Document Detail


Lipoprotein glomerulopathy: first report of 2 not consanguineous Italian men from the same town.
MedLine Citation:
PMID:  21534236     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
It is well known that the abnormal accumulation of lipids can occur in kidneys of patients affected by some metabolic disorders due either to inherited enzymatic deficiency or to an acquired lipid alteration as in nephrotic syndrome. Lipoprotein glomerulopathy (LG), briefly described in a patient of Koitabashi in 1987 in a review on renal lipidoses authored by Faraggiana and Churg, represents an emerging novel storage renal disease. This rare and unique nephropathy is characterized by the presence of lipoprotein thrombi in dilated glomerular capillary lumina associated with type III hyperlipoproteinemia, and high serum levels of apolipoprotein E (apo E). Several specific studies conducted by Saito et al on his patients from 1989, revealed that it was an hereditary disease with an autosomal recessive pattern that predominantly affects patients of Asian ancestry, mainly the Japanese population, but which very seldom, can also occur in white subjects. The disorder is probably due to an inherited altered lipid metabolism due to a mutation of the apo E genetic code. Clinically, LG is characterized by proteinuria generally associated with nephrotic syndrome and progressive renal insufficiency. We describe the cases of 2 Italian adult white male patients affected by LG, admitted in our nephrology unit in 2004 and in 2009, respectively.
Authors:
Antonio Pasquariello; Giovanna Pasquariello; Maurizio Innocenti; Francesca Minnei; Niccola Funel; Paolo Lorusso; Giuliano Barsotti
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-28
Journal Detail:
Title:  Journal of nephrology     Volume:  -     ISSN:  1724-6059     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-5-2     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9012268     Medline TA:  J Nephrol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Departmental Section of Native Kidney Histopathology, Azienda Ospedaliero Universitaria Pisana, Pisa - Italy.
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