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

来源：

PEDIATRIC NEPHROLOGY | 1990年 / 4卷 / 02期

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

页码：122 / 128

页数：7

共 26 条

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