{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T15:09:36Z","timestamp":1787238576934,"version":"build-2736575974"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,5,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Background: Agalsidase-\u03b1 0.2 mg\/kg every other week (eow) and agalsidase-\u03b2 1.0 mg\/kg\/eow are licensed in Europe as equipotent treatment of the \u03b1-galactosidase deficiency in Fabry disease. This case series describes the effects of agalsidase dose adjustments in serial kidney biopsies in switch patients.<\/jats:p>\n                  <jats:p>Methods: All treatment-na\u00efve patients with classical Fabry disease in our centre started on agalsidase-\u03b2 1.0 mg\/kg\/eow and subsequently switched to agalsidase-\u03b1 0.2 mg\/kg\/eow were included (n= 3). The median age at enzyme replacement therapy start was 11 (range 7\u201318) years. Kidney biopsies were performed at baseline, after 5 years of agalsidase-\u03b2 1.0 mg\/kg\/eow and after 3 subsequent years of agalsidase-\u03b1 0.2 mg\/kg\/eow. One patient was re-biopsied 2 years after reswitch to agalsidase-\u03b2 1.0 mg\/kg\/eow. The scoring system of the International Scoring Group of Fabry Nephropathy was used.<\/jats:p>\n                  <jats:p>Results: The patients completely cleared globotriaosylceramide (GL3) from mesangial and endothelial cells and partly cleared podocytes on agalsidase-\u03b2 1.0 mg\/kg\/eow. Reaccumulation of GL3 in podocytes, but not in the mesangium or endothelium, occurred after 3 years of agalsidase-\u03b1 0.2 mg\/kg\/eow. Subsequent reduction of podocyte GL3 was observed in the single patient rebiopsied 2 years after reswitch to agalsidase-\u03b2 1.0 mg\/kg\/eow.<\/jats:p>\n                  <jats:p>Conclusion: Partial clearance, reaccumulation and renewed partial clearance of podocyte GL3 deposits in serial kidney biopsies over 8\u201310 years were seen in parallel with agalsidase dose adjustments. Repeated kidney biopsies may impact therapeutic choices in Fabry disease.<\/jats:p>","DOI":"10.1093\/ndt\/gfw094","type":"journal-article","created":{"date-parts":[[2016,5,17]],"date-time":"2016-05-17T20:58:57Z","timestamp":1463518737000},"page":"807-813","source":"Crossref","is-referenced-by-count":14,"title":["Reaccumulation of globotriaosylceramide in podocytes after agalsidase dose reduction in young Fabry patients"],"prefix":"10.1093","volume":"32","author":[{"given":"Rannveig","family":"Skrunes","sequence":"first","affiliation":[{"name":"1Department of Medicine, Haukeland University Hospital, Bergen, Norway"},{"name":"2Department of Clinical Medicine, University of Bergen, Bergen, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Einar","family":"Svarstad","sequence":"additional","affiliation":[{"name":"1Department of Medicine, Haukeland University Hospital, Bergen, Norway"},{"name":"2Department of Clinical Medicine, University of Bergen, Bergen, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristin","family":"Kampevold Larsen","sequence":"additional","affiliation":[{"name":"3Department of Pathology, Haukeland University Hospital, Bergen, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Leh","sequence":"additional","affiliation":[{"name":"2Department of Clinical Medicine, University of Bergen, Bergen, Norway"},{"name":"3Department of Pathology, Haukeland University Hospital, Bergen, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camilla","family":"T\u00f8ndel","sequence":"additional","affiliation":[{"name":"2Department of Clinical Medicine, University of Bergen, Bergen, Norway"},{"name":"4Department of Pediatrics, Haukeland University Hospital, Bergen, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2016,5,17]]},"reference":[{"key":"2025102312493520700_gfw094C1","first-page":"3733","article-title":"Alpha-galactosidase A deficiency: Fabry disease","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Desnick","year":"2001"},{"key":"2025102312493520700_gfw094C2","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1038\/ki.1978.32","article-title":"Early renal changes in hemizygous and heterozygous patients with Fabry's disease","volume":"13","author":"Gubler","year":"1978","journal-title":"Kidney Int"},{"key":"2025102312493520700_gfw094C3","first-page":"317","article-title":"Fabry disease: clinical features and recent advances in enzyme replacement therapy","volume":"31","author":"Desnick","year":"2001","journal-title":"Adv Nephrol Necker 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