2. Academic Validation
  3. Lipoprotein glomerulopathy-like disease in a patient with type III hyperlipoproteinemia due to apolipoprotein E2 (Arg158 Cys)/3 heterozygosity

Lipoprotein glomerulopathy-like disease in a patient with type III hyperlipoproteinemia due to apolipoprotein E2 (Arg158 Cys)/3 heterozygosity

  • Clin Exp Nephrol. 2007 Jun;11(2):174-179. doi: 10.1007/s10157-007-0469-4.
Miho Karube 1,  Kimimasa Nakabayashi 2,  Yasunori Fujioka 3,  Ken Yoshihara 1,  Akira Yamada 1,  Akira Matsunaga 4,  Takao Saito 5
Affiliations

Affiliations

  • 1 Division of Nephrology and Rheumatology, First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-0061, Japan.
  • 2 Division of Nephrology and Rheumatology, First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-0061, Japan. [email protected].
  • 3 Department of Pathology, Kyorin University School of Medicine, Tokyo, Japan.
  • 4 Division of Cardiology, Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
  • 5 Division of Nephrology and Rheumatology, Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
PMID: 17593519 DOI: 10.1007/s10157-007-0469-4
Abstract

A 77-year-old woman developed nephrotic syndrome associated with type III hyperlipoproteinemia (III HLP) and increased apolipoprotein E (apo E). Apo E analysis disclosed E2/E3 heterozygosity in phenotypic and genotypic expressions, without any other mutations. A renal biopsy showed intraluminal and subendothelial thrombus-like deposits in the dilated capillary loops of the glomerulus that stained positive for lipids and apo E. Electron microscopy revealed tiny granular particles in the capillary lumina, as well as between the glomerular basement membrane and the endothelial cells. It was therefore concluded that III HLP associated with apo E2/E3 heterozygosity could induce lipoprotein glomerulopathy-like disease and nephrotic syndrome.

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