NDT Advance Access originally published online on January 16, 2008
Nephrology Dialysis Transplantation 2008 23(5):1756-1759; doi:10.1093/ndt/gfm866
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© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org
Progressive kidney disease in three sisters with elevated lipoprotein(a)
1 Department of Medicine 2 Division of Nephrology 3 Division of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada 4 Department of Biochemistry, Queen's University, Kingston, Ontario, Canada
Correspondence and offprint requests to: John Staples c/o Dr A Levin, University of British Columbia, Room 6010A 1081 Burrard, Vancouver BC V6Z 1Y8, Canada. Tel: +1-604-682-2344 ext 62232; Fax: +1-604-806-8120; E-mail: john.a.staples@gmail.com
Keywords: biopsy-proven deposition; chronic kidney disease; lipoprotein(a); progressive disease; uric acid
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| Introduction |
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Lipoprotein(a) [Lp(a)] is an atherogenic, LDL-like particle of unknown physiologic function. Elevated serum concentrations of Lp(a) have been linked conceptually and epidemiologically to cardiovascular disease [1–3], but their relationship to the progression of renal disease remains much less clear, despite several hypothesized mechanisms by which this could occur [4–12].
We present a unique case of three sisters with profound elevations in serum Lp(a), progressive but unexplained impairment of kidney function, and renal biopsies demonstrating benign nephrosclerosis and apolipoprotein(a) [apo(a)] deposition.
| Case report |
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Sisters A and B came to our attention independently after screening laboratory examinations revealed asymptomatic elevations in their serum creatinine, while Sister C was referred for assessment in light of her siblings renal impairment. Clinical and laboratory data for each sister, and limited data for their parents, are presented in Table 1.
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At initial presentation, Sister A had no significant medical
Clinical course
Further Lp(a) and biopsy investigations
Control patients
| Discussion |
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