{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T16:06:47Z","timestamp":1789834007107,"version":"4.0.1"},"reference-count":28,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2022,10,13]],"date-time":"2022-10-13T00:00:00Z","timestamp":1665619200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JGH Open"],"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Aim<\/jats:title><jats:p>Portopulmonary hypertension (PoPH) is a complication associated with portal hypertension. Since the epidemiological study in Japan was limited, this study aimed to investigate the characteristics, treatment patterns, and prognosis of PoPH patients in real\u2010world data.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The characteristics and treatment patterns of PoPH (<jats:italic>n<\/jats:italic>\u00a0=\u00a0386) and portal hypertension without pulmonary arterial hypertension (portal hypertension w\/o PAH) (<jats:italic>n<\/jats:italic>\u00a0=\u00a096\u2009463) were analyzed based on the Medical Data Vision (MDV) dataset from April 2008 to September 2020. Survival\u2010time analyses of emergency hospitalization and mortality were also conducted between matched pair cohorts of PoPH (<jats:italic>n<\/jats:italic>\u00a0=\u00a0210) and portal hypertension w\/o PAH (<jats:italic>n<\/jats:italic>\u00a0=\u00a0840).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 386 PoPH patients, the Child\u2013Pugh classification of PoPH group comprised patients with Class A (59 [15.3%]), B (109 [28.2%]), and C (42 [10.9%]), and missing (176 [45.6%]). Regarding the feature of PoPH group, the proportion of primary biliary cholangitis (PBC) (13.7%) and splenomegaly (9.8%) was higher compared with portal hypertension w\/o PAH group. The survival time of all\u2010cause hospitalization in PoPH group was shorter than portal hypertension w\/o PAH group in matched pair cohort (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001 by log\u2010rank test). Of PoPH patients, the frequency of PAH\u2010specific medicine usage within 90\u2009days was monotherapy of 79 patients (20.5%), combination therapy of 64 patients (16.6%), and PAH\u2010specific medicine usage of 243 patients (63.0%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This was the first study demonstrating that high proportion of PBC and splenomegaly and a greater risk of hospitalization were observed in PoPH patients based on the analysis using administrative claim database.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jgh3.12820","type":"journal-article","created":{"date-parts":[[2022,10,14]],"date-time":"2022-10-14T05:44:37Z","timestamp":1665726277000},"page":"763-773","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Treatment pattern and clinical outcomes in portopulmonary hypertension: A database study in Japan"],"prefix":"10.1002","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3374-7111","authenticated-orcid":false,"given":"Masanori","family":"Atsukawa","sequence":"first","affiliation":[{"name":"Division of Gastroenterology and Hepatology Nippon Medical School  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4625-3762","authenticated-orcid":false,"given":"Masashi","family":"Takano","sequence":"additional","affiliation":[{"name":"Medical Affairs Division Janssen Pharmaceutical K.K.  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6015-5739","authenticated-orcid":false,"given":"Junichi","family":"Omura","sequence":"additional","affiliation":[{"name":"Medical Affairs Division Janssen Pharmaceutical K.K.  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,10,13]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22275"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.21431"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/hepr.13560"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehv317"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00032407"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00007890-199702270-00020"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.06.00030206"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(19)30091-8"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.25747"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(91)90225-A"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-021-01452-3"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2014.04.004"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2005.10.013"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1872-034X.2012.01033.x"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.24198"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-3411"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045894020962917"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200804-613OC"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2008.02384.x"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/TXD.0000000000001162"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.2957\/kanzo.61.314"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.3892\/etm.2016.3945"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/hepr.13714"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00138706"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)01786-2"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.4254\/wjh.v6.i11.825"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacasi.2022.02.011"}],"container-title":["JGH Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jgh3.12820","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jgh3.12820","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jgh3.12820","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,22]],"date-time":"2023-08-22T08:29:00Z","timestamp":1692692940000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jgh3.12820"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10,13]]},"references-count":28,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2022,11]]}},"alternative-id":["10.1002\/jgh3.12820"],"URL":"https:\/\/doi.org\/10.1002\/jgh3.12820","archive":["Portico"],"relation":{},"ISSN":["2397-9070","2397-9070"],"issn-type":[{"value":"2397-9070","type":"print"},{"value":"2397-9070","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,10,13]]},"assertion":[{"value":"2022-06-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-09-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-10-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}