Hemolytic uremic syndrome as a cause of chronic kidney disease stage 5 in children is in retreat; results from the Polish Registry of Renal Replacement Therapy in children ( 2000-2023)

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Abstract Backgroud Hemolytic uremic syndrome (HUS) is a life-threatening disease with a poor prognosis in children receiving maintenance renal replacement therapy (RRT). The aim of this study was to analyse the incidence and outcome of chronic kidney disease stage 5 (CKD5) due to Escherichia coli-HUS (STEC-HUS) and complement-mediated HUS (CM-HUS) in children, compared to controls with non-HUS CKD5 over the last 24 years. Methods The study included 1,488 children undergoing RRT in Poland between 2000 and 2023. Thirty-nine patients with CM-HUS and 18 with STEC-HUS were identified and analysed for incidence, RRT modality and survival. Results The incidence rate of CKD5 was 0.09 cases/million age-related population (marp) for STEC-HUS and 0.23/marp for CM-HUS, while no new cases have been observed in recent years. CKD5 due to CM-HUS developed significantly earlier from initial HUS manifestation than in STEC-HUS (median 0,2 year vs. 9,8years). CM-HUS was associated with a younger age at initiation of RRT compared to STEC-HUS and non-HUS controls (median 6.0 years vs. 10.9 and 10.9 years), with a higher risk of death (Hazard Ratio 1.92, 95% Confidence Interval 0.9 - 4.13) and worse 5-year kidney graft survival at 77%, 93% and 90%, respectively (p<0.001). Conclusions In recent years, both CM-HUS and STEC-HUS, have become an increasingly rare causes of CKD5 in children. In the eculizumab era CKD5 due to CM-HUS is exceptional. Children on RRT due to STEC-HUS had a significantly better survival, shorter waiting time for kidney transplantation and better kidney graft survival compared to the CM-HUS group.
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Hemolytic uremic syndrome as a cause of chronic kidney disease stage 5 in children is in retreat; results from the Polish Registry of Renal Replacement Therapy in children ( 2000-2023) | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Hemolytic uremic syndrome as a cause of chronic kidney disease stage 5 in children is in retreat; results from the Polish Registry of Renal Replacement Therapy in children ( 2000-2023) Ilona Zagozdzon, Maria Szczepańska, Jacek Rubik, Katarzyna Zachwieja, and 9 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4864331/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 16 Nov, 2024 Read the published version in Pediatric Nephrology → Version 1 posted 5 You are reading this latest preprint version Abstract Backgroud Hemolytic uremic syndrome (HUS) is a life-threatening disease with a poor prognosis in children receiving maintenance renal replacement therapy (RRT). The aim of this study was to analyse the incidence and outcome of chronic kidney disease stage 5 (CKD5) due to Escherichia coli-HUS (STEC-HUS) and complement-mediated HUS (CM-HUS) in children, compared to controls with non-HUS CKD5 over the last 24 years. Methods The study included 1,488 children undergoing RRT in Poland between 2000 and 2023. Thirty-nine patients with CM-HUS and 18 with STEC-HUS were identified and analysed for incidence, RRT modality and survival. Results The incidence rate of CKD5 was 0.09 cases/million age-related population (marp) for STEC-HUS and 0.23/marp for CM-HUS, while no new cases have been observed in recent years. CKD5 due to CM-HUS developed significantly earlier from initial HUS manifestation than in STEC-HUS (median 0,2 year vs. 9,8years). CM-HUS was associated with a younger age at initiation of RRT compared to STEC-HUS and non-HUS controls (median 6.0 years vs. 10.9 and 10.9 years), with a higher risk of death (Hazard Ratio 1.92, 95% Confidence Interval 0.9 - 4.13) and worse 5-year kidney graft survival at 77%, 93% and 90%, respectively (p<0.001). Conclusions In recent years, both CM-HUS and STEC-HUS, have become an increasingly rare causes of CKD5 in children. In the eculizumab era CKD5 due to CM-HUS is exceptional. Children on RRT due to STEC-HUS had a significantly better survival, shorter waiting time for kidney transplantation and better kidney graft survival compared to the CM-HUS group. hemolytic uremic syndrome chronic kidney disease epidemiology survival children Full Text Cite Share Download PDF Status: Published Journal Publication published 16 Nov, 2024 Read the published version in Pediatric Nephrology → Version 1 posted Editorial decision: Major Revisions Needed 21 Aug, 2024 Reviewers agreed at journal 08 Aug, 2024 Reviewers invited by journal 07 Aug, 2024 Editor assigned by journal 06 Aug, 2024 First submitted to journal 05 Aug, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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The aim of this study was to analyse the incidence and outcome of chronic kidney disease stage 5 (CKD5) due to Escherichia coli-HUS (STEC-HUS) and complement-mediated HUS (CM-HUS) in children, compared to controls with non-HUS CKD5 over the last 24 years.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eMethods The study included 1,488 children undergoing RRT in Poland between 2000 and 2023. Thirty-nine patients with CM-HUS and 18 with STEC-HUS were identified and analysed for incidence, RRT modality and survival.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eResults The incidence rate of CKD5 was 0.09 cases/million age-related population (marp) for STEC-HUS and 0.23/marp for CM-HUS, while no new cases have been observed in recent years. CKD5 due to CM-HUS developed significantly earlier from initial HUS manifestation than in STEC-HUS (median 0,2 year vs. 9,8years). CM-HUS was associated with a younger age at initiation of RRT compared to STEC-HUS and non-HUS controls (median 6.0 years vs. 10.9 and 10.9 years), with a higher risk of death (Hazard Ratio 1.92, 95% Confidence Interval 0.9 - 4.13) and worse 5-year kidney graft survival \u0026nbsp;at 77%, 93% and 90%, respectively (p\u0026lt;0.001).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eConclusions In recent years, \u0026nbsp;both CM-HUS and STEC-HUS, have become an increasingly rare causes of CKD5 in children. In the eculizumab era CKD5 due to CM-HUS is exceptional. Children on RRT due to STEC-HUS had a significantly better survival, shorter waiting time for kidney transplantation and better kidney graft survival compared to the CM-HUS group.\u003c/p\u003e","manuscriptTitle":"Hemolytic uremic syndrome as a cause of chronic kidney disease stage 5 in children is in retreat; results from the Polish Registry of Renal Replacement Therapy in children ( 2000-2023)","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-09-02 18:46:00","doi":"10.21203/rs.3.rs-4864331/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Major Revisions Needed","date":"2024-08-21T17:45:07+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2024-08-08T07:13:42+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-08-07T10:46:36+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-08-06T16:12:42+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Nephrology","date":"2024-08-05T17:29:39+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-nephrology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pnep","sideBox":"Learn more about [Pediatric Nephrology](http://link.springer.com/journal/467)","snPcode":"467","submissionUrl":"https://www.editorialmanager.com/pnep/default2.aspx","title":"Pediatric Nephrology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"cb780389-948b-4392-8f00-0292cdc922ca","owner":[],"postedDate":"September 2nd, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-11-18T19:23:36+00:00","versionOfRecord":{"articleIdentity":"rs-4864331","link":"https://doi.org/10.1007/s00467-024-06584-2","journal":{"identity":"pediatric-nephrology","isVorOnly":false,"title":"Pediatric Nephrology"},"publishedOn":"2024-11-16 15:58:02","publishedOnDateReadable":"November 16th, 2024"},"versionCreatedAt":"2024-09-02 18:46:00","video":"","vorDoi":"10.1007/s00467-024-06584-2","vorDoiUrl":"https://doi.org/10.1007/s00467-024-06584-2","workflowStages":[]},"version":"v1","identity":"rs-4864331","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4864331","identity":"rs-4864331","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}

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