Long-term follow-up of a paediatric case of lipoprotein glomerulopathy

A paediatric case of lipoprotein glomerulopathy, a new kidney disease characterized by glomerular lipoprotein thrombi, is reported. The patient had massive proteinuria from the age of 8 years, when the nephrotic syndrome was first detected. This was resistant to conventional treatment for more than...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 1990-03, Vol.4 (2), p.122-128
Main Authors: KOITABASHI, Y, IKOMA, M, MIYAHIRA, T, FUJITA, R, MIO, H, ISHIDA, M, SHIMIZU, K, SAKAGUCHI, H
Format: Article
Language:English
Subjects:
Apolipoproteins E - blood
Biological and medical sciences
Child
Disorders of blood lipids. Hyperlipoproteinemia
Humans
Hyperlipoproteinemia Type IV - pathology
Hyperlipoproteinemia Type IV - physiopathology
Kidney Diseases - pathology
Kidney Diseases - physiopathology
Kidney Glomerulus - metabolism
Kidney Glomerulus - pathology
Lipoproteins - metabolism
Male
Medical sciences
Metabolic diseases
Microscopy, Electron
Nephrotic Syndrome - pathology
Nephrotic Syndrome - physiopathology
Time Factors
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Summary:A paediatric case of lipoprotein glomerulopathy, a new kidney disease characterized by glomerular lipoprotein thrombi, is reported. The patient had massive proteinuria from the age of 8 years, when the nephrotic syndrome was first detected. This was resistant to conventional treatment for more than 10 years. During the course of the disease, the hyperlipidaemia characteristic of hyper-pre-beta-lipoproteinaemia and elevation of apoprotein E persisted, and renal function gradually deteriorated. The renal histopathological findings from four biopsies were essentially the same, with storage of beta-lipoprotein in dilated, balloon-like glomerular capillary lumina. However, the number of glomeruli showing global sclerosis increased and tubulo-interstitial changes progressed in parallel with the gradual clinical deterioration. As in other cases reported in Japan some familial involvement has been noted.
ISSN:0931-041X
1432-198X
DOI:10.1007/BF00858822