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Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis
Background Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their...
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| Published in: | Pediatric transplantation 2023-12, Vol.27 (8), p.e14600-n/a |
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| container_title | Pediatric transplantation |
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| creator | Vasudevan, Anu K. Shanmugam, Naresh Rammohan, Ashwin Valamparampil, Joseph J. Rinaldhy, Kshetra Menon, Jagadeesh Thambithurai, Ravikumar Namasivayam, Saravanapandian Kaliamoorthy, Ilankumaran Rela, Mohamed |
| description | Background
Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long‐term outcomes, highlighting their individual differences and management strategies.
Methods
Prospectively collected data over a decade (2011–2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1–4) were compared with regard to early and long‐term outcomes including attainment of catch‐up growth.
Results
Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post‐LT chronic diarrhea was observed in 6 (46.1%) children with PFIC‐I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1‐year follow‐up, their graft steatosis resolved and they attained catch‐up growth. Catch‐up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p |
| doi_str_mv | 10.1111/petr.14600 |
| format | article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2862198667</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2862198667</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3520-99e1645c600344815d7e32d90ccc093e0a4e0e7bf3fba7419bae42f11188959f3</originalsourceid><addsrcrecordid>eNp9kEtLAzEUhYMovjf-AAm4EWFqMsk8spRSH1BQpC5lSNMbTZmZjMmM0n_vrVUXLswmWXycfOcQcsLZiOO57KAPIy5zxrbIPhdKJYLJfPvrXSSCy3SPHMS4ZIznspS7ZE8UeZGVpdonz_dDb3wDkXpLO1g43QdnaO3eIdA-6DZ2tW573TvfUusD7YJ_CRAjAtTqxtVO19S1iL5Ch5ih5tXXEHsdXTwiO1bXEY6_70PydD2ZjW-T6f3N3fhqmhiRpSxRCtAsM9hASFnybFGASBeKGWOYEsC0BAbF3Ao714Xkaq5Bpha7Y4dMWXFIzje5aPc24OdV46KBGtXBD7FKyzzlqszzAtGzP-jSD6FFO6SUXBuIFKmLDWWCjzGArbrgGh1WFWfVevRqPXr1NTrCp9-Rw7yBxS_6szICfAN8uBpW_0RVD5PZ4yb0EzPVjX8</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2894003432</pqid></control><display><type>article</type><title>Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis</title><source>Wiley-Blackwell Read & Publish Collection</source><creator>Vasudevan, Anu K. ; Shanmugam, Naresh ; Rammohan, Ashwin ; Valamparampil, Joseph J. ; Rinaldhy, Kshetra ; Menon, Jagadeesh ; Thambithurai, Ravikumar ; Namasivayam, Saravanapandian ; Kaliamoorthy, Ilankumaran ; Rela, Mohamed</creator><creatorcontrib>Vasudevan, Anu K. ; Shanmugam, Naresh ; Rammohan, Ashwin ; Valamparampil, Joseph J. ; Rinaldhy, Kshetra ; Menon, Jagadeesh ; Thambithurai, Ravikumar ; Namasivayam, Saravanapandian ; Kaliamoorthy, Ilankumaran ; Rela, Mohamed</creatorcontrib><description>Background
Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long‐term outcomes, highlighting their individual differences and management strategies.
Methods
Prospectively collected data over a decade (2011–2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1–4) were compared with regard to early and long‐term outcomes including attainment of catch‐up growth.
Results
Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post‐LT chronic diarrhea was observed in 6 (46.1%) children with PFIC‐I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1‐year follow‐up, their graft steatosis resolved and they attained catch‐up growth. Catch‐up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p < .001). Overall 1‐ and 5‐year patient survival of the four PFIC groups (1–4) were 69.2%, 81.8%, 96.8%, 100% & 69.2%, 81.8%, 96.8%, 100%, respectively.
Conclusion
Ours is the largest to‐date series of LT for PFIC illustrating their short‐ and long‐term outcomes. While the results for the whole cohort were excellent, those after LT for PFIC1 was relatively poorer as reflected by catch‐up growth, graft steatosis, and post‐LT diarrhea, which can be optimized by the addition of TIBD during LT.
We present the largest to‐date series of LT for PFIC focusing on its short‐ and long‐term outcomes. Early post‐LT morbidity, post‐LT survival is excellent and comparable across the different types of PFIC. Catch‐up growth though present remains significantly poorer in PFIC‐I, which can be considerably improved by performing TIBD during the LT.</description><identifier>ISSN: 1397-3142</identifier><identifier>ISSN: 1399-3046</identifier><identifier>EISSN: 1399-3046</identifier><identifier>DOI: 10.1111/petr.14600</identifier><identifier>PMID: 37675889</identifier><language>eng</language><publisher>Denmark: Wiley Subscription Services, Inc</publisher><subject>Child ; Children ; Cholestasis ; Cholestasis, Intrahepatic - surgery ; Clinical aspects ; Diarrhea ; Disease Progression ; Fatty Liver ; Gallbladder diseases ; Humans ; Ischemia ; Liver Transplantation ; Morbidity ; outcomes ; Pediatrics ; progressive familial intrahepatic cholestasis ; quality of life ; Steatosis ; Total internal biliary diversion</subject><ispartof>Pediatric transplantation, 2023-12, Vol.27 (8), p.e14600-n/a</ispartof><rights>2023 Wiley Periodicals LLC.</rights><rights>2023 Wiley Periodicals, LLC.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3520-99e1645c600344815d7e32d90ccc093e0a4e0e7bf3fba7419bae42f11188959f3</cites><orcidid>0000-0001-9528-8892 ; 0000-0003-3342-6326 ; 0000-0002-2649-0058 ; 0000-0002-8114-2523</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37675889$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Vasudevan, Anu K.</creatorcontrib><creatorcontrib>Shanmugam, Naresh</creatorcontrib><creatorcontrib>Rammohan, Ashwin</creatorcontrib><creatorcontrib>Valamparampil, Joseph J.</creatorcontrib><creatorcontrib>Rinaldhy, Kshetra</creatorcontrib><creatorcontrib>Menon, Jagadeesh</creatorcontrib><creatorcontrib>Thambithurai, Ravikumar</creatorcontrib><creatorcontrib>Namasivayam, Saravanapandian</creatorcontrib><creatorcontrib>Kaliamoorthy, Ilankumaran</creatorcontrib><creatorcontrib>Rela, Mohamed</creatorcontrib><title>Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis</title><title>Pediatric transplantation</title><addtitle>Pediatr Transplant</addtitle><description>Background
Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long‐term outcomes, highlighting their individual differences and management strategies.
Methods
Prospectively collected data over a decade (2011–2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1–4) were compared with regard to early and long‐term outcomes including attainment of catch‐up growth.
Results
Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post‐LT chronic diarrhea was observed in 6 (46.1%) children with PFIC‐I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1‐year follow‐up, their graft steatosis resolved and they attained catch‐up growth. Catch‐up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p < .001). Overall 1‐ and 5‐year patient survival of the four PFIC groups (1–4) were 69.2%, 81.8%, 96.8%, 100% & 69.2%, 81.8%, 96.8%, 100%, respectively.
Conclusion
Ours is the largest to‐date series of LT for PFIC illustrating their short‐ and long‐term outcomes. While the results for the whole cohort were excellent, those after LT for PFIC1 was relatively poorer as reflected by catch‐up growth, graft steatosis, and post‐LT diarrhea, which can be optimized by the addition of TIBD during LT.
We present the largest to‐date series of LT for PFIC focusing on its short‐ and long‐term outcomes. Early post‐LT morbidity, post‐LT survival is excellent and comparable across the different types of PFIC. Catch‐up growth though present remains significantly poorer in PFIC‐I, which can be considerably improved by performing TIBD during the LT.</description><subject>Child</subject><subject>Children</subject><subject>Cholestasis</subject><subject>Cholestasis, Intrahepatic - surgery</subject><subject>Clinical aspects</subject><subject>Diarrhea</subject><subject>Disease Progression</subject><subject>Fatty Liver</subject><subject>Gallbladder diseases</subject><subject>Humans</subject><subject>Ischemia</subject><subject>Liver Transplantation</subject><subject>Morbidity</subject><subject>outcomes</subject><subject>Pediatrics</subject><subject>progressive familial intrahepatic cholestasis</subject><subject>quality of life</subject><subject>Steatosis</subject><subject>Total internal biliary diversion</subject><issn>1397-3142</issn><issn>1399-3046</issn><issn>1399-3046</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><recordid>eNp9kEtLAzEUhYMovjf-AAm4EWFqMsk8spRSH1BQpC5lSNMbTZmZjMmM0n_vrVUXLswmWXycfOcQcsLZiOO57KAPIy5zxrbIPhdKJYLJfPvrXSSCy3SPHMS4ZIznspS7ZE8UeZGVpdonz_dDb3wDkXpLO1g43QdnaO3eIdA-6DZ2tW573TvfUusD7YJ_CRAjAtTqxtVO19S1iL5Ch5ih5tXXEHsdXTwiO1bXEY6_70PydD2ZjW-T6f3N3fhqmhiRpSxRCtAsM9hASFnybFGASBeKGWOYEsC0BAbF3Ao714Xkaq5Bpha7Y4dMWXFIzje5aPc24OdV46KBGtXBD7FKyzzlqszzAtGzP-jSD6FFO6SUXBuIFKmLDWWCjzGArbrgGh1WFWfVevRqPXr1NTrCp9-Rw7yBxS_6szICfAN8uBpW_0RVD5PZ4yb0EzPVjX8</recordid><startdate>202312</startdate><enddate>202312</enddate><creator>Vasudevan, Anu K.</creator><creator>Shanmugam, Naresh</creator><creator>Rammohan, Ashwin</creator><creator>Valamparampil, Joseph J.</creator><creator>Rinaldhy, Kshetra</creator><creator>Menon, Jagadeesh</creator><creator>Thambithurai, Ravikumar</creator><creator>Namasivayam, Saravanapandian</creator><creator>Kaliamoorthy, Ilankumaran</creator><creator>Rela, Mohamed</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/0000-0001-9528-8892</orcidid><orcidid>https://orcid.org/0000-0003-3342-6326</orcidid><orcidid>https://orcid.org/0000-0002-2649-0058</orcidid><orcidid>https://orcid.org/0000-0002-8114-2523</orcidid></search><sort><creationdate>202312</creationdate><title>Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis</title><author>Vasudevan, Anu K. ; Shanmugam, Naresh ; Rammohan, Ashwin ; Valamparampil, Joseph J. ; Rinaldhy, Kshetra ; Menon, Jagadeesh ; Thambithurai, Ravikumar ; Namasivayam, Saravanapandian ; Kaliamoorthy, Ilankumaran ; Rela, Mohamed</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3520-99e1645c600344815d7e32d90ccc093e0a4e0e7bf3fba7419bae42f11188959f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Child</topic><topic>Children</topic><topic>Cholestasis</topic><topic>Cholestasis, Intrahepatic - surgery</topic><topic>Clinical aspects</topic><topic>Diarrhea</topic><topic>Disease Progression</topic><topic>Fatty Liver</topic><topic>Gallbladder diseases</topic><topic>Humans</topic><topic>Ischemia</topic><topic>Liver Transplantation</topic><topic>Morbidity</topic><topic>outcomes</topic><topic>Pediatrics</topic><topic>progressive familial intrahepatic cholestasis</topic><topic>quality of life</topic><topic>Steatosis</topic><topic>Total internal biliary diversion</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vasudevan, Anu K.</creatorcontrib><creatorcontrib>Shanmugam, Naresh</creatorcontrib><creatorcontrib>Rammohan, Ashwin</creatorcontrib><creatorcontrib>Valamparampil, Joseph J.</creatorcontrib><creatorcontrib>Rinaldhy, Kshetra</creatorcontrib><creatorcontrib>Menon, Jagadeesh</creatorcontrib><creatorcontrib>Thambithurai, Ravikumar</creatorcontrib><creatorcontrib>Namasivayam, Saravanapandian</creatorcontrib><creatorcontrib>Kaliamoorthy, Ilankumaran</creatorcontrib><creatorcontrib>Rela, Mohamed</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & 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>Vasudevan, Anu K.</au><au>Shanmugam, Naresh</au><au>Rammohan, Ashwin</au><au>Valamparampil, Joseph J.</au><au>Rinaldhy, Kshetra</au><au>Menon, Jagadeesh</au><au>Thambithurai, Ravikumar</au><au>Namasivayam, Saravanapandian</au><au>Kaliamoorthy, Ilankumaran</au><au>Rela, Mohamed</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis</atitle><jtitle>Pediatric transplantation</jtitle><addtitle>Pediatr Transplant</addtitle><date>2023-12</date><risdate>2023</risdate><volume>27</volume><issue>8</issue><spage>e14600</spage><epage>n/a</epage><pages>e14600-n/a</pages><issn>1397-3142</issn><issn>1399-3046</issn><eissn>1399-3046</eissn><abstract>Background
Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long‐term outcomes, highlighting their individual differences and management strategies.
Methods
Prospectively collected data over a decade (2011–2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1–4) were compared with regard to early and long‐term outcomes including attainment of catch‐up growth.
Results
Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post‐LT chronic diarrhea was observed in 6 (46.1%) children with PFIC‐I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1‐year follow‐up, their graft steatosis resolved and they attained catch‐up growth. Catch‐up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p < .001). Overall 1‐ and 5‐year patient survival of the four PFIC groups (1–4) were 69.2%, 81.8%, 96.8%, 100% & 69.2%, 81.8%, 96.8%, 100%, respectively.
Conclusion
Ours is the largest to‐date series of LT for PFIC illustrating their short‐ and long‐term outcomes. While the results for the whole cohort were excellent, those after LT for PFIC1 was relatively poorer as reflected by catch‐up growth, graft steatosis, and post‐LT diarrhea, which can be optimized by the addition of TIBD during LT.
We present the largest to‐date series of LT for PFIC focusing on its short‐ and long‐term outcomes. Early post‐LT morbidity, post‐LT survival is excellent and comparable across the different types of PFIC. Catch‐up growth though present remains significantly poorer in PFIC‐I, which can be considerably improved by performing TIBD during the LT.</abstract><cop>Denmark</cop><pub>Wiley Subscription Services, Inc</pub><pmid>37675889</pmid><doi>10.1111/petr.14600</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0001-9528-8892</orcidid><orcidid>https://orcid.org/0000-0003-3342-6326</orcidid><orcidid>https://orcid.org/0000-0002-2649-0058</orcidid><orcidid>https://orcid.org/0000-0002-8114-2523</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Child Children Cholestasis Cholestasis, Intrahepatic - surgery Clinical aspects Diarrhea Disease Progression Fatty Liver Gallbladder diseases Humans Ischemia Liver Transplantation Morbidity outcomes Pediatrics progressive familial intrahepatic cholestasis quality of life Steatosis Total internal biliary diversion |
| title | Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis |
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