Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression
Background The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade o...
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| Veröffentlicht in: | Pediatric nephrology (Berlin, West) West), 2023-05, Vol.38 (5), p.1499-1511 |
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| creator | Trautmann, Agnes Seide, Svenja Lipska-Ziętkiewicz, Beata S. Ozaltin, Fatih Szczepanska, Maria Azocar, Marta Jankauskiene, Augustina Zurowska, Alexandra Caliskan, Salim Saeed, Bassam Morello, William Emma, Francesco Litwin, Mieczyslaw Tsygin, Alexey Fomina, Svitlana Wasilewska, Anna Melk, Anette Benetti, Elisa Gellermann, Jutta Stajic, Natasa Tkaczyk, Marcin Baiko, Sergey Prikhodina, Larisa Csaicsich, Dagmar Medynska, Anna Krisam, Regina Breitschwerdt, Heike Schaefer, Franz |
| description | Background
The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction.
Methods
Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission.
Results
Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (
n
= 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin–angiotensin–aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS.
Conclusion
Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS.
Graphical abstract
A higher resolution version of the Graphical abstract is available as
Supplementary information |
| doi_str_mv | 10.1007/s00467-022-05762-4 |
| format | Article |
| fullrecord | <record><control><sourceid>gale_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_10060323</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A743619710</galeid><sourcerecordid>A743619710</sourcerecordid><originalsourceid>FETCH-LOGICAL-c611t-45f72ced77b56d4b7b81ba4b29707eb2bbbe254567216e329659e7d2799aa2d3</originalsourceid><addsrcrecordid>eNp9kk9rFTEUxQdR7LP6BVzIgCDdpObfJG9WUopVodBNF92FZHLnTepMMiYZpd_ePF9t--QhWQTu_Z2T5ORW1VuCTwnG8mPCmAuJMKUIN1JQxJ9VK8IZRaRd3zyvVrhlBGFObo6qVyndYozXzVq8rI6YYKShkq2q71dL7sIEqQ59nTLE4CyKkFzK2ufawzzEkF1XpztvYwFr5-tucKON4Gsfcp0j6Ay2_uXyUJoZfHK9KwU3TYsPaZnn4pdc8K-rF70eE7y534-r64vP1-df0eXVl2_nZ5eoE4RkxJte0g6slKYRlhtp1sRobmgrsQRDjTFAG94ISYkARlvRtCAtlW2rNbXsuPq0s50XM4HtwOeoRzVHN-l4p4J2ar_j3aA24acqoQrMKCsOJ_cOMfxYIGU1udTBOGoPYUmqJIcFL1Higr7_B70NS_TleYVqKZGcCvlIbfQIyvk-lIO7rak6k5wJ0kqy9UIHqA14KLcMHnpXynv86QG-LAuT6w4KPjwRDKDHPKQwLrl8TtoH6Q7sYkgpQv-QHsHbmKTazZ4qs6f-zJ7iRfTuae4Pkr_DVgC2A1Jp-Q3Ex6z-Y_sb1Afk_w</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2792174267</pqid></control><display><type>article</type><title>Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression</title><source>MEDLINE</source><source>Springer Nature - Complete Springer Journals</source><creator>Trautmann, Agnes ; Seide, Svenja ; Lipska-Ziętkiewicz, Beata S. ; Ozaltin, Fatih ; Szczepanska, Maria ; Azocar, Marta ; Jankauskiene, Augustina ; Zurowska, Alexandra ; Caliskan, Salim ; Saeed, Bassam ; Morello, William ; Emma, Francesco ; Litwin, Mieczyslaw ; Tsygin, Alexey ; Fomina, Svitlana ; Wasilewska, Anna ; Melk, Anette ; Benetti, Elisa ; Gellermann, Jutta ; Stajic, Natasa ; Tkaczyk, Marcin ; Baiko, Sergey ; Prikhodina, Larisa ; Csaicsich, Dagmar ; Medynska, Anna ; Krisam, Regina ; Breitschwerdt, Heike ; Schaefer, Franz</creator><creatorcontrib>Trautmann, Agnes ; Seide, Svenja ; Lipska-Ziętkiewicz, Beata S. ; Ozaltin, Fatih ; Szczepanska, Maria ; Azocar, Marta ; Jankauskiene, Augustina ; Zurowska, Alexandra ; Caliskan, Salim ; Saeed, Bassam ; Morello, William ; Emma, Francesco ; Litwin, Mieczyslaw ; Tsygin, Alexey ; Fomina, Svitlana ; Wasilewska, Anna ; Melk, Anette ; Benetti, Elisa ; Gellermann, Jutta ; Stajic, Natasa ; Tkaczyk, Marcin ; Baiko, Sergey ; Prikhodina, Larisa ; Csaicsich, Dagmar ; Medynska, Anna ; Krisam, Regina ; Breitschwerdt, Heike ; Schaefer, Franz ; PodoNet Consortium ; for the PodoNet Consortium</creatorcontrib><description>Background
The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction.
Methods
Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission.
Results
Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (
n
= 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin–angiotensin–aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS.
Conclusion
Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS.
Graphical abstract
A higher resolution version of the Graphical abstract is available as
Supplementary information</description><identifier>ISSN: 0931-041X</identifier><identifier>EISSN: 1432-198X</identifier><identifier>DOI: 10.1007/s00467-022-05762-4</identifier><identifier>PMID: 36315273</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Aldosterone ; Angiotensin ; Care and treatment ; Child ; Children ; Complications and side effects ; Diseases ; Drug Resistance ; Etiology ; Humans ; Immunosuppression ; Immunosuppression Therapy ; Immunosuppressive Agents - therapeutic use ; Kidney diseases ; Kidneys ; Medicine ; Medicine & Public Health ; Nephrology ; Nephrotic syndrome ; Nephrotic Syndrome - drug therapy ; Nephrotic Syndrome - genetics ; Original ; Original Article ; Patient outcomes ; Patients ; Pediatrics ; Phenotypes ; Proteinuria ; Proteinuria - drug therapy ; Proteinuria - etiology ; Remission ; Renal failure ; Renal Insufficiency - drug therapy ; Renin ; Steroids ; Urology</subject><ispartof>Pediatric nephrology (Berlin, West), 2023-05, Vol.38 (5), p.1499-1511</ispartof><rights>The Author(s) 2022</rights><rights>2022. The Author(s).</rights><rights>COPYRIGHT 2023 Springer</rights><rights>The Author(s) 2022. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c611t-45f72ced77b56d4b7b81ba4b29707eb2bbbe254567216e329659e7d2799aa2d3</citedby><cites>FETCH-LOGICAL-c611t-45f72ced77b56d4b7b81ba4b29707eb2bbbe254567216e329659e7d2799aa2d3</cites><orcidid>0000-0001-7977-3310</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00467-022-05762-4$$EPDF$$P50$$Gspringer$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00467-022-05762-4$$EHTML$$P50$$Gspringer$$Hfree_for_read</linktohtml><link.rule.ids>230,314,776,780,881,27904,27905,41468,42537,51299</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/36315273$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Trautmann, Agnes</creatorcontrib><creatorcontrib>Seide, Svenja</creatorcontrib><creatorcontrib>Lipska-Ziętkiewicz, Beata S.</creatorcontrib><creatorcontrib>Ozaltin, Fatih</creatorcontrib><creatorcontrib>Szczepanska, Maria</creatorcontrib><creatorcontrib>Azocar, Marta</creatorcontrib><creatorcontrib>Jankauskiene, Augustina</creatorcontrib><creatorcontrib>Zurowska, Alexandra</creatorcontrib><creatorcontrib>Caliskan, Salim</creatorcontrib><creatorcontrib>Saeed, Bassam</creatorcontrib><creatorcontrib>Morello, William</creatorcontrib><creatorcontrib>Emma, Francesco</creatorcontrib><creatorcontrib>Litwin, Mieczyslaw</creatorcontrib><creatorcontrib>Tsygin, Alexey</creatorcontrib><creatorcontrib>Fomina, Svitlana</creatorcontrib><creatorcontrib>Wasilewska, Anna</creatorcontrib><creatorcontrib>Melk, Anette</creatorcontrib><creatorcontrib>Benetti, Elisa</creatorcontrib><creatorcontrib>Gellermann, Jutta</creatorcontrib><creatorcontrib>Stajic, Natasa</creatorcontrib><creatorcontrib>Tkaczyk, Marcin</creatorcontrib><creatorcontrib>Baiko, Sergey</creatorcontrib><creatorcontrib>Prikhodina, Larisa</creatorcontrib><creatorcontrib>Csaicsich, Dagmar</creatorcontrib><creatorcontrib>Medynska, Anna</creatorcontrib><creatorcontrib>Krisam, Regina</creatorcontrib><creatorcontrib>Breitschwerdt, Heike</creatorcontrib><creatorcontrib>Schaefer, Franz</creatorcontrib><creatorcontrib>PodoNet Consortium</creatorcontrib><creatorcontrib>for the PodoNet Consortium</creatorcontrib><title>Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression</title><title>Pediatric nephrology (Berlin, West)</title><addtitle>Pediatr Nephrol</addtitle><addtitle>Pediatr Nephrol</addtitle><description>Background
The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction.
Methods
Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission.
Results
Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (
n
= 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin–angiotensin–aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS.
Conclusion
Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS.
Graphical abstract
A higher resolution version of the Graphical abstract is available as
Supplementary information</description><subject>Aldosterone</subject><subject>Angiotensin</subject><subject>Care and treatment</subject><subject>Child</subject><subject>Children</subject><subject>Complications and side effects</subject><subject>Diseases</subject><subject>Drug Resistance</subject><subject>Etiology</subject><subject>Humans</subject><subject>Immunosuppression</subject><subject>Immunosuppression Therapy</subject><subject>Immunosuppressive Agents - therapeutic use</subject><subject>Kidney diseases</subject><subject>Kidneys</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Nephrology</subject><subject>Nephrotic syndrome</subject><subject>Nephrotic Syndrome - drug therapy</subject><subject>Nephrotic Syndrome - genetics</subject><subject>Original</subject><subject>Original Article</subject><subject>Patient outcomes</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Phenotypes</subject><subject>Proteinuria</subject><subject>Proteinuria - drug therapy</subject><subject>Proteinuria - etiology</subject><subject>Remission</subject><subject>Renal failure</subject><subject>Renal Insufficiency - drug therapy</subject><subject>Renin</subject><subject>Steroids</subject><subject>Urology</subject><issn>0931-041X</issn><issn>1432-198X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><sourceid>C6C</sourceid><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp9kk9rFTEUxQdR7LP6BVzIgCDdpObfJG9WUopVodBNF92FZHLnTepMMiYZpd_ePF9t--QhWQTu_Z2T5ORW1VuCTwnG8mPCmAuJMKUIN1JQxJ9VK8IZRaRd3zyvVrhlBGFObo6qVyndYozXzVq8rI6YYKShkq2q71dL7sIEqQ59nTLE4CyKkFzK2ufawzzEkF1XpztvYwFr5-tucKON4Gsfcp0j6Ay2_uXyUJoZfHK9KwU3TYsPaZnn4pdc8K-rF70eE7y534-r64vP1-df0eXVl2_nZ5eoE4RkxJte0g6slKYRlhtp1sRobmgrsQRDjTFAG94ISYkARlvRtCAtlW2rNbXsuPq0s50XM4HtwOeoRzVHN-l4p4J2ar_j3aA24acqoQrMKCsOJ_cOMfxYIGU1udTBOGoPYUmqJIcFL1Higr7_B70NS_TleYVqKZGcCvlIbfQIyvk-lIO7rak6k5wJ0kqy9UIHqA14KLcMHnpXynv86QG-LAuT6w4KPjwRDKDHPKQwLrl8TtoH6Q7sYkgpQv-QHsHbmKTazZ4qs6f-zJ7iRfTuae4Pkr_DVgC2A1Jp-Q3Ex6z-Y_sb1Afk_w</recordid><startdate>20230501</startdate><enddate>20230501</enddate><creator>Trautmann, Agnes</creator><creator>Seide, Svenja</creator><creator>Lipska-Ziętkiewicz, Beata S.</creator><creator>Ozaltin, Fatih</creator><creator>Szczepanska, Maria</creator><creator>Azocar, Marta</creator><creator>Jankauskiene, Augustina</creator><creator>Zurowska, Alexandra</creator><creator>Caliskan, Salim</creator><creator>Saeed, Bassam</creator><creator>Morello, William</creator><creator>Emma, Francesco</creator><creator>Litwin, Mieczyslaw</creator><creator>Tsygin, Alexey</creator><creator>Fomina, Svitlana</creator><creator>Wasilewska, Anna</creator><creator>Melk, Anette</creator><creator>Benetti, Elisa</creator><creator>Gellermann, Jutta</creator><creator>Stajic, Natasa</creator><creator>Tkaczyk, Marcin</creator><creator>Baiko, Sergey</creator><creator>Prikhodina, Larisa</creator><creator>Csaicsich, Dagmar</creator><creator>Medynska, Anna</creator><creator>Krisam, Regina</creator><creator>Breitschwerdt, Heike</creator><creator>Schaefer, Franz</creator><general>Springer Berlin Heidelberg</general><general>Springer</general><general>Springer Nature B.V</general><scope>C6C</scope><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>3V.</scope><scope>7QP</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-7977-3310</orcidid></search><sort><creationdate>20230501</creationdate><title>Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression</title><author>Trautmann, Agnes ; Seide, Svenja ; Lipska-Ziętkiewicz, Beata S. ; Ozaltin, Fatih ; Szczepanska, Maria ; Azocar, Marta ; Jankauskiene, Augustina ; Zurowska, Alexandra ; Caliskan, Salim ; Saeed, Bassam ; Morello, William ; Emma, Francesco ; Litwin, Mieczyslaw ; Tsygin, Alexey ; Fomina, Svitlana ; Wasilewska, Anna ; Melk, Anette ; Benetti, Elisa ; Gellermann, Jutta ; Stajic, Natasa ; Tkaczyk, Marcin ; Baiko, Sergey ; Prikhodina, Larisa ; Csaicsich, Dagmar ; Medynska, Anna ; Krisam, Regina ; Breitschwerdt, Heike ; Schaefer, Franz</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c611t-45f72ced77b56d4b7b81ba4b29707eb2bbbe254567216e329659e7d2799aa2d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Aldosterone</topic><topic>Angiotensin</topic><topic>Care and treatment</topic><topic>Child</topic><topic>Children</topic><topic>Complications and side effects</topic><topic>Diseases</topic><topic>Drug Resistance</topic><topic>Etiology</topic><topic>Humans</topic><topic>Immunosuppression</topic><topic>Immunosuppression Therapy</topic><topic>Immunosuppressive Agents - therapeutic use</topic><topic>Kidney diseases</topic><topic>Kidneys</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Nephrology</topic><topic>Nephrotic syndrome</topic><topic>Nephrotic Syndrome - drug therapy</topic><topic>Nephrotic Syndrome - genetics</topic><topic>Original</topic><topic>Original Article</topic><topic>Patient outcomes</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Phenotypes</topic><topic>Proteinuria</topic><topic>Proteinuria - drug therapy</topic><topic>Proteinuria - etiology</topic><topic>Remission</topic><topic>Renal failure</topic><topic>Renal Insufficiency - drug therapy</topic><topic>Renin</topic><topic>Steroids</topic><topic>Urology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Trautmann, Agnes</creatorcontrib><creatorcontrib>Seide, Svenja</creatorcontrib><creatorcontrib>Lipska-Ziętkiewicz, Beata S.</creatorcontrib><creatorcontrib>Ozaltin, Fatih</creatorcontrib><creatorcontrib>Szczepanska, Maria</creatorcontrib><creatorcontrib>Azocar, Marta</creatorcontrib><creatorcontrib>Jankauskiene, Augustina</creatorcontrib><creatorcontrib>Zurowska, Alexandra</creatorcontrib><creatorcontrib>Caliskan, Salim</creatorcontrib><creatorcontrib>Saeed, Bassam</creatorcontrib><creatorcontrib>Morello, William</creatorcontrib><creatorcontrib>Emma, Francesco</creatorcontrib><creatorcontrib>Litwin, Mieczyslaw</creatorcontrib><creatorcontrib>Tsygin, Alexey</creatorcontrib><creatorcontrib>Fomina, Svitlana</creatorcontrib><creatorcontrib>Wasilewska, Anna</creatorcontrib><creatorcontrib>Melk, Anette</creatorcontrib><creatorcontrib>Benetti, Elisa</creatorcontrib><creatorcontrib>Gellermann, Jutta</creatorcontrib><creatorcontrib>Stajic, Natasa</creatorcontrib><creatorcontrib>Tkaczyk, Marcin</creatorcontrib><creatorcontrib>Baiko, Sergey</creatorcontrib><creatorcontrib>Prikhodina, Larisa</creatorcontrib><creatorcontrib>Csaicsich, Dagmar</creatorcontrib><creatorcontrib>Medynska, Anna</creatorcontrib><creatorcontrib>Krisam, Regina</creatorcontrib><creatorcontrib>Breitschwerdt, Heike</creatorcontrib><creatorcontrib>Schaefer, Franz</creatorcontrib><creatorcontrib>PodoNet Consortium</creatorcontrib><creatorcontrib>for the PodoNet Consortium</creatorcontrib><collection>Springer Nature OA Free Journals</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>Consumer Health Database (Alumni Edition)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Consumer Health Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</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>Trautmann, Agnes</au><au>Seide, Svenja</au><au>Lipska-Ziętkiewicz, Beata S.</au><au>Ozaltin, Fatih</au><au>Szczepanska, Maria</au><au>Azocar, Marta</au><au>Jankauskiene, Augustina</au><au>Zurowska, Alexandra</au><au>Caliskan, Salim</au><au>Saeed, Bassam</au><au>Morello, William</au><au>Emma, Francesco</au><au>Litwin, Mieczyslaw</au><au>Tsygin, Alexey</au><au>Fomina, Svitlana</au><au>Wasilewska, Anna</au><au>Melk, Anette</au><au>Benetti, Elisa</au><au>Gellermann, Jutta</au><au>Stajic, Natasa</au><au>Tkaczyk, Marcin</au><au>Baiko, Sergey</au><au>Prikhodina, Larisa</au><au>Csaicsich, Dagmar</au><au>Medynska, Anna</au><au>Krisam, Regina</au><au>Breitschwerdt, Heike</au><au>Schaefer, Franz</au><aucorp>PodoNet Consortium</aucorp><aucorp>for the PodoNet Consortium</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression</atitle><jtitle>Pediatric nephrology (Berlin, West)</jtitle><stitle>Pediatr Nephrol</stitle><addtitle>Pediatr Nephrol</addtitle><date>2023-05-01</date><risdate>2023</risdate><volume>38</volume><issue>5</issue><spage>1499</spage><epage>1511</epage><pages>1499-1511</pages><issn>0931-041X</issn><eissn>1432-198X</eissn><abstract>Background
The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction.
Methods
Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission.
Results
Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (
n
= 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin–angiotensin–aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS.
Conclusion
Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS.
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>36315273</pmid><doi>10.1007/s00467-022-05762-4</doi><tpages>13</tpages><orcidid>https://orcid.org/0000-0001-7977-3310</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0931-041X |
| ispartof | Pediatric nephrology (Berlin, West), 2023-05, Vol.38 (5), p.1499-1511 |
| issn | 0931-041X 1432-198X |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_10060323 |
| source | MEDLINE; Springer Nature - Complete Springer Journals |
| subjects | Aldosterone Angiotensin Care and treatment Child Children Complications and side effects Diseases Drug Resistance Etiology Humans Immunosuppression Immunosuppression Therapy Immunosuppressive Agents - therapeutic use Kidney diseases Kidneys Medicine Medicine & Public Health Nephrology Nephrotic syndrome Nephrotic Syndrome - drug therapy Nephrotic Syndrome - genetics Original Original Article Patient outcomes Patients Pediatrics Phenotypes Proteinuria Proteinuria - drug therapy Proteinuria - etiology Remission Renal failure Renal Insufficiency - drug therapy Renin Steroids Urology |
| title | Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-21T10%3A15%3A01IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Outcomes%20of%20steroid-resistant%20nephrotic%20syndrome%20in%20children%20not%20treated%20with%20intensified%20immunosuppression&rft.jtitle=Pediatric%20nephrology%20(Berlin,%20West)&rft.au=Trautmann,%20Agnes&rft.aucorp=PodoNet%20Consortium&rft.date=2023-05-01&rft.volume=38&rft.issue=5&rft.spage=1499&rft.epage=1511&rft.pages=1499-1511&rft.issn=0931-041X&rft.eissn=1432-198X&rft_id=info:doi/10.1007/s00467-022-05762-4&rft_dat=%3Cgale_pubme%3EA743619710%3C/gale_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2792174267&rft_id=info:pmid/36315273&rft_galeid=A743619710&rfr_iscdi=true |