Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders
Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters comp...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Posset, Roland - 1986- [verfasserIn] Garbade, Sven - 1971- [verfasserIn] Gleich, Florian [verfasserIn] Scharré, Svenja [verfasserIn] Okun, Jürgen G. - 1968- [verfasserIn] Gropman, Andrea L. [verfasserIn] Nagamani, Sandesh C. S. [verfasserIn] Druck, Ann-Catrin [verfasserIn] Epp, Friederike [verfasserIn] Hoffmann, Georg F. - 1957- [verfasserIn] Kölker, Stefan [verfasserIn] Zielonka, Matthias - 1983- [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
April 2024 |
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| Anmerkung: |
Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 |
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| Umfang: |
Illustrationen 12 |
| Übergeordnetes Werk: |
Enthalten in: Genetics in medicine - Amsterdam : Elsevier, 1998, 26(2024), 4 vom: Apr., Artikel-ID 101039, Seite 1-12 |
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| Übergeordnetes Werk: |
volume:26 ; year:2024 ; number:4 ; month:04 ; elocationid:101039 ; pages:1-12 ; extent:12 |
| Links: |
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| DOI / URN: |
10.1016/j.gim.2023.101039 |
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| Katalog-ID: |
1897187726 |
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| 245 | 1 | 0 | |a Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders |c Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group |
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| 520 | |a Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. | ||
| 650 | 4 | |a Argininosuccinic aciduria | |
| 650 | 4 | |a Citrullinemia type 1 | |
| 650 | 4 | |a Liver transplantation | |
| 650 | 4 | |a Ornithine transcarbamylase deficiency | |
| 650 | 4 | |a Urea cycle disorders | |
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| 700 | 1 | |a Gropman, Andrea L. |e verfasserin |4 aut | |
| 700 | 1 | |a Nagamani, Sandesh C. S. |e verfasserin |4 aut | |
| 700 | 1 | |a Druck, Ann-Catrin |e verfasserin |4 aut | |
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10.1016/j.gim.2023.101039 doi (DE-627)1897187726 (DE-599)KXP1897187726 DE-627 ger DE-627 rda eng Posset, Roland 1986- verfasserin (DE-588)1058087347 (DE-627)796372160 (DE-576)414059034 aut Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group April 2024 Illustrationen 12 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Argininosuccinic aciduria Citrullinemia type 1 Liver transplantation Ornithine transcarbamylase deficiency Urea cycle disorders Garbade, Sven 1971- verfasserin (DE-588)129234362 (DE-627)707186889 (DE-576)297554263 aut Gleich, Florian verfasserin (DE-588)1114740195 (DE-627)86900445X (DE-576)47746100X aut Scharré, Svenja verfasserin (DE-588)1322650187 (DE-627)1882604911 aut Okun, Jürgen G. 1968- verfasserin (DE-588)121578232 (DE-627)705551415 (DE-576)292781296 aut Gropman, Andrea L. verfasserin aut Nagamani, Sandesh C. S. verfasserin aut Druck, Ann-Catrin verfasserin aut Epp, Friederike verfasserin (DE-588)1322650551 (DE-627)188260668X aut Hoffmann, Georg F. 1957- verfasserin (DE-588)115652868 (DE-627)077386116 (DE-576)261230042 aut Kölker, Stefan verfasserin (DE-588)1022937758 (DE-627)717335771 (DE-576)366197568 aut Zielonka, Matthias 1983- verfasserin (DE-588)1056891297 (DE-627)79418670X (DE-576)412871637 aut Enthalten in Genetics in medicine Amsterdam : Elsevier, 1998 26(2024), 4 vom: Apr., Artikel-ID 101039, Seite 1-12 Online-Ressource (DE-627)338073361 (DE-600)2063504-7 (DE-576)109132475 1530-0366 nnns volume:26 year:2024 number:4 month:04 elocationid:101039 pages:1-12 extent:12 https://doi.org/10.1016/j.gim.2023.101039 Verlag Resolving-System kostenfrei Volltext https://www.sciencedirect.com/science/article/pii/S1098360023010559 Verlag kostenfrei Volltext GBV_USEFLAG_U GBV_ILN_2013 ISIL_DE-16-250 SYSFLAG_1 GBV_KXP GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 GBV_ILN_2403 GBV_ILN_2403 ISIL_DE-LFER AR 26 2024 4 4 101039 1-12 12 2013 01 DE-16-250 4560976023 00 --%%-- --%%-- --%%-- --%%-- l01 31-07-24 2403 01 DE-LFER 456713155X 00 --%%-- --%%-- n --%%-- l01 16-08-24 2403 01 DE-LFER https://doi.org/10.1016/j.gim.2023.101039 2403 01 DE-LFER https://www.sciencedirect.com/science/article/pii/S1098360023010559 2013 01 DE-16-250 00 s hd2024 2013 01 DE-16-250 01 s (DE-627)1410508463 wissenschaftlicher Artikel (Zeitschrift) 2013 01 DE-16-250 02 s per_12 2013 01 DE-16-250 03 s s_12 2013 01 DE-16-250 04 p (DE-627)1493265547 Posset, Roland 2013 01 DE-16-250 04 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 04 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 04 s pos_1 2013 01 DE-16-250 05 p (DE-627)1529261783 Garbade, Sven 2013 01 DE-16-250 05 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 05 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 05 s pos_2 2013 01 DE-16-250 06 p (DE-627)1547461748 Gleich, Florian 2013 01 DE-16-250 06 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 06 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 06 s pos_3 2013 01 DE-16-250 07 p (DE-627)1882604946 Scharré, Svenja 2013 01 DE-16-250 07 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 07 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 07 s pos_4 2013 01 DE-16-250 08 p (DE-627)148007991X Okun, Jürgen G. 2013 01 DE-16-250 08 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 08 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 08 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 08 s pos_5 2013 01 DE-16-250 09 p (DE-627)1882611136 Epp, Friederike 2013 01 DE-16-250 09 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 09 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 09 s pos_9 2013 01 DE-16-250 10 p (DE-627)1436198674 Hoffmann, Georg F. 2013 01 DE-16-250 10 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 10 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 10 s pos_10 2013 01 DE-16-250 11 p (DE-627)1436198933 Kölker, Stefan 2013 01 DE-16-250 11 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 11 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 11 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 11 s pos_11 2013 01 DE-16-250 12 p (DE-627)1493468650 Zielonka, Matthias 2013 01 DE-16-250 12 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 12 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 12 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 12 s pos_12 |
| spelling |
10.1016/j.gim.2023.101039 doi (DE-627)1897187726 (DE-599)KXP1897187726 DE-627 ger DE-627 rda eng Posset, Roland 1986- verfasserin (DE-588)1058087347 (DE-627)796372160 (DE-576)414059034 aut Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group April 2024 Illustrationen 12 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Argininosuccinic aciduria Citrullinemia type 1 Liver transplantation Ornithine transcarbamylase deficiency Urea cycle disorders Garbade, Sven 1971- verfasserin (DE-588)129234362 (DE-627)707186889 (DE-576)297554263 aut Gleich, Florian verfasserin (DE-588)1114740195 (DE-627)86900445X (DE-576)47746100X aut Scharré, Svenja verfasserin (DE-588)1322650187 (DE-627)1882604911 aut Okun, Jürgen G. 1968- verfasserin (DE-588)121578232 (DE-627)705551415 (DE-576)292781296 aut Gropman, Andrea L. verfasserin aut Nagamani, Sandesh C. S. verfasserin aut Druck, Ann-Catrin verfasserin aut Epp, Friederike verfasserin (DE-588)1322650551 (DE-627)188260668X aut Hoffmann, Georg F. 1957- verfasserin (DE-588)115652868 (DE-627)077386116 (DE-576)261230042 aut Kölker, Stefan verfasserin (DE-588)1022937758 (DE-627)717335771 (DE-576)366197568 aut Zielonka, Matthias 1983- verfasserin (DE-588)1056891297 (DE-627)79418670X (DE-576)412871637 aut Enthalten in Genetics in medicine Amsterdam : Elsevier, 1998 26(2024), 4 vom: Apr., Artikel-ID 101039, Seite 1-12 Online-Ressource (DE-627)338073361 (DE-600)2063504-7 (DE-576)109132475 1530-0366 nnns volume:26 year:2024 number:4 month:04 elocationid:101039 pages:1-12 extent:12 https://doi.org/10.1016/j.gim.2023.101039 Verlag Resolving-System kostenfrei Volltext https://www.sciencedirect.com/science/article/pii/S1098360023010559 Verlag kostenfrei Volltext GBV_USEFLAG_U GBV_ILN_2013 ISIL_DE-16-250 SYSFLAG_1 GBV_KXP GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 GBV_ILN_2403 GBV_ILN_2403 ISIL_DE-LFER AR 26 2024 4 4 101039 1-12 12 2013 01 DE-16-250 4560976023 00 --%%-- --%%-- --%%-- --%%-- l01 31-07-24 2403 01 DE-LFER 456713155X 00 --%%-- --%%-- n --%%-- l01 16-08-24 2403 01 DE-LFER https://doi.org/10.1016/j.gim.2023.101039 2403 01 DE-LFER https://www.sciencedirect.com/science/article/pii/S1098360023010559 2013 01 DE-16-250 00 s hd2024 2013 01 DE-16-250 01 s (DE-627)1410508463 wissenschaftlicher Artikel (Zeitschrift) 2013 01 DE-16-250 02 s per_12 2013 01 DE-16-250 03 s s_12 2013 01 DE-16-250 04 p (DE-627)1493265547 Posset, Roland 2013 01 DE-16-250 04 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 04 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 04 s pos_1 2013 01 DE-16-250 05 p (DE-627)1529261783 Garbade, Sven 2013 01 DE-16-250 05 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 05 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 05 s pos_2 2013 01 DE-16-250 06 p (DE-627)1547461748 Gleich, Florian 2013 01 DE-16-250 06 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 06 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 06 s pos_3 2013 01 DE-16-250 07 p (DE-627)1882604946 Scharré, Svenja 2013 01 DE-16-250 07 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 07 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 07 s pos_4 2013 01 DE-16-250 08 p (DE-627)148007991X Okun, Jürgen G. 2013 01 DE-16-250 08 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 08 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 08 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 08 s pos_5 2013 01 DE-16-250 09 p (DE-627)1882611136 Epp, Friederike 2013 01 DE-16-250 09 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 09 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 09 s pos_9 2013 01 DE-16-250 10 p (DE-627)1436198674 Hoffmann, Georg F. 2013 01 DE-16-250 10 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 10 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 10 s pos_10 2013 01 DE-16-250 11 p (DE-627)1436198933 Kölker, Stefan 2013 01 DE-16-250 11 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 11 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 11 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 11 s pos_11 2013 01 DE-16-250 12 p (DE-627)1493468650 Zielonka, Matthias 2013 01 DE-16-250 12 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 12 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 12 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 12 s pos_12 |
| allfields_unstemmed |
10.1016/j.gim.2023.101039 doi (DE-627)1897187726 (DE-599)KXP1897187726 DE-627 ger DE-627 rda eng Posset, Roland 1986- verfasserin (DE-588)1058087347 (DE-627)796372160 (DE-576)414059034 aut Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group April 2024 Illustrationen 12 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Argininosuccinic aciduria Citrullinemia type 1 Liver transplantation Ornithine transcarbamylase deficiency Urea cycle disorders Garbade, Sven 1971- verfasserin (DE-588)129234362 (DE-627)707186889 (DE-576)297554263 aut Gleich, Florian verfasserin (DE-588)1114740195 (DE-627)86900445X (DE-576)47746100X aut Scharré, Svenja verfasserin (DE-588)1322650187 (DE-627)1882604911 aut Okun, Jürgen G. 1968- verfasserin (DE-588)121578232 (DE-627)705551415 (DE-576)292781296 aut Gropman, Andrea L. verfasserin aut Nagamani, Sandesh C. S. verfasserin aut Druck, Ann-Catrin verfasserin aut Epp, Friederike verfasserin (DE-588)1322650551 (DE-627)188260668X aut Hoffmann, Georg F. 1957- verfasserin (DE-588)115652868 (DE-627)077386116 (DE-576)261230042 aut Kölker, Stefan verfasserin (DE-588)1022937758 (DE-627)717335771 (DE-576)366197568 aut Zielonka, Matthias 1983- verfasserin (DE-588)1056891297 (DE-627)79418670X (DE-576)412871637 aut Enthalten in Genetics in medicine Amsterdam : Elsevier, 1998 26(2024), 4 vom: Apr., Artikel-ID 101039, Seite 1-12 Online-Ressource (DE-627)338073361 (DE-600)2063504-7 (DE-576)109132475 1530-0366 nnns volume:26 year:2024 number:4 month:04 elocationid:101039 pages:1-12 extent:12 https://doi.org/10.1016/j.gim.2023.101039 Verlag Resolving-System kostenfrei Volltext https://www.sciencedirect.com/science/article/pii/S1098360023010559 Verlag kostenfrei Volltext GBV_USEFLAG_U GBV_ILN_2013 ISIL_DE-16-250 SYSFLAG_1 GBV_KXP GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 GBV_ILN_2403 GBV_ILN_2403 ISIL_DE-LFER AR 26 2024 4 4 101039 1-12 12 2013 01 DE-16-250 4560976023 00 --%%-- --%%-- --%%-- --%%-- l01 31-07-24 2403 01 DE-LFER 456713155X 00 --%%-- --%%-- n --%%-- l01 16-08-24 2403 01 DE-LFER https://doi.org/10.1016/j.gim.2023.101039 2403 01 DE-LFER https://www.sciencedirect.com/science/article/pii/S1098360023010559 2013 01 DE-16-250 00 s hd2024 2013 01 DE-16-250 01 s (DE-627)1410508463 wissenschaftlicher Artikel (Zeitschrift) 2013 01 DE-16-250 02 s per_12 2013 01 DE-16-250 03 s s_12 2013 01 DE-16-250 04 p (DE-627)1493265547 Posset, Roland 2013 01 DE-16-250 04 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 04 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 04 s pos_1 2013 01 DE-16-250 05 p (DE-627)1529261783 Garbade, Sven 2013 01 DE-16-250 05 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 05 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 05 s pos_2 2013 01 DE-16-250 06 p (DE-627)1547461748 Gleich, Florian 2013 01 DE-16-250 06 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 06 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 06 s pos_3 2013 01 DE-16-250 07 p (DE-627)1882604946 Scharré, Svenja 2013 01 DE-16-250 07 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 07 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 07 s pos_4 2013 01 DE-16-250 08 p (DE-627)148007991X Okun, Jürgen G. 2013 01 DE-16-250 08 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 08 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 08 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 08 s pos_5 2013 01 DE-16-250 09 p (DE-627)1882611136 Epp, Friederike 2013 01 DE-16-250 09 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 09 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 09 s pos_9 2013 01 DE-16-250 10 p (DE-627)1436198674 Hoffmann, Georg F. 2013 01 DE-16-250 10 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 10 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 10 s pos_10 2013 01 DE-16-250 11 p (DE-627)1436198933 Kölker, Stefan 2013 01 DE-16-250 11 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 11 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 11 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 11 s pos_11 2013 01 DE-16-250 12 p (DE-627)1493468650 Zielonka, Matthias 2013 01 DE-16-250 12 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 12 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 12 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 12 s pos_12 |
| allfieldsGer |
10.1016/j.gim.2023.101039 doi (DE-627)1897187726 (DE-599)KXP1897187726 DE-627 ger DE-627 rda eng Posset, Roland 1986- verfasserin (DE-588)1058087347 (DE-627)796372160 (DE-576)414059034 aut Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group April 2024 Illustrationen 12 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Argininosuccinic aciduria Citrullinemia type 1 Liver transplantation Ornithine transcarbamylase deficiency Urea cycle disorders Garbade, Sven 1971- verfasserin (DE-588)129234362 (DE-627)707186889 (DE-576)297554263 aut Gleich, Florian verfasserin (DE-588)1114740195 (DE-627)86900445X (DE-576)47746100X aut Scharré, Svenja verfasserin (DE-588)1322650187 (DE-627)1882604911 aut Okun, Jürgen G. 1968- verfasserin (DE-588)121578232 (DE-627)705551415 (DE-576)292781296 aut Gropman, Andrea L. verfasserin aut Nagamani, Sandesh C. S. verfasserin aut Druck, Ann-Catrin verfasserin aut Epp, Friederike verfasserin (DE-588)1322650551 (DE-627)188260668X aut Hoffmann, Georg F. 1957- verfasserin (DE-588)115652868 (DE-627)077386116 (DE-576)261230042 aut Kölker, Stefan verfasserin (DE-588)1022937758 (DE-627)717335771 (DE-576)366197568 aut Zielonka, Matthias 1983- verfasserin (DE-588)1056891297 (DE-627)79418670X (DE-576)412871637 aut Enthalten in Genetics in medicine Amsterdam : Elsevier, 1998 26(2024), 4 vom: Apr., Artikel-ID 101039, Seite 1-12 Online-Ressource (DE-627)338073361 (DE-600)2063504-7 (DE-576)109132475 1530-0366 nnns volume:26 year:2024 number:4 month:04 elocationid:101039 pages:1-12 extent:12 https://doi.org/10.1016/j.gim.2023.101039 Verlag Resolving-System kostenfrei Volltext https://www.sciencedirect.com/science/article/pii/S1098360023010559 Verlag kostenfrei Volltext GBV_USEFLAG_U GBV_ILN_2013 ISIL_DE-16-250 SYSFLAG_1 GBV_KXP GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 GBV_ILN_2403 GBV_ILN_2403 ISIL_DE-LFER AR 26 2024 4 4 101039 1-12 12 2013 01 DE-16-250 4560976023 00 --%%-- --%%-- --%%-- --%%-- l01 31-07-24 2403 01 DE-LFER 456713155X 00 --%%-- --%%-- n --%%-- l01 16-08-24 2403 01 DE-LFER https://doi.org/10.1016/j.gim.2023.101039 2403 01 DE-LFER https://www.sciencedirect.com/science/article/pii/S1098360023010559 2013 01 DE-16-250 00 s hd2024 2013 01 DE-16-250 01 s (DE-627)1410508463 wissenschaftlicher Artikel (Zeitschrift) 2013 01 DE-16-250 02 s per_12 2013 01 DE-16-250 03 s s_12 2013 01 DE-16-250 04 p (DE-627)1493265547 Posset, Roland 2013 01 DE-16-250 04 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 04 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 04 s pos_1 2013 01 DE-16-250 05 p (DE-627)1529261783 Garbade, Sven 2013 01 DE-16-250 05 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 05 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 05 s pos_2 2013 01 DE-16-250 06 p (DE-627)1547461748 Gleich, Florian 2013 01 DE-16-250 06 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 06 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 06 s pos_3 2013 01 DE-16-250 07 p (DE-627)1882604946 Scharré, Svenja 2013 01 DE-16-250 07 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 07 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 07 s pos_4 2013 01 DE-16-250 08 p (DE-627)148007991X Okun, Jürgen G. 2013 01 DE-16-250 08 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 08 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 08 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 08 s pos_5 2013 01 DE-16-250 09 p (DE-627)1882611136 Epp, Friederike 2013 01 DE-16-250 09 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 09 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 09 s pos_9 2013 01 DE-16-250 10 p (DE-627)1436198674 Hoffmann, Georg F. 2013 01 DE-16-250 10 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 10 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 10 s pos_10 2013 01 DE-16-250 11 p (DE-627)1436198933 Kölker, Stefan 2013 01 DE-16-250 11 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 11 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 11 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 11 s pos_11 2013 01 DE-16-250 12 p (DE-627)1493468650 Zielonka, Matthias 2013 01 DE-16-250 12 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 12 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 12 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 12 s pos_12 |
| allfieldsSound |
10.1016/j.gim.2023.101039 doi (DE-627)1897187726 (DE-599)KXP1897187726 DE-627 ger DE-627 rda eng Posset, Roland 1986- verfasserin (DE-588)1058087347 (DE-627)796372160 (DE-576)414059034 aut Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group April 2024 Illustrationen 12 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Argininosuccinic aciduria Citrullinemia type 1 Liver transplantation Ornithine transcarbamylase deficiency Urea cycle disorders Garbade, Sven 1971- verfasserin (DE-588)129234362 (DE-627)707186889 (DE-576)297554263 aut Gleich, Florian verfasserin (DE-588)1114740195 (DE-627)86900445X (DE-576)47746100X aut Scharré, Svenja verfasserin (DE-588)1322650187 (DE-627)1882604911 aut Okun, Jürgen G. 1968- verfasserin (DE-588)121578232 (DE-627)705551415 (DE-576)292781296 aut Gropman, Andrea L. verfasserin aut Nagamani, Sandesh C. S. verfasserin aut Druck, Ann-Catrin verfasserin aut Epp, Friederike verfasserin (DE-588)1322650551 (DE-627)188260668X aut Hoffmann, Georg F. 1957- verfasserin (DE-588)115652868 (DE-627)077386116 (DE-576)261230042 aut Kölker, Stefan verfasserin (DE-588)1022937758 (DE-627)717335771 (DE-576)366197568 aut Zielonka, Matthias 1983- verfasserin (DE-588)1056891297 (DE-627)79418670X (DE-576)412871637 aut Enthalten in Genetics in medicine Amsterdam : Elsevier, 1998 26(2024), 4 vom: Apr., Artikel-ID 101039, Seite 1-12 Online-Ressource (DE-627)338073361 (DE-600)2063504-7 (DE-576)109132475 1530-0366 nnns volume:26 year:2024 number:4 month:04 elocationid:101039 pages:1-12 extent:12 https://doi.org/10.1016/j.gim.2023.101039 Verlag Resolving-System kostenfrei Volltext https://www.sciencedirect.com/science/article/pii/S1098360023010559 Verlag kostenfrei Volltext GBV_USEFLAG_U GBV_ILN_2013 ISIL_DE-16-250 SYSFLAG_1 GBV_KXP GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 GBV_ILN_2403 GBV_ILN_2403 ISIL_DE-LFER AR 26 2024 4 4 101039 1-12 12 2013 01 DE-16-250 4560976023 00 --%%-- --%%-- --%%-- --%%-- l01 31-07-24 2403 01 DE-LFER 456713155X 00 --%%-- --%%-- n --%%-- l01 16-08-24 2403 01 DE-LFER https://doi.org/10.1016/j.gim.2023.101039 2403 01 DE-LFER https://www.sciencedirect.com/science/article/pii/S1098360023010559 2013 01 DE-16-250 00 s hd2024 2013 01 DE-16-250 01 s (DE-627)1410508463 wissenschaftlicher Artikel (Zeitschrift) 2013 01 DE-16-250 02 s per_12 2013 01 DE-16-250 03 s s_12 2013 01 DE-16-250 04 p (DE-627)1493265547 Posset, Roland 2013 01 DE-16-250 04 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 04 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 04 s pos_1 2013 01 DE-16-250 05 p (DE-627)1529261783 Garbade, Sven 2013 01 DE-16-250 05 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 05 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 05 s pos_2 2013 01 DE-16-250 06 p (DE-627)1547461748 Gleich, Florian 2013 01 DE-16-250 06 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 06 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 06 s pos_3 2013 01 DE-16-250 07 p (DE-627)1882604946 Scharré, Svenja 2013 01 DE-16-250 07 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 07 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 07 s pos_4 2013 01 DE-16-250 08 p (DE-627)148007991X Okun, Jürgen G. 2013 01 DE-16-250 08 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 08 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 08 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 08 s pos_5 2013 01 DE-16-250 09 p (DE-627)1882611136 Epp, Friederike 2013 01 DE-16-250 09 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 09 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 09 s pos_9 2013 01 DE-16-250 10 p (DE-627)1436198674 Hoffmann, Georg F. 2013 01 DE-16-250 10 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 10 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 10 s pos_10 2013 01 DE-16-250 11 p (DE-627)1436198933 Kölker, Stefan 2013 01 DE-16-250 11 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 11 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 11 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 11 s pos_11 2013 01 DE-16-250 12 p (DE-627)1493468650 Zielonka, Matthias 2013 01 DE-16-250 12 k (DE-627)1416740988 Zentrum für Kinder- und Jugendmedizin 2013 01 DE-16-250 12 k (DE-627)1416466967 Medizinische Fakultät Heidelberg 2013 01 DE-16-250 12 s (DE-627)1410501914 Verfasser 2013 01 DE-16-250 12 s pos_12 |
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Argininosuccinic aciduria Citrullinemia type 1 Liver transplantation Ornithine transcarbamylase deficiency Urea cycle disorders |
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Posset, Roland @@aut@@ Garbade, Sven @@aut@@ Gleich, Florian @@aut@@ Scharré, Svenja @@aut@@ Okun, Jürgen G. @@aut@@ Gropman, Andrea L. @@aut@@ Nagamani, Sandesh C. S. @@aut@@ Druck, Ann-Catrin @@aut@@ Epp, Friederike @@aut@@ Hoffmann, Georg F. @@aut@@ Kölker, Stefan @@aut@@ Zielonka, Matthias @@aut@@ |
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Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">April 2024</subfield></datafield><datafield tag="300" ind1=" " ind2=" "><subfield code="b">Illustrationen</subfield></datafield><datafield tag="300" ind1=" " ind2=" "><subfield code="a">12</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">Gesehen am 31.07.2024</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Argininosuccinic aciduria</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Citrullinemia type 1</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Liver transplantation</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Ornithine transcarbamylase deficiency</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Urea cycle disorders</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Garbade, Sven</subfield><subfield code="d">1971-</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)129234362</subfield><subfield code="0">(DE-627)707186889</subfield><subfield code="0">(DE-576)297554263</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Gleich, Florian</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)1114740195</subfield><subfield code="0">(DE-627)86900445X</subfield><subfield code="0">(DE-576)47746100X</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Scharré, Svenja</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)1322650187</subfield><subfield code="0">(DE-627)1882604911</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Okun, Jürgen G.</subfield><subfield code="d">1968-</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)121578232</subfield><subfield code="0">(DE-627)705551415</subfield><subfield code="0">(DE-576)292781296</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Gropman, Andrea L.</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Nagamani, Sandesh C. 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Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group |
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severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders |
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Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders |
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Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 |
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Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 |
| abstract_unstemmed |
Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders. Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024 Gesehen am 31.07.2024 |
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<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a2200265 4500</leader><controlfield tag="001">1897187726</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20240806110650.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">240731s2024 xx |||||o 00| ||eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1016/j.gim.2023.101039</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)1897187726</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-599)KXP1897187726</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rda</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Posset, Roland</subfield><subfield code="d">1986-</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)1058087347</subfield><subfield code="0">(DE-627)796372160</subfield><subfield code="0">(DE-576)414059034</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Severity-adjusted evaluation of liver transplantation on health outcomes in urea cycle disorders</subfield><subfield code="c">Roland Posset, Sven F. Garbade, Florian Gleich, Svenja Scharre, Jürgen G. Okun, Andrea L. Gropman, Sandesh C. S. Nagamani, Ann-Catrin Druck, Friederike Epp, Georg F. Hoffmann, Stefan Kölker, Matthias Zielonka, on behalf of the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) Consortia Study Group</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">April 2024</subfield></datafield><datafield tag="300" ind1=" " ind2=" "><subfield code="b">Illustrationen</subfield></datafield><datafield tag="300" ind1=" " ind2=" "><subfield code="a">12</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">Online verfügbar: 3. Dezember 2023, Artikelversion: 13. Februar 2024</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">Gesehen am 31.07.2024</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Purpose - Liver transplantation (LTx) is performed in individuals with urea cycle disorders when medical management (MM) insufficiently prevents the occurrence of hyperammonemic events. However, there is a paucity of systematic analyses on the effects of LTx on health-related outcome parameters compared to individuals with comparable severity who are medically managed. - Methods - We investigated the effects of LTx and MM on validated health-related outcome parameters, including the metabolic disease course, linear growth, and neurocognitive outcomes. Individuals were stratified into “severe” and “attenuated” categories based on the genotype-specific and validated in vitro enzyme activity. - Results - LTx enabled metabolic stability by prevention of further hyperammonemic events after transplantation and was associated with a more favorable growth outcome compared with individuals remaining under MM. However, neurocognitive outcome in individuals with LTx did not differ from the medically managed counterparts as reflected by the frequency of motor abnormality and cognitive standard deviation score at last observation. - Conclusion - Whereas LTx enabled metabolic stability without further need of protein restriction or nitrogen-scavenging therapy and was associated with a more favorable growth outcome, LTx - as currently performed - was not associated with improved neurocognitive outcomes compared with long-term MM in the investigated urea cycle disorders.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Argininosuccinic aciduria</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Citrullinemia type 1</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Liver transplantation</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Ornithine transcarbamylase deficiency</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Urea cycle disorders</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Garbade, Sven</subfield><subfield code="d">1971-</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)129234362</subfield><subfield code="0">(DE-627)707186889</subfield><subfield code="0">(DE-576)297554263</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Gleich, Florian</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)1114740195</subfield><subfield code="0">(DE-627)86900445X</subfield><subfield code="0">(DE-576)47746100X</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Scharré, Svenja</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)1322650187</subfield><subfield code="0">(DE-627)1882604911</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Okun, Jürgen G.</subfield><subfield code="d">1968-</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(DE-588)121578232</subfield><subfield code="0">(DE-627)705551415</subfield><subfield code="0">(DE-576)292781296</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Gropman, Andrea L.</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Nagamani, Sandesh C. 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