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Kidney non-tumor

Hereditary renal disease

Lipoprotein glomerulopathy


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 30 August 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare genetic disorder of lipid metabolism (Arch Pathol Lab Med 2010;134:279, Curr Opin Lipidol 2011;22:262)
● Various mutations; not all with mutations have glomerulopathy (Nephron Clin Pract 2010;114:c260)
● 2/3 male, often Japanese and wide age range (2 weeks to 69 years)
● Presents with heavy proteinuria, usually in adults
● 50% progress to renal failure
● Usually no systemic manifestations of hyperlipidemia

Treatment
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● Anti-lipidemic drugs (Clin Exp Nephrol 2010;14:619, Clin Exp Nephrol 2009;13:659); LDL-apheresis (J Med Case Rep 2009;3:9311) or immunoadsorption onto staphylococcal protein A for refractory cases (Nephrol Dial Transplant 2009;24:864)
● May recur in transplants

Micro description
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● Lipoprotein thrombi containing apolipoprotein apo E, usually E2

Micro images
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Lipoprotein thrombi

       
Dilated capillary lumina


Various lipid peroxidation-protein adducts

Electron microscopy description
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● Characteristic lamellar accumulations of variably sized lipid droplets

Electron microscopy images
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Lipid granules and concentrically laminated vacuoles


Glomerular capillary lumina filled with partially lamellated, finely vacuolated lipoprotein thrombi; mesangium is thickened by cell processes and increased matrix


Osmiophilic substances (arrows) are deposited in dilated subendothelial space

End of Kidney non-tumor > Hereditary renal disease > Lipoprotein glomerulopathy

Ref Updated: 6/14/12


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