Atypical hyperlipidemia and nephropathy associated with apolipoprotein E homozygosity.

Journal of the American Society of Nephrology : JASN
D EllisJ R Diamond

Abstract

Hyperlipidemia has been implicated in the pathogenesis of experimental progressive glomerulosclerosis, but its role in human renal injury is controversial. This report describes a 12-yr-old boy presenting with massive proteinuria, hepatomegaly, anemia, severe mixed hyperlipidemia, and progressive renal failure. The initial renal biopsy disclosed large numbers of foam cells that were shown to be monocytes. Evidence is presented suggesting that apoprotein-E2 homozygosity in our patient, together with an 88% reduction in plasma lipoprotein lipase activity associated with severe nephrotic syndrome, is responsible for the atypical clinical features, lipoprotein phenotype III with chylomicronemia, and renal lipidosis. A regimen of dietary lipid restriction, gemfibrozil, and niacin resulted in significant but partial improvement of the dyslipidemia and resolution of the hepatomegaly and ascites. This report stresses the importance of characterizing unique lipid disorders in patients with nephrotic syndrome in order to prescribe effective lipid-lowering strategies. Moreover, the striking resemblance of the clinical and nephrohistologic features of this patient to those occurring in experimental models of coexisting glomerular injury an...Continue Reading

Citations

Jul 21, 1999·Kidney International. Supplement·T SaitoJ Sasaki
Jan 30, 2004·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Evagelos LiberopoulosMoses Elisaf
Apr 29, 1998·Kidney International·J A JolesH A Koomans
Mar 1, 1997·The Tohoku Journal of Experimental Medicine·T Saito
Jan 25, 2006·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Takao SaitoShinichi Oikawa
Jul 31, 2021·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·Madhuri RamakrishnanDiane M Cibrik
Mar 6, 2002·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Mikito HayakawaAkira Yamada
Oct 14, 2006·Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine·Takao Saito
Mar 20, 2009·Canadian Journal of Physiology and Pharmacology·Amit R RahalkarTisha Joy
Apr 6, 2011·Current Opinion in Lipidology·Vasilis Tsimihodimos, Moses Elisaf

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