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Managing recurrent portal steal in auxiliary liver transplantation for non‐cirrhotic metabolic liver disease
Background APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and...
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| Published in: | Pediatric transplantation 2022-12, Vol.26 (8), p.n/a |
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| container_issue | 8 |
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| container_title | Pediatric transplantation |
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| creator | Vasudevan, Anu K. Shanmugam, Naresh P. Rammohan, Ashwin Rela, Mohamed |
| description | Background
APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and developed recurrent PS.
Case Report
A 2‐year‐old male child with a diagnosis of Citrullinemia type I underwent APOLT with a left lateral segment from a split deceased donor liver, and his postoperative period was unremarkable. Ammonia‐lowering agents were stopped 1 week following the operation and the child was discharged home on a normal diet. Four years following APOLT, the child presented with altered sensorium and seizures. A diagnosis of PS was made. Subsequent to an embolization of the native liver's right anterior portal vein his sensorium improved and he remained clinically stable on a normal diet. Six years following the APOLT, the child again presented with features of acute encephalopathy. Imaging was suggestive of PS. A portal vein embolization of the native portal vein was performed and the child's clinical condition improved. At 6 months' follow‐up, the child remains well on a normal diet.
Conclusions
While the early impediments in this technique may have been overcome, in the absence of any realistic clinical application gene therapy, the debate of long‐term phenotypic metabolic correction for NCMLD by APOLT needs to be revisited. |
| doi_str_mv | 10.1111/petr.14389 |
| format | article |
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APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and developed recurrent PS.
Case Report
A 2‐year‐old male child with a diagnosis of Citrullinemia type I underwent APOLT with a left lateral segment from a split deceased donor liver, and his postoperative period was unremarkable. Ammonia‐lowering agents were stopped 1 week following the operation and the child was discharged home on a normal diet. Four years following APOLT, the child presented with altered sensorium and seizures. A diagnosis of PS was made. Subsequent to an embolization of the native liver's right anterior portal vein his sensorium improved and he remained clinically stable on a normal diet. Six years following the APOLT, the child again presented with features of acute encephalopathy. Imaging was suggestive of PS. A portal vein embolization of the native portal vein was performed and the child's clinical condition improved. At 6 months' follow‐up, the child remains well on a normal diet.
Conclusions
While the early impediments in this technique may have been overcome, in the absence of any realistic clinical application gene therapy, the debate of long‐term phenotypic metabolic correction for NCMLD by APOLT needs to be revisited.</description><identifier>ISSN: 1397-3142</identifier><identifier>EISSN: 1399-3046</identifier><identifier>DOI: 10.1111/petr.14389</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc</publisher><subject>Ammonia ; CTLN1 ; Diagnosis ; Diet ; Embolization ; Encephalopathy ; Gene therapy ; Liver ; Liver diseases ; Liver transplantation ; Liver transplants ; metabolic profile ; Metabolism ; NCMLD ; Portal vein ; PVE ; Seizures</subject><ispartof>Pediatric transplantation, 2022-12, Vol.26 (8), p.n/a</ispartof><rights>2022 Wiley Periodicals LLC.</rights><rights>2022 Wiley Periodicals, LLC.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c2319-ff6eba5c749ae61bb4b457793d089aba26e2fbc6b3156480aa78c88875445e833</citedby><cites>FETCH-LOGICAL-c2319-ff6eba5c749ae61bb4b457793d089aba26e2fbc6b3156480aa78c88875445e833</cites><orcidid>0000-0001-9528-8892 ; 0000-0003-3342-6326</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.14389$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fpetr.14389$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>315,786,790,27957,27958,50923,51032</link.rule.ids></links><search><creatorcontrib>Vasudevan, Anu K.</creatorcontrib><creatorcontrib>Shanmugam, Naresh P.</creatorcontrib><creatorcontrib>Rammohan, Ashwin</creatorcontrib><creatorcontrib>Rela, Mohamed</creatorcontrib><title>Managing recurrent portal steal in auxiliary liver transplantation for non‐cirrhotic metabolic liver disease</title><title>Pediatric transplantation</title><description>Background
APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and developed recurrent PS.
Case Report
A 2‐year‐old male child with a diagnosis of Citrullinemia type I underwent APOLT with a left lateral segment from a split deceased donor liver, and his postoperative period was unremarkable. Ammonia‐lowering agents were stopped 1 week following the operation and the child was discharged home on a normal diet. Four years following APOLT, the child presented with altered sensorium and seizures. A diagnosis of PS was made. Subsequent to an embolization of the native liver's right anterior portal vein his sensorium improved and he remained clinically stable on a normal diet. Six years following the APOLT, the child again presented with features of acute encephalopathy. Imaging was suggestive of PS. A portal vein embolization of the native portal vein was performed and the child's clinical condition improved. At 6 months' follow‐up, the child remains well on a normal diet.
Conclusions
While the early impediments in this technique may have been overcome, in the absence of any realistic clinical application gene therapy, the debate of long‐term phenotypic metabolic correction for NCMLD by APOLT needs to be revisited.</description><subject>Ammonia</subject><subject>CTLN1</subject><subject>Diagnosis</subject><subject>Diet</subject><subject>Embolization</subject><subject>Encephalopathy</subject><subject>Gene therapy</subject><subject>Liver</subject><subject>Liver diseases</subject><subject>Liver transplantation</subject><subject>Liver transplants</subject><subject>metabolic profile</subject><subject>Metabolism</subject><subject>NCMLD</subject><subject>Portal vein</subject><subject>PVE</subject><subject>Seizures</subject><issn>1397-3142</issn><issn>1399-3046</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><recordid>eNp9kE1OwzAQhS0EEqWw4QSW2CGlxLHzt0RV-ZGKQKisrbHrFFepHWwH6I4jcEZOgtuwZhYzb_HNjN5D6JykExLrqlPBTQijVX2ARoTWdUJTVhzudZlQwrJjdOL9Ok1JwSo2QuYBDKy0WWGnZO-cMgF31gVosQ8qdm0w9J-61eC2uNXvyuHgwPiuBRMgaGtwYx021vx8fUvt3KsNWuKNCiBsG9Wws9RegVen6KiB1quzvzlGLzezxfQumT_e3k-v54nMKKmTpimUgFyWrAZVECGYYHlZ1nSZVjUIyAqVNUIWgpI8-kgBykpWVVXmjOWqonSMLoa7nbNvvfKBr23vTHzJs5IWJNoneaQuB0o6671TDe-c3kSjnKR8lyff5cn3eUaYDPCHbtX2H5I_zRbPw84vDq57xA</recordid><startdate>202212</startdate><enddate>202212</enddate><creator>Vasudevan, Anu K.</creator><creator>Shanmugam, Naresh P.</creator><creator>Rammohan, Ashwin</creator><creator>Rela, Mohamed</creator><general>Wiley Subscription Services, Inc</general><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><orcidid>https://orcid.org/0000-0001-9528-8892</orcidid><orcidid>https://orcid.org/0000-0003-3342-6326</orcidid></search><sort><creationdate>202212</creationdate><title>Managing recurrent portal steal in auxiliary liver transplantation for non‐cirrhotic metabolic liver disease</title><author>Vasudevan, Anu K. ; Shanmugam, Naresh P. ; Rammohan, Ashwin ; Rela, Mohamed</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c2319-ff6eba5c749ae61bb4b457793d089aba26e2fbc6b3156480aa78c88875445e833</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Ammonia</topic><topic>CTLN1</topic><topic>Diagnosis</topic><topic>Diet</topic><topic>Embolization</topic><topic>Encephalopathy</topic><topic>Gene therapy</topic><topic>Liver</topic><topic>Liver diseases</topic><topic>Liver transplantation</topic><topic>Liver transplants</topic><topic>metabolic profile</topic><topic>Metabolism</topic><topic>NCMLD</topic><topic>Portal vein</topic><topic>PVE</topic><topic>Seizures</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vasudevan, Anu K.</creatorcontrib><creatorcontrib>Shanmugam, Naresh P.</creatorcontrib><creatorcontrib>Rammohan, Ashwin</creatorcontrib><creatorcontrib>Rela, Mohamed</creatorcontrib><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><jtitle>Pediatric transplantation</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vasudevan, Anu K.</au><au>Shanmugam, Naresh P.</au><au>Rammohan, Ashwin</au><au>Rela, Mohamed</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Managing recurrent portal steal in auxiliary liver transplantation for non‐cirrhotic metabolic liver disease</atitle><jtitle>Pediatric transplantation</jtitle><date>2022-12</date><risdate>2022</risdate><volume>26</volume><issue>8</issue><epage>n/a</epage><issn>1397-3142</issn><eissn>1399-3046</eissn><abstract>Background
APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and developed recurrent PS.
Case Report
A 2‐year‐old male child with a diagnosis of Citrullinemia type I underwent APOLT with a left lateral segment from a split deceased donor liver, and his postoperative period was unremarkable. Ammonia‐lowering agents were stopped 1 week following the operation and the child was discharged home on a normal diet. Four years following APOLT, the child presented with altered sensorium and seizures. A diagnosis of PS was made. Subsequent to an embolization of the native liver's right anterior portal vein his sensorium improved and he remained clinically stable on a normal diet. Six years following the APOLT, the child again presented with features of acute encephalopathy. Imaging was suggestive of PS. A portal vein embolization of the native portal vein was performed and the child's clinical condition improved. At 6 months' follow‐up, the child remains well on a normal diet.
Conclusions
While the early impediments in this technique may have been overcome, in the absence of any realistic clinical application gene therapy, the debate of long‐term phenotypic metabolic correction for NCMLD by APOLT needs to be revisited.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc</pub><doi>10.1111/petr.14389</doi><tpages>3</tpages><orcidid>https://orcid.org/0000-0001-9528-8892</orcidid><orcidid>https://orcid.org/0000-0003-3342-6326</orcidid></addata></record> |
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| language | eng |
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| source | Wiley |
| subjects | Ammonia CTLN1 Diagnosis Diet Embolization Encephalopathy Gene therapy Liver Liver diseases Liver transplantation Liver transplants metabolic profile Metabolism NCMLD Portal vein PVE Seizures |
| title | Managing recurrent portal steal in auxiliary liver transplantation for non‐cirrhotic metabolic liver disease |
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