Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation
Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Me...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Dharnidharka, Vikas R. [verfasserIn] Scobell, Rebecca R. [verfasserIn] Kallash, Mahmoud [verfasserIn] Davies, Amy J. Goodwin [verfasserIn] Marchesani, Nicole [verfasserIn] Maltenfort, Mitchell G. [verfasserIn] Walther, Leslie [verfasserIn] Kelton, Megan [verfasserIn] Bock, Margret [verfasserIn] Blanchette, Eliza [verfasserIn] Stone, Hillarey K. [verfasserIn] Gluck, Caroline [verfasserIn] Hullekes, Frank [verfasserIn] Riella, Leonardo V. [verfasserIn] Smoyer, William E. [verfasserIn] Mitsnefes, Mark [verfasserIn] Dixon, Bradley P. [verfasserIn] Flynn, Joseph T. [verfasserIn] Somers, Michael J. G. [verfasserIn] Forrest, Christopher B. [verfasserIn] Furth, Susan [verfasserIn] Denburg, Michelle R. [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2024 |
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| Schlagwörter: |
Focal segmental glomerulosclerosis |
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| Anmerkung: |
© The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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| Übergeordnetes Werk: |
Enthalten in: Pediatric nephrology - Springer Berlin Heidelberg, 1987, 39(2024), 11 vom: 13. Juli, Seite 3317-3331 |
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| Übergeordnetes Werk: |
volume:39 ; year:2024 ; number:11 ; day:13 ; month:07 ; pages:3317-3331 |
| Links: |
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| DOI / URN: |
10.1007/s00467-024-06452-z |
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SPR057379033 |
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| 520 | |a Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information | ||
| 650 | 4 | |a Focal segmental glomerulosclerosis |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Steroid-resistant nephrotic syndrome |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Kidney transplant |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Pediatrics |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Recurrent disease |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Scobell, Rebecca R. |e verfasserin |4 aut | |
| 700 | 1 | |a Kallash, Mahmoud |e verfasserin |4 aut | |
| 700 | 1 | |a Davies, Amy J. Goodwin |e verfasserin |4 aut | |
| 700 | 1 | |a Marchesani, Nicole |e verfasserin |4 aut | |
| 700 | 1 | |a Maltenfort, Mitchell G. |e verfasserin |4 aut | |
| 700 | 1 | |a Walther, Leslie |e verfasserin |4 aut | |
| 700 | 1 | |a Kelton, Megan |e verfasserin |4 aut | |
| 700 | 1 | |a Bock, Margret |e verfasserin |4 aut | |
| 700 | 1 | |a Blanchette, Eliza |e verfasserin |4 aut | |
| 700 | 1 | |a Stone, Hillarey K. |e verfasserin |4 aut | |
| 700 | 1 | |a Gluck, Caroline |e verfasserin |4 aut | |
| 700 | 1 | |a Hullekes, Frank |e verfasserin |4 aut | |
| 700 | 1 | |a Riella, Leonardo V. |e verfasserin |4 aut | |
| 700 | 1 | |a Smoyer, William E. |e verfasserin |4 aut | |
| 700 | 1 | |a Mitsnefes, Mark |e verfasserin |4 aut | |
| 700 | 1 | |a Dixon, Bradley P. |e verfasserin |4 aut | |
| 700 | 1 | |a Flynn, Joseph T. |e verfasserin |4 aut | |
| 700 | 1 | |a Somers, Michael J. G. |e verfasserin |4 aut | |
| 700 | 1 | |a Forrest, Christopher B. |e verfasserin |4 aut | |
| 700 | 1 | |a Furth, Susan |e verfasserin |4 aut | |
| 700 | 1 | |a Denburg, Michelle R. |e verfasserin |4 aut | |
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10.1007/s00467-024-06452-z doi (DE-627)SPR057379033 (SPR)s00467-024-06452-z-e DE-627 ger DE-627 rakwb eng 610 VZ 44.88 bkl 44.67 bkl Dharnidharka, Vikas R. verfasserin (orcid)0000-0001-8000-1385 aut Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation 2024 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Focal segmental glomerulosclerosis (dpeaa)DE-He213 Steroid-resistant nephrotic syndrome (dpeaa)DE-He213 Kidney transplant (dpeaa)DE-He213 Pediatrics (dpeaa)DE-He213 Recurrent disease (dpeaa)DE-He213 Scobell, Rebecca R. verfasserin aut Kallash, Mahmoud verfasserin aut Davies, Amy J. Goodwin verfasserin aut Marchesani, Nicole verfasserin aut Maltenfort, Mitchell G. verfasserin aut Walther, Leslie verfasserin aut Kelton, Megan verfasserin aut Bock, Margret verfasserin aut Blanchette, Eliza verfasserin aut Stone, Hillarey K. verfasserin aut Gluck, Caroline verfasserin aut Hullekes, Frank verfasserin aut Riella, Leonardo V. verfasserin aut Smoyer, William E. verfasserin aut Mitsnefes, Mark verfasserin aut Dixon, Bradley P. verfasserin aut Flynn, Joseph T. verfasserin aut Somers, Michael J. G. verfasserin aut Forrest, Christopher B. verfasserin aut Furth, Susan verfasserin aut Denburg, Michelle R. verfasserin aut Enthalten in Pediatric nephrology Springer Berlin Heidelberg, 1987 39(2024), 11 vom: 13. Juli, Seite 3317-3331 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 https://dx.doi.org/10.1007/s00467-024-06452-z X:SPRINGER Resolving-System lizenzpflichtig Volltext SYSFLAG_0 GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 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_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 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_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 VZ 44.67 VZ AR 39 2024 11 13 07 3317-3331 |
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10.1007/s00467-024-06452-z doi (DE-627)SPR057379033 (SPR)s00467-024-06452-z-e DE-627 ger DE-627 rakwb eng 610 VZ 44.88 bkl 44.67 bkl Dharnidharka, Vikas R. verfasserin (orcid)0000-0001-8000-1385 aut Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation 2024 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Focal segmental glomerulosclerosis (dpeaa)DE-He213 Steroid-resistant nephrotic syndrome (dpeaa)DE-He213 Kidney transplant (dpeaa)DE-He213 Pediatrics (dpeaa)DE-He213 Recurrent disease (dpeaa)DE-He213 Scobell, Rebecca R. verfasserin aut Kallash, Mahmoud verfasserin aut Davies, Amy J. Goodwin verfasserin aut Marchesani, Nicole verfasserin aut Maltenfort, Mitchell G. verfasserin aut Walther, Leslie verfasserin aut Kelton, Megan verfasserin aut Bock, Margret verfasserin aut Blanchette, Eliza verfasserin aut Stone, Hillarey K. verfasserin aut Gluck, Caroline verfasserin aut Hullekes, Frank verfasserin aut Riella, Leonardo V. verfasserin aut Smoyer, William E. verfasserin aut Mitsnefes, Mark verfasserin aut Dixon, Bradley P. verfasserin aut Flynn, Joseph T. verfasserin aut Somers, Michael J. G. verfasserin aut Forrest, Christopher B. verfasserin aut Furth, Susan verfasserin aut Denburg, Michelle R. verfasserin aut Enthalten in Pediatric nephrology Springer Berlin Heidelberg, 1987 39(2024), 11 vom: 13. Juli, Seite 3317-3331 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 https://dx.doi.org/10.1007/s00467-024-06452-z X:SPRINGER Resolving-System lizenzpflichtig Volltext SYSFLAG_0 GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 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_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 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_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 VZ 44.67 VZ AR 39 2024 11 13 07 3317-3331 |
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10.1007/s00467-024-06452-z doi (DE-627)SPR057379033 (SPR)s00467-024-06452-z-e DE-627 ger DE-627 rakwb eng 610 VZ 44.88 bkl 44.67 bkl Dharnidharka, Vikas R. verfasserin (orcid)0000-0001-8000-1385 aut Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation 2024 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Focal segmental glomerulosclerosis (dpeaa)DE-He213 Steroid-resistant nephrotic syndrome (dpeaa)DE-He213 Kidney transplant (dpeaa)DE-He213 Pediatrics (dpeaa)DE-He213 Recurrent disease (dpeaa)DE-He213 Scobell, Rebecca R. verfasserin aut Kallash, Mahmoud verfasserin aut Davies, Amy J. Goodwin verfasserin aut Marchesani, Nicole verfasserin aut Maltenfort, Mitchell G. verfasserin aut Walther, Leslie verfasserin aut Kelton, Megan verfasserin aut Bock, Margret verfasserin aut Blanchette, Eliza verfasserin aut Stone, Hillarey K. verfasserin aut Gluck, Caroline verfasserin aut Hullekes, Frank verfasserin aut Riella, Leonardo V. verfasserin aut Smoyer, William E. verfasserin aut Mitsnefes, Mark verfasserin aut Dixon, Bradley P. verfasserin aut Flynn, Joseph T. verfasserin aut Somers, Michael J. G. verfasserin aut Forrest, Christopher B. verfasserin aut Furth, Susan verfasserin aut Denburg, Michelle R. verfasserin aut Enthalten in Pediatric nephrology Springer Berlin Heidelberg, 1987 39(2024), 11 vom: 13. Juli, Seite 3317-3331 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 https://dx.doi.org/10.1007/s00467-024-06452-z X:SPRINGER Resolving-System lizenzpflichtig Volltext SYSFLAG_0 GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 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_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 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_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 VZ 44.67 VZ AR 39 2024 11 13 07 3317-3331 |
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10.1007/s00467-024-06452-z doi (DE-627)SPR057379033 (SPR)s00467-024-06452-z-e DE-627 ger DE-627 rakwb eng 610 VZ 44.88 bkl 44.67 bkl Dharnidharka, Vikas R. verfasserin (orcid)0000-0001-8000-1385 aut Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation 2024 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Focal segmental glomerulosclerosis (dpeaa)DE-He213 Steroid-resistant nephrotic syndrome (dpeaa)DE-He213 Kidney transplant (dpeaa)DE-He213 Pediatrics (dpeaa)DE-He213 Recurrent disease (dpeaa)DE-He213 Scobell, Rebecca R. verfasserin aut Kallash, Mahmoud verfasserin aut Davies, Amy J. Goodwin verfasserin aut Marchesani, Nicole verfasserin aut Maltenfort, Mitchell G. verfasserin aut Walther, Leslie verfasserin aut Kelton, Megan verfasserin aut Bock, Margret verfasserin aut Blanchette, Eliza verfasserin aut Stone, Hillarey K. verfasserin aut Gluck, Caroline verfasserin aut Hullekes, Frank verfasserin aut Riella, Leonardo V. verfasserin aut Smoyer, William E. verfasserin aut Mitsnefes, Mark verfasserin aut Dixon, Bradley P. verfasserin aut Flynn, Joseph T. verfasserin aut Somers, Michael J. G. verfasserin aut Forrest, Christopher B. verfasserin aut Furth, Susan verfasserin aut Denburg, Michelle R. verfasserin aut Enthalten in Pediatric nephrology Springer Berlin Heidelberg, 1987 39(2024), 11 vom: 13. Juli, Seite 3317-3331 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 https://dx.doi.org/10.1007/s00467-024-06452-z X:SPRINGER Resolving-System lizenzpflichtig Volltext SYSFLAG_0 GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 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_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 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_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 VZ 44.67 VZ AR 39 2024 11 13 07 3317-3331 |
| allfieldsSound |
10.1007/s00467-024-06452-z doi (DE-627)SPR057379033 (SPR)s00467-024-06452-z-e DE-627 ger DE-627 rakwb eng 610 VZ 44.88 bkl 44.67 bkl Dharnidharka, Vikas R. verfasserin (orcid)0000-0001-8000-1385 aut Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation 2024 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Focal segmental glomerulosclerosis (dpeaa)DE-He213 Steroid-resistant nephrotic syndrome (dpeaa)DE-He213 Kidney transplant (dpeaa)DE-He213 Pediatrics (dpeaa)DE-He213 Recurrent disease (dpeaa)DE-He213 Scobell, Rebecca R. verfasserin aut Kallash, Mahmoud verfasserin aut Davies, Amy J. Goodwin verfasserin aut Marchesani, Nicole verfasserin aut Maltenfort, Mitchell G. verfasserin aut Walther, Leslie verfasserin aut Kelton, Megan verfasserin aut Bock, Margret verfasserin aut Blanchette, Eliza verfasserin aut Stone, Hillarey K. verfasserin aut Gluck, Caroline verfasserin aut Hullekes, Frank verfasserin aut Riella, Leonardo V. verfasserin aut Smoyer, William E. verfasserin aut Mitsnefes, Mark verfasserin aut Dixon, Bradley P. verfasserin aut Flynn, Joseph T. verfasserin aut Somers, Michael J. G. verfasserin aut Forrest, Christopher B. verfasserin aut Furth, Susan verfasserin aut Denburg, Michelle R. verfasserin aut Enthalten in Pediatric nephrology Springer Berlin Heidelberg, 1987 39(2024), 11 vom: 13. Juli, Seite 3317-3331 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 https://dx.doi.org/10.1007/s00467-024-06452-z X:SPRINGER Resolving-System lizenzpflichtig Volltext SYSFLAG_0 GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 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_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 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_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 VZ 44.67 VZ AR 39 2024 11 13 07 3317-3331 |
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English |
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Enthalten in Pediatric nephrology 39(2024), 11 vom: 13. Juli, Seite 3317-3331 volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 |
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Enthalten in Pediatric nephrology 39(2024), 11 vom: 13. Juli, Seite 3317-3331 volume:39 year:2024 number:11 day:13 month:07 pages:3317-3331 |
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Article |
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findex.gbv.de |
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Focal segmental glomerulosclerosis Steroid-resistant nephrotic syndrome Kidney transplant Pediatrics Recurrent disease |
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Dharnidharka, Vikas R. @@aut@@ Scobell, Rebecca R. @@aut@@ Kallash, Mahmoud @@aut@@ Davies, Amy J. Goodwin @@aut@@ Marchesani, Nicole @@aut@@ Maltenfort, Mitchell G. @@aut@@ Walther, Leslie @@aut@@ Kelton, Megan @@aut@@ Bock, Margret @@aut@@ Blanchette, Eliza @@aut@@ Stone, Hillarey K. @@aut@@ Gluck, Caroline @@aut@@ Hullekes, Frank @@aut@@ Riella, Leonardo V. @@aut@@ Smoyer, William E. @@aut@@ Mitsnefes, Mark @@aut@@ Dixon, Bradley P. @@aut@@ Flynn, Joseph T. @@aut@@ Somers, Michael J. G. @@aut@@ Forrest, Christopher B. @@aut@@ Furth, Susan @@aut@@ Denburg, Michelle R. @@aut@@ |
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2024-07-13T00:00:00Z |
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<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000naa a22002652 4500</leader><controlfield tag="001">SPR057379033</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20240920064707.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">240920s2024 xx |||||o 00| ||eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1007/s00467-024-06452-z</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR057379033</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)s00467-024-06452-z-e</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">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="082" ind1="0" ind2="4"><subfield code="a">610</subfield><subfield code="q">VZ</subfield></datafield><datafield tag="084" ind1=" " ind2=" "><subfield code="a">44.88</subfield><subfield code="2">bkl</subfield></datafield><datafield tag="084" ind1=" " ind2=" "><subfield code="a">44.67</subfield><subfield code="2">bkl</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Dharnidharka, Vikas R.</subfield><subfield code="e">verfasserin</subfield><subfield code="0">(orcid)0000-0001-8000-1385</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2024</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">© The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. 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Dharnidharka, Vikas R. Scobell, Rebecca R. Kallash, Mahmoud Davies, Amy J. Goodwin Marchesani, Nicole Maltenfort, Mitchell G. Walther, Leslie Kelton, Megan Bock, Margret Blanchette, Eliza Stone, Hillarey K. Gluck, Caroline Hullekes, Frank Riella, Leonardo V. Smoyer, William E. Mitsnefes, Mark Dixon, Bradley P. Flynn, Joseph T. Somers, Michael J. G. Forrest, Christopher B. Furth, Susan Denburg, Michelle R. |
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clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation |
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Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation |
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Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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Background Recurrence of focal segmental glomerulosclerosis (FSGS) or steroid-resistant nephrotic syndrome (SRNS) after kidney transplant leads to significant morbidity and potentially earlier allograft loss. To date however, reported rates, risk factors and treatment outcomes have varied widely. Methods We applied computational phenotypes to a multicenter aggregation of electronic health records data from 7 large pediatric health systems in the USA, to identify recurrence rates, risk factors, and treatment outcomes. We refined the data collection by chart review. Results From > 7 million patients, we compared children with primary FSGS/SRNS who received a kidney transplant between 2009 and 2020 and who either developed recurrence (n = 67/165; 40.6%) or did not (n = 98/165). Serum albumin level at time of transplant was significantly lower and recipient HLA DR7 presence was significantly higher in the recurrence group. By 36 months post-transplant, complete remission occurred in 58.2% and partial remission in 17.9%. Through 6 years post-transplant, no remission after recurrence was associated with an increased risk of allograft loss over time (p < 0.0001), but any remission showed similar allograft survival and function decline to those with no recurrence. Since treatments were used in non-random fashion, using spline curves and multivariable non-linear analyses, complete + partial remission chance was significantly higher with greater plasmapheresis sessions, CTLA4-Ig doses or LDL-apheresis sessions. Only treatment with anti-CD20, CTLA4-Ig agents, or LDL-apheresis sessions were associated with complete remission. Excluding 25 patients with mutations did not significantly change our results. Conclusions Our contemporary high-risk cohort had higher favorable response rates than most prior reports, from combinations of agents. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information © The Author(s), under exclusive licence to International Pediatric Nephrology Association 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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