ArticlePediatric surgery international2025
Portal hypertension in biliary atresia: a Japanese biliary atresia registry study.
Article in Pediatric surgery international, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers, 1 of them a synthesis that pooled it.
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Who cites it
3 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Hypermanganesemia in Children With Liver and Portosystemic Circulation Disorders: A Systematic Review.Journal of paediatrics and child health · 2026Pooled it
- Association between post-Kasai cholangitis episodes, ursodeoxycholic acid regimen, and portal hypertension risk in biliary atresia: a multicenter cross-sectional study.Pediatric surgery international · 2026Article
- Comment on "Portal hypertension in biliary atresia: a Japanese Biliary Atresia Registry study".Pediatric surgery international · 2025Article
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Authors and funding
11 authors.
Funding
Abstract
purposeThis study aimed to clarify the incidence of portal hypertension (PH) in biliary atresia (BA), its impact on long-term native liver survival rates (NLSRs), and the role of PH treatment.
methodsData were analyzed from 3,777 patients in the Japanese Biliary Atresia Registry (1989-2021). Incidence and treatment of PH were examined in all patients and in 20-year native liver survivors. A subgroup of 596 jaundice-free native liver survivors was divided into three groups: without PH (A), untreated PH (B), and treated PH (C). Patient characteristics, clinical outcomes, and survival analyses were performed.
resultsPH occurred in 46.4% of all patients, with gastroesophageal varices (35.1%) and hypersplenism (36.8%) being most common. Among 759 20-year native liver survivors, 49.8% had PH, largely diagnosed between 2 and 15 years. Group C patients were older at Kasai portoenterostomy and had more cholangitis. Survival analysis showed best outcomes in group A and worst in group C. Conditional native liver survival analysis revealed group B initially had better NLSRs than group C, but differences diminished beyond 15 years.
conclusionIn native liver survivors without jaundice following KP, PH influenced prognosis, but successful treatment enabled long-term survival comparable to patients with mild or no PH.
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