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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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>PH 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00383-025-06222-4","type":"journal-article","created":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T07:57:56Z","timestamp":1761897476000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Portal hypertension in biliary atresia: a Japanese biliary atresia registry study"],"prefix":"10.1007","volume":"41","author":[{"given":"Ryuji","family":"Okubo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Nio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideyuki","family":"Sasaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taichi","family":"Fukuzawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hironori","family":"Kudo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Ando","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuyoshi","family":"Sakurai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masatoshi","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisuke","family":"Tada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Motoshi","family":"Wada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Japanese Biliary Atresia Society","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,10,31]]},"reference":[{"key":"6222_CR1","doi-asserted-by":"publisher","first-page":"665","DOI":"10.1016\/0022-3468(68)90897-X","volume":"3","author":"M Kasai","year":"1968","unstructured":"Kasai M, Kimura S, Asakura Y et al (1968) Surgical treatment of biliary Atresia. 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