{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T17:44:10Z","timestamp":1790790250222,"version":"4.1.0"},"reference-count":39,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2023,1,16]],"date-time":"2023-01-16T00:00:00Z","timestamp":1673827200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/100013995","name":"Sanofi Genzyme","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013995","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,8,31]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Cardiac and neurological involvements are the main clinical features of hereditary transthyretin (ATTRv) amyloidosis. Few data are available about ATTRv amyloid nephropathy (ATTRvN).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We retrospectively included 30 patients with biopsy-proven ATTRvN [V30M (26\/30) including two domino liver recipients, S77Y (2\/30), V122I (1\/30) and S50R (1\/30) variants] from two French reference centers. We described the pathological features by comparing amyloid deposits distribution to patients with AL or AA amyloidosis, and sought to determine clinicopathological correlation with known disease-modifying factors such as TTR variant, gender and age at diagnosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>In comparison with AL and AA amyloidosis, ATTRv patients had similar glomerular, arteriolar and arterial amyloid deposits, but more cortical and medullary tubulointerstitial (33%, 44%, 77%, P\u00a0=\u00a0.03) involvement. While the presence of glomerular deposits is associated with the range of proteinuria, some patients with abundant glomerular ATTRv amyloidosis had no significant proteinuria. V30M patients had more glomerular (100% and 25%, odds ratio\u00a0=\u00a0114, 95% confidence interval 3.85\u20133395.00, P\u00a0=\u00a0.001) deposits, and higher estimated glomerular filtration rate [50 (interquartile range 44\u201382) and 27 (interquartile range 6\u201331) mL\/min\/1.73 m\u00b2, P\u00a0=\u00a0.004] than non-V30M patients. We did not find difference in amyloid deposition according to gender or age at diagnosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>ATTRvN affects all kidney compartments, but compared with AL\/AA amyloidosis, ATTRvN seems to involve more frequently tubulointerstitial areas. V30M patients represents the dominant face of the disease with a higher risk of glomerular\/arteriolar involvement. ATTRvN should thus be considered in patients, and potential relatives, with ATTRv amyloidosis and kidney dysfunction, regardless of proteinuria level.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ndt\/gfad006","type":"journal-article","created":{"date-parts":[[2023,1,17]],"date-time":"2023-01-17T00:50:18Z","timestamp":1673916618000},"page":"2019-2030","source":"Crossref","is-referenced-by-count":10,"title":["Pathological spectrum of hereditary transthyretin renal amyloidosis and clinicopathologic correlation: a French observational study"],"prefix":"10.1093","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5690-7424","authenticated-orcid":false,"given":"Julien","family":"Dang","sequence":"first","affiliation":[{"name":"Assistance Publique des H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris-Saclay, H\u00f4pital de Bic\u00eatre, Service de N\u00e9phrologie et Transplantation , Le Kremlin-Bic\u00eatre , France"},{"name":"Centre de Comp\u00e9tence Maladies Rares \u00ab Syndrome N\u00e9phrotique Idiopathique \u00bb, H\u00f4pital de Bic\u00eatre , Le Kremlin-Bic\u00eatre , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4124-9227","authenticated-orcid":false,"given":"Sophie","family":"Ferlicot","sequence":"additional","affiliation":[{"name":"AP-HP, Universit\u00e9 Paris-Saclay, H\u00f4pital de Bic\u00eatre, Service d'Anatomie Pathologique , Le Kremlin-Bic\u00eatre , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5379-8859","authenticated-orcid":false,"given":"Micheline","family":"Misrahi","sequence":"additional","affiliation":[{"name":"AP-HP, Universit\u00e9 Paris-Saclay, H\u00f4pital de Bic\u00eatre, Unit\u00e9 de G\u00e9n\u00e9tique Mol\u00e9culaire des Maladies M\u00e9taboliques et de la Reproduction , Le Kremlin-Bic\u00eatre , 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