Risk factors of interest included demographic variables, medical history, and laboratory measurements (collected during routine care). Summary statistics were calculated to describe the study population. Kruskal–Wallis or chi-squared tests were used to compare characteristics between groups. Kaplan–Meier curves were used to estimate the time to transplant/death after GIHi. The log-rank test was used to test the difference in time to transplant/death between those who received banding/sclerotherapy and those who did not. 180 participants with BA and native liver who had GIHi were included (PROBE-96, BASIC-84; Table S1). Thirty-three (38%) PROBE participants had TB < 2. The median age at GIHi was 11 and 77 months in PROBE and BASIC, respectively (Table S1). The median (Q1, Q3) time-to-bleed after the first visit with dpCEPH was 5 months (2, 18) for PROBE and 9 months (2, 34) for BASIC participants. Figure 1A,B demonstrates cumulative incidence curves for each group. Time to GIHi was significantly shorter in TB > 2 PROBE participants at 4 months (2, 15) compared to TB < 2 at 11 months4, 5 (p = 0.01). At GIHi, 110 (61%) participants underwent endoscopy; 96 (87%) had esophageal varices. Fifty had varices that were considered large or did not flatten with insufflation (Table S1), while 45 had varices that were graded as small to medium or flattened with insufflation. At GIHi and in follo","journal":"Journal of Pediatric Gastroenterology and Nutrition","year":2025,"id":582695,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9463,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":868900,"name":"Kieran Hawthorne","orcid":"0009-0008-8099-9366","position":1,"is_corresponding":false},{"id":96836,"name":"John C. Magee","orcid":"0000-0001-8416-7905","position":2,"is_corresponding":false},{"id":418725,"name":"Benjamin L. Shneider","orcid":"0000-0002-7208-0949","position":3,"is_corresponding":false},{"id":333864,"name":"Lee M. Bass","orcid":"0000-0003-1253-714X","position":0,"is_corresponding":true}],"reference_count":11,"raw_metadata":null,"created_at":"2026-07-19T02:58:59.653747Z","pmid":"41267542","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}