{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T20:32:02Z","timestamp":1786480322586,"version":"build-2736575974"},"reference-count":60,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"10","license":[{"start":{"date-parts":[[2025,6,19]],"date-time":"2025-06-19T00:00:00Z","timestamp":1750291200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["U24DK126110"],"award-info":[{"award-number":["U24DK126110"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["NIHU24DK12110"],"award-info":[{"award-number":["NIHU24DK12110"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"crossref","award":["UL1TR002377"],"award-info":[{"award-number":["UL1TR002377"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Mayo Clinic Center for Clinical and Translational Science","award":["Career Development Award"],"award-info":[{"award-number":["Career Development Award"]}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["U24DK126110"],"award-info":[{"award-number":["U24DK126110"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["NIHU24DK12110"],"award-info":[{"award-number":["NIHU24DK12110"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"crossref","award":["UL1TR002377"],"award-info":[{"award-number":["UL1TR002377"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Mayo Clinic Center for Clinical and Translational Science","award":["Career Development Award"],"award-info":[{"award-number":["Career Development Award"]}]}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Kidney360"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:p>Tuberous sclerosis complex (TSC) is a highly variable autosomal dominant disease characterized by dysregulated organ development and growth. Benign tumors, termed hamartomas, may occur across organ systems but typically involve the kidney, brain, skin, heart, and lung. The diagnosis, surveillance, and clinical management of TSC requires a multidisciplinary approach, adopted by dedicated multispecialty centers worldwide. Nephrology involvement predominantly stems from the morbidity and mortality related to the prototypical kidney lesion, angiomyolipomas, whose presence and degree confers risk of CKD, hypertension, retroperitoneal bleeding, and possibly renal cell carcinoma. Surveillance of kidney structural lesions, kidney function, and BP may enable early interventions that limit kidney-related morbidity and mortality, such as mammalian target of rapamycin inhibitor therapy. We review the epidemiology, genetics, and pathogenesis of TSC and how these inform the evaluation, diagnosis, and clinical management of TSC from the vantage point of the treating nephrologist.<\/jats:p>","DOI":"10.34067\/kid.0000000904","type":"journal-article","created":{"date-parts":[[2025,6,19]],"date-time":"2025-06-19T12:04:30Z","timestamp":1750334670000},"page":"1818-1825","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Management of Tuberous Sclerosis"],"prefix":"10.34067","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0760-745X","authenticated-orcid":false,"given":"Jielu Hao","family":"Robichaud","sequence":"first","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Humayra","family":"Afrin","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2008-1576","authenticated-orcid":false,"given":"Vicente E.","family":"Torres","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-7005-4098","authenticated-orcid":false,"given":"Navin R.","family":"Gupta","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"},{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,6,19]]},"reference":[{"issue":"3","key":"B1-20251101","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1016\/j.pcl.2015.03.005","article-title":"Tuberous sclerosis complex","volume":"62","author":"DiMario","year":"2015","journal-title":"Pediatr Clin North Am."},{"key":"B2-20251101","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.pediatrneurol.2021.07.011","article-title":"Updated international tuberous sclerosis complex diagnostic criteria and surveillance and management recommendations","volume":"123","author":"Northrup","year":"2021","journal-title":"Pediatr Neurol."},{"issue":"9639","key":"B3-20251101","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1016\/S0140-6736(08)61279-9","article-title":"Tuberous sclerosis","volume":"372","author":"Curatolo","year":"2008","journal-title":"Lancet."},{"issue":"2","key":"B4-20251101","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.jaad.2007.05.004","article-title":"Tuberous sclerosis complex: advances in diagnosis, genetics, and management","volume":"57","author":"Schwartz","year":"2007","journal-title":"J Am Acad Dermatol."},{"issue":"12","key":"B5-20251101","doi-asserted-by":"crossref","first-page":"1348","DOI":"10.1177\/0883073807307093","article-title":"Intrafamilial phenotypic variability in tuberous sclerosis complex","volume":"22","author":"Lyczkowski","year":"2007","journal-title":"J Child Neurol."},{"issue":"11","key":"B6-20251101","doi-asserted-by":"crossref","first-page":"1020","DOI":"10.1136\/adc.2011.211995","article-title":"The Tuberous Sclerosis 2000 Study: presentation, initial assessments and implications for diagnosis and management","volume":"96","author":"Yates","year":"2011","journal-title":"Arch Dis Child."},{"issue":"1","key":"B7-20251101","doi-asserted-by":"crossref","first-page":"e117","DOI":"10.1542\/peds.2010-0192","article-title":"Tuberous sclerosis complex: diagnostic challenges, presenting symptoms, and commonly missed signs","volume":"127","author":"Staley","year":"2011","journal-title":"Pediatrics."},{"issue":"7","key":"B8-20251101","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1016\/0092-8674(93)90618-Z","article-title":"Identification and characterization of the tuberous sclerosis gene on chromosome 16","volume":"75","year":"1993","journal-title":"Cell."},{"issue":"5327","key":"B9-20251101","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1126\/science.277.5327.805","article-title":"Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34","volume":"277","author":"van Slegtenhorst","year":"1997","journal-title":"Science."},{"key":"B10-20251101","doi-asserted-by":"crossref","first-page":"806521","DOI":"10.3389\/fcell.2021.806521","article-title":"Beyond protein synthesis; 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