{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T14:37:18Z","timestamp":1783089438117,"version":"3.54.6"},"reference-count":16,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","license":[{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2018,9,24]],"date-time":"2018-09-24T00:00:00Z","timestamp":1537747200000},"content-version":"vor","delay-in-days":23,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver Transpl"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p>Hepatopulmonary syndrome (HPS) in stable patients with cirrhosis can easily be overlooked. We report on the presenting symptoms, disease progression, and outcomes after liver transplantation (LT) in children with HPS. Twenty patients were diagnosed with HPS between 1996 and 2016. The etiologies were as follows: biliary atresia (n = 9); alpha\u20101\u2010antitrypsin deficiency (n = 2); cryptogenic liver disease (n = 3); and others (n = 6). HPS presentations were as follows; dyspnea (n = 17) and pneumonia (n = 3). For diagnostic confirmation, the following techniques were used: technetium\u201099m\u2010labeled macroaggregated albumin lung perfusion scan (n = 13) or contrast echocardiogram (n = 7). There were 16 patients listed for LT, with a median age at HPS diagnosis of 10 years and an average wait from listing to LT of 9 weeks. A marked rise in hemoglobin (Hb; median, 125\u2010143.5 g\/L) and modest decrease in oxygen saturation (SpO<jats:sub>2<\/jats:sub>; median 91% to 88% room air) were evident over this time. Patients\u2019 need for assisted ventilation (1 day), pediatric intensive care unit (PICU) stay (3 days), and total hospital stay (20 days) were similar to our general LT recipients\u2014the key difference in the postoperative period was the duration of supplementary O<jats:sub>2<\/jats:sub> requirement. Hb of \u2265130 g\/L on the day of LT correlated with a longer PICU stay (<jats:italic toggle=\"yes\">P <\/jats:italic>value = 0.02), duration of supplementary O<jats:sub>2 <\/jats:sub>(<jats:italic toggle=\"yes\">P<\/jats:italic> value = 0.005), and the need for the latter beyond 7 days after LT (<jats:italic toggle=\"yes\">P <\/jats:italic>value = 0.01). Fifteen patients had resolution of their HPS after LT. The 5\u2010, 10\u2010, and 20\u2010year survival rates were unchanged at 87.5%. None had a recurrence of HPS. In conclusion, HPS is a life\u2010threatening complication of cirrhosis which usually develops insidiously. This combined with the often\u2010stable nature of the liver disease leads to delays in diagnosis and listing for LT. Progressive polycythemia extends the need for supplementary O<jats:sub>2 <\/jats:sub>and PICU stay. We advocate screening for HPS with a combination of SpO<jats:sub>2 <\/jats:sub>and Hb monitoring to facilitate earlier recognition, timely LT, and shortened recovery periods.<\/jats:p>","DOI":"10.1002\/lt.25296","type":"journal-article","created":{"date-parts":[[2018,8,1]],"date-time":"2018-08-01T14:02:18Z","timestamp":1533132138000},"page":"1271-1279","source":"Crossref","is-referenced-by-count":38,"title":["Hepatopulmonary Syndrome in Children: A 20\u2010Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome"],"prefix":"10.1097","volume":"24","author":[{"given":"Suz","family":"Warner","sequence":"first","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"},{"name":"Centre for Liver Research and National Institute for Health Research Biomedical Research Unit in Liver Disease,Institute of Immunology and Immunotherapy,University of Birmingham,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick J.","family":"McKiernan","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane","family":"Hartley","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelyn","family":"Ong","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Indra D.","family":"van Mourik","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Girish","family":"Gupte","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mona","family":"Abdel\u2010Hady","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Muiesan","sequence":"additional","affiliation":[{"name":"University Hospitals Birmingham National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thamera","family":"Perera","sequence":"additional","affiliation":[{"name":"University Hospitals Birmingham National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darius","family":"Mirza","sequence":"additional","affiliation":[{"name":"University Hospitals Birmingham National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khalid","family":"Sharif","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deirdre A.","family":"Kelly","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan V.","family":"Beath","sequence":"additional","affiliation":[{"name":"The Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospitals National Health Service Foundation Trust,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2018,9,24]]},"reference":[{"key":"lt25296-bib-0001-20240115","doi-asserted-by":"crossref","first-page":"2427","DOI":"10.1002\/hep.26070","article-title":"Hepatopulmonary syndrome: favorable outcomes in the MELD exception era","volume":"57","year":"2013","journal-title":"Hepatology"},{"key":"lt25296-bib-0002-20240115","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1111\/j.1399-3046.2007.00807.x","article-title":"Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation","volume":"12","year":"2008","journal-title":"Pediatr Transplant"},{"key":"lt25296-bib-0003-20240115","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1002\/hep.20658","article-title":"Natural history of hepatopulmonary syndrome: impact of liver transplantation","volume":"41","year":"2005","journal-title":"Hepatology"},{"key":"lt25296-bib-0004-20240115","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/j.paed.2015.02.002","article-title":"Hepatopulmonary syndrome in children\u2010an update","volume":"25","year":"2015","journal-title":"Paediatr Child Health"},{"key":"lt25296-bib-0005-20240115","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1111\/j.1399-3046.2009.01218.x","article-title":"Long\u2010term outcome and management of hepatopulmonary syndrome in children","volume":"14","year":"2010","journal-title":"Pediatr Transplant"},{"key":"lt25296-bib-0006-20240115","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1111\/j.1440-1843.2006.00796.x","article-title":"Hepatopulmonary syndrome can show spontaneous resolution: possible mechanism of portopulmonary hypertension overlap?","volume":"11","year":"2006","journal-title":"Respirology"},{"key":"lt25296-bib-0007-20240115","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.cld.2014.01.003","article-title":"Hepatopulmonary syndrome","volume":"18","year":"2014","journal-title":"Clin Liver Dis"},{"key":"lt25296-bib-0008-20240115","doi-asserted-by":"crossref","first-page":"1914","DOI":"10.1007\/s10620-015-3593-0","article-title":"Hepatopulmonary syndrome","volume":"60","year":"2015","journal-title":"Dig Dis Sci"},{"key":"lt25296-bib-0009-20240115","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1002\/lt.20362","article-title":"Evidence\u2010based evolution of the MELD\/PELD liver allocation policy","volume":"11","year":"2005","journal-title":"Liver Transpl"},{"key":"lt25296-bib-0010-20240115","doi-asserted-by":"crossref","first-page":"1862","DOI":"10.1007\/s10620-011-1600-7","article-title":"Serial pulse oximetry in hepatopulmonary syndrome","volume":"56","year":"2011","journal-title":"Dig Dis Sci"},{"issue":"pt 2","key":"lt25296-bib-0011-20240115","first-page":"2460","article-title":"Low haematocrit reduces hepatic artery thrombosis after liver transplantation","volume":"21","year":"1989","journal-title":"Transplant Proc"},{"key":"lt25296-bib-0012-20240115","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1002\/lt.23874","article-title":"Etiology and management of hepatic artery thrombosis after adult liver transplantation","volume":"20","year":"2014","journal-title":"Liver Transpl"},{"key":"lt25296-bib-0013-20240115","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1111\/ped.12711","article-title":"Brain abscess in hepatopulmonary syndrome associated with biliary atresia","volume":"57","year":"2015","journal-title":"Pediatr Int"},{"key":"lt25296-bib-0014-20240115","first-page":"E130","article-title":"Nitric oxide for post\u2010liver\u2010transplantation hypoxemia in pediatric hepatopulmonary syndrome: case report and review","volume":"15","year":"2011","journal-title":"Pediatr Transplant"},{"key":"lt25296-bib-0015-20240115","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1183\/09059180.00007211","article-title":"Portopulmonary hypertension and hepatopulmonary syndrome: a clinician\u2010oriented overview","volume":"21","year":"2012","journal-title":"Eur Respir Rev"},{"key":"lt25296-bib-0016-20240115","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1016\/j.jpeds.2013.10.070","article-title":"Pulse oximetry is insufficient for timely diagnosis of hepatopulmonary syndrome in children with liver cirrhosis","volume":"164","year":"2014","journal-title":"J Pediatr"}],"container-title":["Liver 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