| Progressive podocyte injury and globotriaosylceramide (GL-3) accumulation in young patients with Fabry disease. | |
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MedLine Citation:
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PMID: 21160462 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Progressive renal failure often complicates Fabry disease, the pathogenesis of which is not well understood. To further explore this we applied unbiased stereological quantitative methods to electron microscopic changes of Fabry nephropathy and the relationship between parameters of glomerular structure and renal function in 14 young Fabry patients (median age 12 years). Renal biopsies were obtained shortly before enzyme replacement therapy from these patients and from nine normal living kidney donors as controls. Podocyte globotriaosylceramide (GL-3) inclusion volume density increased progressively with age; however, there were no significant relationships between age and endothelial or mesangial inclusion volume densities. Foot process width, greater in male Fabry patients, also progressively increased with age compared with the controls, and correlated directly with proteinuria. In comparison to the biopsies of the controls, endothelial fenestration was reduced in Fabry patients. Thus, our study found relationships between quantitative parameters of glomerular structure in Fabry nephropathy and age, as well as urinary protein excretion. Hence, podocyte injury may play a pivotal role in the development and progression of Fabry nephropathy. |
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Authors:
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Behzad Najafian; Einar Svarstad; Leif Bostad; Marie-Claire Gubler; Camilla Tøndel; Chester Whitley; Michael Mauer |
Publication Detail:
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Type: Journal Article; Multicenter Study Date: 2010-12-15 |
Journal Detail:
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Title: Kidney international Volume: 79 ISSN: 1523-1755 ISO Abbreviation: Kidney Int. Publication Date: 2011 Mar |
Date Detail:
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Created Date: 2011-03-01 Completed Date: 2011-06-13 Revised Date: 2013-05-06 |
Medline Journal Info:
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Nlm Unique ID: 0323470 Medline TA: Kidney Int Country: United States |
Other Details:
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Languages: eng Pagination: 663-70 Citation Subset: IM |
Affiliation:
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Department of Pathology, University of Washington, Seattle, Washington, USA. najafian@u.washington.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Age Factors Biological Markers / blood, urine Biopsy Case-Control Studies Child Child, Preschool Creatinine / blood, urine Disease Progression Endothelial Cells / chemistry, ultrastructure Europe Fabry Disease / complications*, metabolism, pathology, physiopathology Female Glomerular Filtration Rate Humans Kidney Glomerulus / blood supply, chemistry*, pathology, physiopathology Least-Squares Analysis Linear Models Male Podocytes / chemistry*, ultrastructure* Proteinuria / etiology, metabolism, pathology Renal Insufficiency / etiology*, metabolism, pathology, physiopathology Risk Assessment Risk Factors Sex Factors Trihexosylceramides / metabolism* United States Up-Regulation Young Adult |
| Grant Support | |
ID/Acronym/Agency:
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U54 NS065768/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Biological Markers; 0/Trihexosylceramides; 60-27-5/Creatinine; 71965-57-6/globotriaosylceramide |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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