HEART IN FABRYS DISEASE - A HISTOCHEMICAL AND ELECTRON MICROSCOPIC STUDY

被引：115

作者：

FERRANS, VJ

HIBBS, RG

BURDA, CD

机构：

[1] Departments of Medicine and Anatomy, Tulane University School of Medicine, New Orleans, LA

[2] Confederate Memorial Medical Center, Shreveport, LA

来源：

AMERICAN JOURNAL OF CARDIOLOGY | 1969年 / 24卷 / 01期

关键词：

D O I：

10.1016/0002-9149(69)90055-1

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Histochemical and electron microscopic studies of the heart of a patient with Fabry's disease (angiokeratoma corporis diffusum universale) revealed the presence of extensive glycolipid deposits in the cardiac muscle fibers, vascular smooth muscle, endothelium and connective tissue cells of the mitral valve. These deposits formed lamellae arranged either concentrically or in parallel stacks; the lamellae were often enclosed by limiting membranes and showed a periodic spacing of 40 to 45 Å. The cardiovascular manifestations of Fabry's disease, which consist of cardiac hypertrophy and dilatation, congestive heart failure, anginal chest pain, myocardial infarction and hypertensive heart disease, are reviewed in detail. The association of this disorder with valvular heart disease is discussed. © 1969.

引用

页码：95 / &

共 83 条

[1] PHYTANIC ACID IN REFSUMS SYNDROME [J].