LONG-TERM FOLLOW-UP OF A PEDIATRIC CASE OF LIPOPROTEIN GLOMERULOPATHY

被引:24
作者
KOITABASHI, Y
IKOMA, M
MIYAHIRA, T
FUJITA, R
MIO, H
ISHIDA, M
SHIMIZU, K
SAKAGUCHI, H
机构
[1] Department of Paediatrics, St. Marianna University School of Medicine, Kawasaki, Kanagawa, 213, 2-16-1 Sugao, Miyamae-ku
[2] First Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Kanagawa, 213, 2-16-1 Sugao, Miyamae-ku
[3] Division of Pathology, Department of Laboratory Medicine, National Children's Hospital, Tokyo
[4] Department of Pathology, Keio University School of Medicine, Tokyo
关键词
Apoprotein E; Glomerular lipoprotein thrombi; Hyper-pre-β-lipoproteinaemia; Lipoprotein glomerulopathy; Mesangiolysis; Nephrotic syndrome;
D O I
10.1007/BF00858822
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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-β-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 β-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. © 1990 IPNA.
引用
收藏
页码:122 / 128
页数:7
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