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.
Koitabashi
Y
Y
Department of Paediatrics, St. Marianna University School of Medicine, Kanagawa, Japan.
Ikoma
M
M
Miyahira
T
T
Fujita
R
R
Mio
H
H
Ishida
M
M
Shimizu
K
K
Sakaguchi
H
H
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