{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T11:17:36Z","timestamp":1791285456513,"version":"4.1.0"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T00:00:00Z","timestamp":1772496000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008792","name":"Novartis Pharma","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008792","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,4,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Renal angiomyolipomas (AMLs) drive substantial morbidity in tuberous sclerosis complex (TSC) through haemorrhage and repeated invasive procedures. While mammalian target of rapamycin inhibitors (mTORis) reduce AML volume, long-term real-world data on clinically meaningful bleeding and procedure outcomes remain limited, particularly in cohorts enriched for high-risk imaging phenotypes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a multicentre observational study (2004\u20132020) in three French tertiary centres. Among 103 included patients, the 96 with TSC constituted the primary analysis set. We assessed AML-related haemorrhage and selective arterial embolization (SAE). For patients initiating mTORi, follow-up was split into pre- and post-initiation periods and incidence rate ratios (IRRs) were estimated using Poisson regression with patient-time offsets and patient-clustered robust standard errors; generalized estimating equation Poisson and negative binomial models were used as sensitivity analyses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Total follow-up was 694.3 patient-years. Thirty-nine patients (40.6%) initiated an mTORi (everolimus 94.9%). Haemorrhage rates decreased from 0.061 to 0.007 events per patient-year after mTORi initiation {IRR 0.11 [95% confidence interval (CI) 0.02\u20130.62]} and SAE rates decreased from 0.197 to 0.020 sessions per patient-year [IRR 0.10 (95% CI 0.04\u20130.29)]. Therapeutic inertia remained substantial: among 57 never-treated patients, high-risk imaging features and active complications were frequent at the last assessment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In this long-term real-world cohort of TSC-associated renal AMLs, mTORi initiation was associated with markedly lower rates of AML haemorrhage and SAE. Despite these benefits, many high-risk patients remained untreated in routine care, supporting earlier nephrology referral, systematic risk stratification and timely treatment initiation to reduce preventable AML-related morbidity.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ckj\/sfag072","type":"journal-article","created":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T12:35:41Z","timestamp":1772541341000},"source":"Crossref","is-referenced-by-count":1,"title":["Real-world management of tuberous sclerosis complex\u2013associated renal angiomyolipomas: the impact of mTOR inhibitors"],"prefix":"10.1093","volume":"19","author":[{"given":"Nicolas","family":"Gemander","sequence":"first","affiliation":[{"name":"Department of Nephrology, Dialysis and Renal Transplantation, H\u00f4pital Universitaire de Bruxelles (H.U.B.), Erasme Hospital, Universit\u00e9 libre de Bruxelles (ULB) , Brussels 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,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Provot","sequence":"additional","affiliation":[{"name":"Department of Nephrology, H\u00f4pital Claude Huriez, CHU de Lille , Lille ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Michel","family":"Correas","sequence":"additional","affiliation":[{"name":"Department of Radiology, H\u00f4pital Necker\u2013Enfants Malades, APHP, Universit\u00e9 Paris Cit\u00e9 , Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3568-3369","authenticated-orcid":false,"given":"Dominique","family":"Joly","sequence":"additional","affiliation":[{"name":"Department of Nephrology, H\u00f4pital Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris (APHP), Universit\u00e9 Paris Cit\u00e9 , Paris ,","place":["France"]},{"name":"F-CRIN INI-CRCT (Cardiovascular and Renal 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