Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents

Background Severe HPS increases morbidity and mortality after LT in children. We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. Methods All children with “proven” HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were cat...

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Veröffentlicht in:Pediatric transplantation 2021-11, Vol.25 (7), p.e14088-n/a
Hauptverfasser: Shanmugam, Naresh, Hakeem, Abdul Rahman, Valamparampil, Joseph J., Aldouri, Ahmed, Bansal, Mehak, Reddy, Mettu Srinivas, Thiruchunapalli, Deepashree, Vilca‐Melendez, Hector, Baker, Alastair, Dhawan, Anil, Heaton, Nigel, Rela, Mohamed, Deep, Akash
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container_issue 7
container_start_page e14088
container_title Pediatric transplantation
container_volume 25
creator Shanmugam, Naresh
Hakeem, Abdul Rahman
Valamparampil, Joseph J.
Aldouri, Ahmed
Bansal, Mehak
Reddy, Mettu Srinivas
Thiruchunapalli, Deepashree
Vilca‐Melendez, Hector
Baker, Alastair
Dhawan, Anil
Heaton, Nigel
Rela, Mohamed
Deep, Akash
description Background Severe HPS increases morbidity and mortality after LT in children. We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. Methods All children with “proven” HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were categorized into M (PaO2 ≥80 mmHg), Mo (PaO2 = 60–79 mmHg), S (50–59 mmHg), and VS (PaO2
doi_str_mv 10.1111/petr.14088
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We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. Methods All children with “proven” HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were categorized into M (PaO2 ≥80 mmHg), Mo (PaO2 = 60–79 mmHg), S (50–59 mmHg), and VS (PaO2 &lt;50 mmHg) HPS, based on room air PaO2. Results Twenty‐four children with HPS underwent 25 LT (one re‐transplantation) at a median age of 8 years (IQR, 5–12), after a median duration of 8 (4–12) months following HPS diagnosis. Mechanical ventilation was required for a median of 3 (1.5–27) days after LT. Ten children had “S” post‐operative hypoxemia, requiring iNO for a median of 5 (6–27) days. “VS” category patients had significantly prolonged invasive ventilation (median 35 vs. 3 and 1.5 days; p = .008), ICU stay (median 39 vs. 8 and 8 days; p = .007), and hospital stay (64 vs. 26.5 and 23 days; p &lt; .001) when compared to “S” and “M/Mo” groups, respectively. The need for pre‐transplant home oxygen therapy was the only factor predicting need for re‐intubation. Patient and graft survival at 32 (17–98) months were 100% and 95.8%. All children ultimately had complete resolution of HPS. Conclusions VS HPS is associated with longer duration of mechanical ventilation and hospital stay, which emphasizes the need for early LT in these children.</description><identifier>ISSN: 1397-3142</identifier><identifier>EISSN: 1399-3046</identifier><identifier>DOI: 10.1111/petr.14088</identifier><identifier>PMID: 34351678</identifier><language>eng</language><publisher>Denmark: Wiley Subscription Services, Inc</publisher><subject>Adolescent ; Child ; Child, Preschool ; Children ; failed extubation ; Female ; Graft Survival ; Hepatopulmonary Syndrome - mortality ; Hepatopulmonary Syndrome - surgery ; HPS ; Humans ; Hypoxemia ; Infant ; intrapulmonary vascular dilatation ; Intubation ; Liver Transplantation ; Liver transplants ; London - epidemiology ; Male ; Mechanical ventilation ; Morbidity ; Patients ; portosystemic shunt ; prolonged ventilation ; Retrospective Studies ; Survival ; Survival Analysis ; Transplants &amp; implants ; Ventilation ; Ventilators</subject><ispartof>Pediatric transplantation, 2021-11, Vol.25 (7), p.e14088-n/a</ispartof><rights>2021 Wiley Periodicals LLC</rights><rights>2021 Wiley Periodicals LLC.</rights><rights>2021 John Wiley &amp; Sons A/S. Published by John Wiley &amp; Sons Ltd.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3578-f6235fb2682ab93bb80f75145dbe36f798ac2d7f1bddb9b498ba4831d5134d983</citedby><cites>FETCH-LOGICAL-c3578-f6235fb2682ab93bb80f75145dbe36f798ac2d7f1bddb9b498ba4831d5134d983</cites><orcidid>0000-0002-8114-2523 ; 0000-0001-7266-3848 ; 0000-0001-7152-0546</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fpetr.14088$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fpetr.14088$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34351678$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Shanmugam, Naresh</creatorcontrib><creatorcontrib>Hakeem, Abdul Rahman</creatorcontrib><creatorcontrib>Valamparampil, Joseph J.</creatorcontrib><creatorcontrib>Aldouri, Ahmed</creatorcontrib><creatorcontrib>Bansal, Mehak</creatorcontrib><creatorcontrib>Reddy, Mettu Srinivas</creatorcontrib><creatorcontrib>Thiruchunapalli, Deepashree</creatorcontrib><creatorcontrib>Vilca‐Melendez, Hector</creatorcontrib><creatorcontrib>Baker, Alastair</creatorcontrib><creatorcontrib>Dhawan, Anil</creatorcontrib><creatorcontrib>Heaton, Nigel</creatorcontrib><creatorcontrib>Rela, Mohamed</creatorcontrib><creatorcontrib>Deep, Akash</creatorcontrib><title>Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents</title><title>Pediatric transplantation</title><addtitle>Pediatr Transplant</addtitle><description>Background Severe HPS increases morbidity and mortality after LT in children. We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. Methods All children with “proven” HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were categorized into M (PaO2 ≥80 mmHg), Mo (PaO2 = 60–79 mmHg), S (50–59 mmHg), and VS (PaO2 &lt;50 mmHg) HPS, based on room air PaO2. Results Twenty‐four children with HPS underwent 25 LT (one re‐transplantation) at a median age of 8 years (IQR, 5–12), after a median duration of 8 (4–12) months following HPS diagnosis. Mechanical ventilation was required for a median of 3 (1.5–27) days after LT. Ten children had “S” post‐operative hypoxemia, requiring iNO for a median of 5 (6–27) days. “VS” category patients had significantly prolonged invasive ventilation (median 35 vs. 3 and 1.5 days; p = .008), ICU stay (median 39 vs. 8 and 8 days; p = .007), and hospital stay (64 vs. 26.5 and 23 days; p &lt; .001) when compared to “S” and “M/Mo” groups, respectively. The need for pre‐transplant home oxygen therapy was the only factor predicting need for re‐intubation. Patient and graft survival at 32 (17–98) months were 100% and 95.8%. All children ultimately had complete resolution of HPS. Conclusions VS HPS is associated with longer duration of mechanical ventilation and hospital stay, which emphasizes the need for early LT in these children.</description><subject>Adolescent</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>failed extubation</subject><subject>Female</subject><subject>Graft Survival</subject><subject>Hepatopulmonary Syndrome - mortality</subject><subject>Hepatopulmonary Syndrome - surgery</subject><subject>HPS</subject><subject>Humans</subject><subject>Hypoxemia</subject><subject>Infant</subject><subject>intrapulmonary vascular dilatation</subject><subject>Intubation</subject><subject>Liver Transplantation</subject><subject>Liver transplants</subject><subject>London - epidemiology</subject><subject>Male</subject><subject>Mechanical ventilation</subject><subject>Morbidity</subject><subject>Patients</subject><subject>portosystemic shunt</subject><subject>prolonged ventilation</subject><subject>Retrospective Studies</subject><subject>Survival</subject><subject>Survival Analysis</subject><subject>Transplants &amp; implants</subject><subject>Ventilation</subject><subject>Ventilators</subject><issn>1397-3142</issn><issn>1399-3046</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kU9r3DAQxUVJaf60l36AIOilFJxIlmRLvYWwTQKBLun2bCRr3FWwZUeyvc23j3Y3ySGHzGWG4cdj3jyEvlJyRlOdDzCGM8qJlB_QEWVKZYzw4mA3lxmjPD9ExzHeE0ILLvkndMg4E7Qo5REKN90Q-hksjlOY3axb7Dyu1661ATzeuHGNr5d_fuLF_wGCA18DbkLf4XHT47X7t8Zz304d4NbNEPAYtI9Dq_2Ia_AjhIh1HfoYcd370fm0i5_Rx0a3Eb489xP099didXmd3f6-urm8uM1qJkqZNUXORGPyQubaKGaMJE0pKBfWACuaUkld57ZsqLHWKMOVNJpLRq2gjFsl2Qn6vtdNBh8miGPVuVhDm66DfopVLoTkIid8i357g973U_DpukRJUijKuUrUjz21cxSgqYbgOh0eK0qqbRLVNolql0SCT58lJ9OBfUVfXp8Augc2roXHd6Sq5WJ1txd9AlnqlQw</recordid><startdate>202111</startdate><enddate>202111</enddate><creator>Shanmugam, Naresh</creator><creator>Hakeem, Abdul Rahman</creator><creator>Valamparampil, Joseph J.</creator><creator>Aldouri, Ahmed</creator><creator>Bansal, Mehak</creator><creator>Reddy, Mettu Srinivas</creator><creator>Thiruchunapalli, Deepashree</creator><creator>Vilca‐Melendez, Hector</creator><creator>Baker, Alastair</creator><creator>Dhawan, Anil</creator><creator>Heaton, Nigel</creator><creator>Rela, Mohamed</creator><creator>Deep, Akash</creator><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-8114-2523</orcidid><orcidid>https://orcid.org/0000-0001-7266-3848</orcidid><orcidid>https://orcid.org/0000-0001-7152-0546</orcidid></search><sort><creationdate>202111</creationdate><title>Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents</title><author>Shanmugam, Naresh ; Hakeem, Abdul Rahman ; Valamparampil, Joseph J. ; Aldouri, Ahmed ; Bansal, Mehak ; Reddy, Mettu Srinivas ; Thiruchunapalli, Deepashree ; Vilca‐Melendez, Hector ; Baker, Alastair ; Dhawan, Anil ; Heaton, Nigel ; Rela, Mohamed ; Deep, Akash</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3578-f6235fb2682ab93bb80f75145dbe36f798ac2d7f1bddb9b498ba4831d5134d983</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Adolescent</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Children</topic><topic>failed extubation</topic><topic>Female</topic><topic>Graft Survival</topic><topic>Hepatopulmonary Syndrome - mortality</topic><topic>Hepatopulmonary Syndrome - surgery</topic><topic>HPS</topic><topic>Humans</topic><topic>Hypoxemia</topic><topic>Infant</topic><topic>intrapulmonary vascular dilatation</topic><topic>Intubation</topic><topic>Liver Transplantation</topic><topic>Liver transplants</topic><topic>London - epidemiology</topic><topic>Male</topic><topic>Mechanical ventilation</topic><topic>Morbidity</topic><topic>Patients</topic><topic>portosystemic shunt</topic><topic>prolonged ventilation</topic><topic>Retrospective Studies</topic><topic>Survival</topic><topic>Survival Analysis</topic><topic>Transplants &amp; 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Medical Complete (Alumni)</collection><collection>Nursing &amp; Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric transplantation</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Shanmugam, Naresh</au><au>Hakeem, Abdul Rahman</au><au>Valamparampil, Joseph J.</au><au>Aldouri, Ahmed</au><au>Bansal, Mehak</au><au>Reddy, Mettu Srinivas</au><au>Thiruchunapalli, Deepashree</au><au>Vilca‐Melendez, Hector</au><au>Baker, Alastair</au><au>Dhawan, Anil</au><au>Heaton, Nigel</au><au>Rela, Mohamed</au><au>Deep, Akash</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents</atitle><jtitle>Pediatric transplantation</jtitle><addtitle>Pediatr Transplant</addtitle><date>2021-11</date><risdate>2021</risdate><volume>25</volume><issue>7</issue><spage>e14088</spage><epage>n/a</epage><pages>e14088-n/a</pages><issn>1397-3142</issn><eissn>1399-3046</eissn><abstract>Background Severe HPS increases morbidity and mortality after LT in children. We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. Methods All children with “proven” HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were categorized into M (PaO2 ≥80 mmHg), Mo (PaO2 = 60–79 mmHg), S (50–59 mmHg), and VS (PaO2 &lt;50 mmHg) HPS, based on room air PaO2. Results Twenty‐four children with HPS underwent 25 LT (one re‐transplantation) at a median age of 8 years (IQR, 5–12), after a median duration of 8 (4–12) months following HPS diagnosis. Mechanical ventilation was required for a median of 3 (1.5–27) days after LT. Ten children had “S” post‐operative hypoxemia, requiring iNO for a median of 5 (6–27) days. “VS” category patients had significantly prolonged invasive ventilation (median 35 vs. 3 and 1.5 days; p = .008), ICU stay (median 39 vs. 8 and 8 days; p = .007), and hospital stay (64 vs. 26.5 and 23 days; p &lt; .001) when compared to “S” and “M/Mo” groups, respectively. The need for pre‐transplant home oxygen therapy was the only factor predicting need for re‐intubation. Patient and graft survival at 32 (17–98) months were 100% and 95.8%. All children ultimately had complete resolution of HPS. Conclusions VS HPS is associated with longer duration of mechanical ventilation and hospital stay, which emphasizes the need for early LT in these children.</abstract><cop>Denmark</cop><pub>Wiley Subscription Services, Inc</pub><pmid>34351678</pmid><doi>10.1111/petr.14088</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-8114-2523</orcidid><orcidid>https://orcid.org/0000-0001-7266-3848</orcidid><orcidid>https://orcid.org/0000-0001-7152-0546</orcidid></addata></record>
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subjects Adolescent
Child
Child, Preschool
Children
failed extubation
Female
Graft Survival
Hepatopulmonary Syndrome - mortality
Hepatopulmonary Syndrome - surgery
HPS
Humans
Hypoxemia
Infant
intrapulmonary vascular dilatation
Intubation
Liver Transplantation
Liver transplants
London - epidemiology
Male
Mechanical ventilation
Morbidity
Patients
portosystemic shunt
prolonged ventilation
Retrospective Studies
Survival
Survival Analysis
Transplants & implants
Ventilation
Ventilators
title Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents
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