Clinical outcomes and risk factors of hepatopulmonary syndrome in children

Abstract Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to investigate the clinical outcomes of pediatric HPS and to identify the risk factors for HPS in children with biliary atresia (BA)...

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Autores principales:	Kwang Yeon Kim, Tae Hyeong Kim, Jeong-Moo Lee, Nam-Joon Yi, Hyun-Young Kim, Jin Soo Moon, Jae Sung Ko
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/aa048a9f4a2d4f528c617a81821c08bf
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spelling	oai:doaj.org-article:aa048a9f4a2d4f528c617a81821c08bf2021-12-02T14:04:00ZClinical outcomes and risk factors of hepatopulmonary syndrome in children10.1038/s41598-021-83785-x2045-2322https://doaj.org/article/aa048a9f4a2d4f528c617a81821c08bf2021-02-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-83785-xhttps://doaj.org/toc/2045-2322Abstract Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to investigate the clinical outcomes of pediatric HPS and to identify the risk factors for HPS in children with biliary atresia (BA). We performed a retrospective cohort study of all children who were diagnosed with HPS between 2000 and 2018 at Seoul National University Hospital. The clinical features and outcomes of the 10 patients diagnosed with HPS were reviewed. To clarify the risk factors of HPS in patients with BA, we reviewed 120 patients diagnosed with BA. Underlying liver disease was BA in 8 patients, portal vein agenesis in 1 patient, and portal vein thrombosis in 1 patient. A total of 7 patients underwent liver transplantation (LT). Currently, all seven patients, including 3 patients with severe HPS, survived after LT. The prevalence of HPS in children with BA was 7%. Polysplenia/interrupted inferior vena was the only risk factor for HPS in BA patients in multivariate analysis. The Pediatric End-Stage Liver Disease score was not associated with the development of HPS. Children with severe HPS undergoing LT had excellent outcomes. Screening for HPS in children with BA is required regardless of the severity of liver diseases.Kwang Yeon KimTae Hyeong KimJeong-Moo LeeNam-Joon YiHyun-Young KimJin Soo MoonJae Sung KoNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-6 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Kwang Yeon Kim Tae Hyeong Kim Jeong-Moo Lee Nam-Joon Yi Hyun-Young Kim Jin Soo Moon Jae Sung Ko Clinical outcomes and risk factors of hepatopulmonary syndrome in children
description	Abstract Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to investigate the clinical outcomes of pediatric HPS and to identify the risk factors for HPS in children with biliary atresia (BA). We performed a retrospective cohort study of all children who were diagnosed with HPS between 2000 and 2018 at Seoul National University Hospital. The clinical features and outcomes of the 10 patients diagnosed with HPS were reviewed. To clarify the risk factors of HPS in patients with BA, we reviewed 120 patients diagnosed with BA. Underlying liver disease was BA in 8 patients, portal vein agenesis in 1 patient, and portal vein thrombosis in 1 patient. A total of 7 patients underwent liver transplantation (LT). Currently, all seven patients, including 3 patients with severe HPS, survived after LT. The prevalence of HPS in children with BA was 7%. Polysplenia/interrupted inferior vena was the only risk factor for HPS in BA patients in multivariate analysis. The Pediatric End-Stage Liver Disease score was not associated with the development of HPS. Children with severe HPS undergoing LT had excellent outcomes. Screening for HPS in children with BA is required regardless of the severity of liver diseases.
format	article
author	Kwang Yeon Kim Tae Hyeong Kim Jeong-Moo Lee Nam-Joon Yi Hyun-Young Kim Jin Soo Moon Jae Sung Ko
author_facet	Kwang Yeon Kim Tae Hyeong Kim Jeong-Moo Lee Nam-Joon Yi Hyun-Young Kim Jin Soo Moon Jae Sung Ko
author_sort	Kwang Yeon Kim
title	Clinical outcomes and risk factors of hepatopulmonary syndrome in children
title_short	Clinical outcomes and risk factors of hepatopulmonary syndrome in children
title_full	Clinical outcomes and risk factors of hepatopulmonary syndrome in children
title_fullStr	Clinical outcomes and risk factors of hepatopulmonary syndrome in children
title_full_unstemmed	Clinical outcomes and risk factors of hepatopulmonary syndrome in children
title_sort	clinical outcomes and risk factors of hepatopulmonary syndrome in children
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/aa048a9f4a2d4f528c617a81821c08bf
work_keys_str_mv	AT kwangyeonkim clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren AT taehyeongkim clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren AT jeongmoolee clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren AT namjoonyi clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren AT hyunyoungkim clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren AT jinsoomoon clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren AT jaesungko clinicaloutcomesandriskfactorsofhepatopulmonarysyndromeinchildren
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Clinical outcomes and risk factors of hepatopulmonary syndrome in children

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