{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T16:09:35Z","timestamp":1790698175680,"version":"4.1.0"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T00:00:00Z","timestamp":1768953600000},"content-version":"vor","delay-in-days":398,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,12,19]],"date-time":"2024-12-19T00:00:00Z","timestamp":1734566400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,12,19]],"date-time":"2024-12-19T00:00:00Z","timestamp":1734566400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Fabry disease is a rare genetic lysosomal storage disorder, whereby the accumulation of sphingolipids consequently leads to kidney structural damage and dysfunction. We explored the epidemiology of chronic kidney disease (CKD) among patients with Fabry disease at a major UK referral centre\u00a0in Greater Manchester serving over 7 million people, to inform early predictors of kidney disease and possible treatment planning.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data were sourced from the electronic records of registered participants from November 2020 to February 2022 of adults diagnosed with Fabry disease, with at least 1\u00a0year of follow-up. Four hundred and five participants (female\u2009=\u2009223, male\u2009=\u2009182) met the initial eligibility criteria. Our study focused on identifying factors linked to incident CKD, with 395 evaluable individuals undergoing outcome analysis over a median of 6.4\u00a0years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Findings concluded that 60.5% of participants received disease-modifying treatments, 29.7% experienced non-fatal cardiovascular events, 3.3% developed end-stage kidney disease (ESKD), and 7.3% died. Men had higher use\u00a0of disease modifying therapy, progression to ESKD requiring kidney replacement therapy, cardiovascular events, and mortality compared to women. Subgroup analysis over 9\u00a0years revealed that older age, cardiovascular history, renin\u2013angiotensin\u2013aldosterone system inhibitor use, and higher urine albumin-to-creatinine ratio (uACR) were predictors of faster estimated glomerular filtration rate (eGFR) decline and increased mortality. At baseline, 47.8% of 249 patients with uACR data had CKD, and 25.4% of the remaining individuals developed CKD during follow-up, associated with higher uACR and lower, albeit normal eGFR levels.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Over 60% of Fabry disease patients are at lifetime risk of developing CKD, with a substantial risk of mortality, even with initially normal uACR and eGFR values.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Graphical abstract<\/jats:title>\n                  <\/jats:sec>","DOI":"10.1007\/s40620-024-02170-9","type":"journal-article","created":{"date-parts":[[2024,12,19]],"date-time":"2024-12-19T09:11:26Z","timestamp":1734599486000},"page":"579-587","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Epidemiology and early predictors of Fabry nephropathy: evaluation of long-term outcomes from a national Fabry centre"],"prefix":"10.1093","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3094-784X","authenticated-orcid":false,"given":"Fahmida","family":"Mannan","sequence":"first","affiliation":[{"name":"The School of Medicine, Manchester Academic Health Sciences Centre, Manchester University, Manchester, UK 1"},{"name":"Division of Cardiovascular Sciences, Faculty of Biology, Medicine and Health, Manchester University, Manchester, UK 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University NHS Foundation Trust, Manchester, UK 7"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolina M.","family":"Stepien","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Sciences, Faculty of Biology, Medicine and Health, Manchester University, Manchester, UK 2"},{"name":"Adult Inherited Metabolic Diseases, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, Greater Manchester, UK 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reena","family":"Sharma","sequence":"additional","affiliation":[{"name":"Adult Inherited Metabolic Diseases, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, Greater Manchester, UK 3"},{"name":"Division of Diabetes, Endocrinology and Gastroenterology, University of Manchester, Manchester, UK 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Northern Care Alliance NHS Foundation Trust, Salford, Greater Manchester, UK 4"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Jovanovic","sequence":"additional","affiliation":[{"name":"The School of Medicine, Manchester Academic Health Sciences Centre, Manchester University, Manchester, UK 1"},{"name":"Adult Inherited Metabolic Diseases, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, Greater Manchester, UK 3"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,12,19]]},"reference":[{"issue":"5","key":"2026012110525766100_CR1","doi-asserted-by":"publisher","first-page":"1631","DOI":"10.1681\/ASN.2016090964 27979989","article-title":"Characterization of classical and nonclassical Fabry disease: a multicenter study","volume":"28","author":"Arends","year":"2017","journal-title":"J Am Soc 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June 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 November 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 December 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"No conflicts of interest to declare.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The study had institutional (study reference: S19MET08-S) and Health Research Authority (identification number: 262706, reference: 19\/HRA\/5221) approval, and was exempt from review by a regional ethics committee.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"This study was conducted in accordance with the ethical 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