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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
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...
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| Published in: | Pediatric nephrology (Berlin, West) West), 2024-11, Vol.39 (11), p.3317-3331 |
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| container_title | Pediatric nephrology (Berlin, West) |
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| creator | 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. |
| description | 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
|
| doi_str_mv | 10.1007/s00467-024-06452-z |
| format | article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_11662369</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>3079955924</sourcerecordid><originalsourceid>FETCH-LOGICAL-c312t-eb57573dfe795a88b4171043d625f51fe20f9897b444511e81ef80cf8b2e31ba3</originalsourceid><addsrcrecordid>eNp9Ustu1DAUjRCIDoUfYIEisWET8DOJVwiNKCBVYgNSd5bjXM-4OPZgJ5WmX8OHsOqXcYcp5bFg5dd53GOdqnpKyUtKSPeqECLariFMNKQVkjXX96oVFZw1VPUX96sVUZw2RNCLk-pRKZeEkF727cPqhCtCqKJ0VX1fBx-9NaG2W5ONnSH7MntbahPH2pmrlM0QoJ4zmHmCONcZyi7FAqVODg92yflw7dJBpMDmAMLdJqQJ8hJSsQFyKh7x-eZbQYPkxwZV0McgMcJumxNa1mUfx4ys2kecxocRhWvjkFF_8WOEPU5hYtkFpJnZp_i4euBMKPDkdj2tPp-9_bR-35x_fPdh_ea8sZyyuYFBdrLjo4NOSdP3g6AdJYKPLZNOUgeMONWrbhBCSEqhp-B6Yl0_MOB0MPy0en3U3S3DBKPFhNkEvct-Mnmvk_H675fot3qTrjSlbct4q1Dhxa1CTl8XKLOefLEQMAqkpWhOOqWkVEwg9Pk_0Mu05Ij5NKek7SUTqkMUO6Is_m3J4O6moUQf2qGP7dDYDv2zHfoaSc_-zHFH-VUHBPAjoOBT3ED-7f0f2R8iVM7M</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>3106852497</pqid></control><display><type>article</type><title>Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation</title><source>Springer Nature</source><creator>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.</creator><creatorcontrib>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.</creatorcontrib><description>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</description><identifier>ISSN: 0931-041X</identifier><identifier>ISSN: 1432-198X</identifier><identifier>EISSN: 1432-198X</identifier><identifier>DOI: 10.1007/s00467-024-06452-z</identifier><identifier>PMID: 39001911</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Adolescent ; Allografts ; Apheresis ; CD20 antigen ; Child ; Clinical outcomes ; CTLA-4 protein ; Data collection ; Electronic medical records ; Female ; Glomerulosclerosis, Focal Segmental - diagnosis ; Glomerulosclerosis, Focal Segmental - drug therapy ; Glomerulosclerosis, Focal Segmental - surgery ; Graft Survival - drug effects ; Humans ; Immunoglobulins ; Immunosuppressive Agents - adverse effects ; Immunosuppressive Agents - therapeutic use ; Kidney diseases ; Kidney transplantation ; Kidney Transplantation - adverse effects ; Kidney transplants ; Low density lipoprotein ; Male ; Medicine ; Medicine & Public Health ; Morbidity ; Nephrology ; Nephrotic syndrome ; Nephrotic Syndrome - diagnosis ; Nephrotic Syndrome - drug therapy ; Nephrotic Syndrome - surgery ; Original Article ; Patients ; Pediatrics ; Phenotypes ; Plasmapheresis ; Recurrence ; Remission ; Remission Induction ; Retrospective Studies ; Risk Factors ; Steroids ; Treatment Outcome ; United States - epidemiology ; Urology ; What’s New in Renal Transplantation</subject><ispartof>Pediatric nephrology (Berlin, West), 2024-11, Vol.39 (11), p.3317-3331</ispartof><rights>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.</rights><rights>2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c312t-eb57573dfe795a88b4171043d625f51fe20f9897b444511e81ef80cf8b2e31ba3</cites><orcidid>0000-0001-8000-1385</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,780,784,885,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/39001911$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Dharnidharka, Vikas R.</creatorcontrib><creatorcontrib>Scobell, Rebecca R.</creatorcontrib><creatorcontrib>Kallash, Mahmoud</creatorcontrib><creatorcontrib>Davies, Amy J. Goodwin</creatorcontrib><creatorcontrib>Marchesani, Nicole</creatorcontrib><creatorcontrib>Maltenfort, Mitchell G.</creatorcontrib><creatorcontrib>Walther, Leslie</creatorcontrib><creatorcontrib>Kelton, Megan</creatorcontrib><creatorcontrib>Bock, Margret</creatorcontrib><creatorcontrib>Blanchette, Eliza</creatorcontrib><creatorcontrib>Stone, Hillarey K.</creatorcontrib><creatorcontrib>Gluck, Caroline</creatorcontrib><creatorcontrib>Hullekes, Frank</creatorcontrib><creatorcontrib>Riella, Leonardo V.</creatorcontrib><creatorcontrib>Smoyer, William E.</creatorcontrib><creatorcontrib>Mitsnefes, Mark</creatorcontrib><creatorcontrib>Dixon, Bradley P.</creatorcontrib><creatorcontrib>Flynn, Joseph T.</creatorcontrib><creatorcontrib>Somers, Michael J. G.</creatorcontrib><creatorcontrib>Forrest, Christopher B.</creatorcontrib><creatorcontrib>Furth, Susan</creatorcontrib><creatorcontrib>Denburg, Michelle R.</creatorcontrib><title>Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation</title><title>Pediatric nephrology (Berlin, West)</title><addtitle>Pediatr Nephrol</addtitle><addtitle>Pediatr Nephrol</addtitle><description>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</description><subject>Adolescent</subject><subject>Allografts</subject><subject>Apheresis</subject><subject>CD20 antigen</subject><subject>Child</subject><subject>Clinical outcomes</subject><subject>CTLA-4 protein</subject><subject>Data collection</subject><subject>Electronic medical records</subject><subject>Female</subject><subject>Glomerulosclerosis, Focal Segmental - diagnosis</subject><subject>Glomerulosclerosis, Focal Segmental - drug therapy</subject><subject>Glomerulosclerosis, Focal Segmental - surgery</subject><subject>Graft Survival - drug effects</subject><subject>Humans</subject><subject>Immunoglobulins</subject><subject>Immunosuppressive Agents - adverse effects</subject><subject>Immunosuppressive Agents - therapeutic use</subject><subject>Kidney diseases</subject><subject>Kidney transplantation</subject><subject>Kidney Transplantation - adverse effects</subject><subject>Kidney transplants</subject><subject>Low density lipoprotein</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Morbidity</subject><subject>Nephrology</subject><subject>Nephrotic syndrome</subject><subject>Nephrotic Syndrome - diagnosis</subject><subject>Nephrotic Syndrome - drug therapy</subject><subject>Nephrotic Syndrome - surgery</subject><subject>Original Article</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Phenotypes</subject><subject>Plasmapheresis</subject><subject>Recurrence</subject><subject>Remission</subject><subject>Remission Induction</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>Steroids</subject><subject>Treatment Outcome</subject><subject>United States - epidemiology</subject><subject>Urology</subject><subject>What’s New in Renal Transplantation</subject><issn>0931-041X</issn><issn>1432-198X</issn><issn>1432-198X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNp9Ustu1DAUjRCIDoUfYIEisWET8DOJVwiNKCBVYgNSd5bjXM-4OPZgJ5WmX8OHsOqXcYcp5bFg5dd53GOdqnpKyUtKSPeqECLariFMNKQVkjXX96oVFZw1VPUX96sVUZw2RNCLk-pRKZeEkF727cPqhCtCqKJ0VX1fBx-9NaG2W5ONnSH7MntbahPH2pmrlM0QoJ4zmHmCONcZyi7FAqVODg92yflw7dJBpMDmAMLdJqQJ8hJSsQFyKh7x-eZbQYPkxwZV0McgMcJumxNa1mUfx4ys2kecxocRhWvjkFF_8WOEPU5hYtkFpJnZp_i4euBMKPDkdj2tPp-9_bR-35x_fPdh_ea8sZyyuYFBdrLjo4NOSdP3g6AdJYKPLZNOUgeMONWrbhBCSEqhp-B6Yl0_MOB0MPy0en3U3S3DBKPFhNkEvct-Mnmvk_H675fot3qTrjSlbct4q1Dhxa1CTl8XKLOefLEQMAqkpWhOOqWkVEwg9Pk_0Mu05Ij5NKek7SUTqkMUO6Is_m3J4O6moUQf2qGP7dDYDv2zHfoaSc_-zHFH-VUHBPAjoOBT3ED-7f0f2R8iVM7M</recordid><startdate>20241101</startdate><enddate>20241101</enddate><creator>Dharnidharka, Vikas R.</creator><creator>Scobell, Rebecca R.</creator><creator>Kallash, Mahmoud</creator><creator>Davies, Amy J. Goodwin</creator><creator>Marchesani, Nicole</creator><creator>Maltenfort, Mitchell G.</creator><creator>Walther, Leslie</creator><creator>Kelton, Megan</creator><creator>Bock, Margret</creator><creator>Blanchette, Eliza</creator><creator>Stone, Hillarey K.</creator><creator>Gluck, Caroline</creator><creator>Hullekes, Frank</creator><creator>Riella, Leonardo V.</creator><creator>Smoyer, William E.</creator><creator>Mitsnefes, Mark</creator><creator>Dixon, Bradley P.</creator><creator>Flynn, Joseph T.</creator><creator>Somers, Michael J. G.</creator><creator>Forrest, Christopher B.</creator><creator>Furth, Susan</creator><creator>Denburg, Michelle R.</creator><general>Springer Berlin Heidelberg</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7QP</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-8000-1385</orcidid></search><sort><creationdate>20241101</creationdate><title>Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation</title><author>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.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c312t-eb57573dfe795a88b4171043d625f51fe20f9897b444511e81ef80cf8b2e31ba3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Adolescent</topic><topic>Allografts</topic><topic>Apheresis</topic><topic>CD20 antigen</topic><topic>Child</topic><topic>Clinical outcomes</topic><topic>CTLA-4 protein</topic><topic>Data collection</topic><topic>Electronic medical records</topic><topic>Female</topic><topic>Glomerulosclerosis, Focal Segmental - diagnosis</topic><topic>Glomerulosclerosis, Focal Segmental - drug therapy</topic><topic>Glomerulosclerosis, Focal Segmental - surgery</topic><topic>Graft Survival - drug effects</topic><topic>Humans</topic><topic>Immunoglobulins</topic><topic>Immunosuppressive Agents - adverse effects</topic><topic>Immunosuppressive Agents - therapeutic use</topic><topic>Kidney diseases</topic><topic>Kidney transplantation</topic><topic>Kidney Transplantation - adverse effects</topic><topic>Kidney transplants</topic><topic>Low density lipoprotein</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Morbidity</topic><topic>Nephrology</topic><topic>Nephrotic syndrome</topic><topic>Nephrotic Syndrome - diagnosis</topic><topic>Nephrotic Syndrome - drug therapy</topic><topic>Nephrotic Syndrome - surgery</topic><topic>Original Article</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Phenotypes</topic><topic>Plasmapheresis</topic><topic>Recurrence</topic><topic>Remission</topic><topic>Remission Induction</topic><topic>Retrospective Studies</topic><topic>Risk Factors</topic><topic>Steroids</topic><topic>Treatment Outcome</topic><topic>United States - epidemiology</topic><topic>Urology</topic><topic>What’s New in Renal Transplantation</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Dharnidharka, Vikas R.</creatorcontrib><creatorcontrib>Scobell, Rebecca R.</creatorcontrib><creatorcontrib>Kallash, Mahmoud</creatorcontrib><creatorcontrib>Davies, Amy J. Goodwin</creatorcontrib><creatorcontrib>Marchesani, Nicole</creatorcontrib><creatorcontrib>Maltenfort, Mitchell G.</creatorcontrib><creatorcontrib>Walther, Leslie</creatorcontrib><creatorcontrib>Kelton, Megan</creatorcontrib><creatorcontrib>Bock, Margret</creatorcontrib><creatorcontrib>Blanchette, Eliza</creatorcontrib><creatorcontrib>Stone, Hillarey K.</creatorcontrib><creatorcontrib>Gluck, Caroline</creatorcontrib><creatorcontrib>Hullekes, Frank</creatorcontrib><creatorcontrib>Riella, Leonardo V.</creatorcontrib><creatorcontrib>Smoyer, William E.</creatorcontrib><creatorcontrib>Mitsnefes, Mark</creatorcontrib><creatorcontrib>Dixon, Bradley P.</creatorcontrib><creatorcontrib>Flynn, Joseph T.</creatorcontrib><creatorcontrib>Somers, Michael J. G.</creatorcontrib><creatorcontrib>Forrest, Christopher B.</creatorcontrib><creatorcontrib>Furth, Susan</creatorcontrib><creatorcontrib>Denburg, Michelle R.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Pediatric nephrology (Berlin, West)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Dharnidharka, Vikas R.</au><au>Scobell, Rebecca R.</au><au>Kallash, Mahmoud</au><au>Davies, Amy J. Goodwin</au><au>Marchesani, Nicole</au><au>Maltenfort, Mitchell G.</au><au>Walther, Leslie</au><au>Kelton, Megan</au><au>Bock, Margret</au><au>Blanchette, Eliza</au><au>Stone, Hillarey K.</au><au>Gluck, Caroline</au><au>Hullekes, Frank</au><au>Riella, Leonardo V.</au><au>Smoyer, William E.</au><au>Mitsnefes, Mark</au><au>Dixon, Bradley P.</au><au>Flynn, Joseph T.</au><au>Somers, Michael J. G.</au><au>Forrest, Christopher B.</au><au>Furth, Susan</au><au>Denburg, Michelle R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation</atitle><jtitle>Pediatric nephrology (Berlin, West)</jtitle><stitle>Pediatr Nephrol</stitle><addtitle>Pediatr Nephrol</addtitle><date>2024-11-01</date><risdate>2024</risdate><volume>39</volume><issue>11</issue><spage>3317</spage><epage>3331</epage><pages>3317-3331</pages><issn>0931-041X</issn><issn>1432-198X</issn><eissn>1432-198X</eissn><abstract>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</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>39001911</pmid><doi>10.1007/s00467-024-06452-z</doi><tpages>15</tpages><orcidid>https://orcid.org/0000-0001-8000-1385</orcidid></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0931-041X |
| ispartof | Pediatric nephrology (Berlin, West), 2024-11, Vol.39 (11), p.3317-3331 |
| issn | 0931-041X 1432-198X 1432-198X |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_11662369 |
| source | Springer Nature |
| subjects | Adolescent Allografts Apheresis CD20 antigen Child Clinical outcomes CTLA-4 protein Data collection Electronic medical records Female Glomerulosclerosis, Focal Segmental - diagnosis Glomerulosclerosis, Focal Segmental - drug therapy Glomerulosclerosis, Focal Segmental - surgery Graft Survival - drug effects Humans Immunoglobulins Immunosuppressive Agents - adverse effects Immunosuppressive Agents - therapeutic use Kidney diseases Kidney transplantation Kidney Transplantation - adverse effects Kidney transplants Low density lipoprotein Male Medicine Medicine & Public Health Morbidity Nephrology Nephrotic syndrome Nephrotic Syndrome - diagnosis Nephrotic Syndrome - drug therapy Nephrotic Syndrome - surgery Original Article Patients Pediatrics Phenotypes Plasmapheresis Recurrence Remission Remission Induction Retrospective Studies Risk Factors Steroids Treatment Outcome United States - epidemiology Urology What’s New in Renal Transplantation |
| title | Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-07T07%3A37%3A43IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Clinical%20characteristics%20and%20favorable%20treatment%20responses%20of%20recurrent%20focal%20segmental%20glomerulosclerosis%20or%C2%A0steroid-resistant%20nephrotic%20syndrome%20in%20children%20after%20kidney%20transplantation&rft.jtitle=Pediatric%20nephrology%20(Berlin,%20West)&rft.au=Dharnidharka,%20Vikas%20R.&rft.date=2024-11-01&rft.volume=39&rft.issue=11&rft.spage=3317&rft.epage=3331&rft.pages=3317-3331&rft.issn=0931-041X&rft.eissn=1432-198X&rft_id=info:doi/10.1007/s00467-024-06452-z&rft_dat=%3Cproquest_pubme%3E3079955924%3C/proquest_pubme%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c312t-eb57573dfe795a88b4171043d625f51fe20f9897b444511e81ef80cf8b2e31ba3%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=3106852497&rft_id=info:pmid/39001911&rfr_iscdi=true |