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 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.
Department of Paediatrics, St. Marianna University School of Medicine, Kanagawa, Japan.
Y
Y
Koitabashi
M
M
Ikoma
T
T
Miyahira
R
R
Fujita
H
H
Mio
M
M
Ishida
K
K
Shimizu
H
H
Sakaguchi
D001057:Apolipoproteins E / Q000097:blood; D002648:Child; D006801:Humans; D006953:Hyperlipoproteinemia Type IV / Q000473:pathology / Q000503:physiopathology; D007674:Kidney Diseases / Q000473:pathology / Q000503:physiopathology*; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology; D008074:Lipoproteins / Q000378:metabolism*; D008297:Male; D008854:Microscopy, Electron; D009404:Nephrotic Syndrome / Q000473:pathology / Q000503:physiopathology; D013997:Time Factors
D001057:Apolipoproteins E; D008074:Lipoproteins