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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)

J. Staples1, P. Taylor1,2, A. Magil1,3, J. Frohlich3, S. M. Johnston4, M. Koschinsky4, C. Chan-Yan1,2 and A. Levin1,2

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

The first 150 words of the full text of this article appear below.



   Introduction
 
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
 
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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Table 1 Clinical and laboratory results

 
At initial presentation, Sister A had no significant medical . . . [Full Text of this Article]

Clinical course
Further Lp(a) and biopsy investigations
Control patients


   Discussion
 

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