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伴有完全性房室传导阻滞的法布里病：传导系统受累的组织学证据。

Fabry's disease with complete atrioventricular block: histological evidence of involvement of the conduction system.

作者信息

Ikari Y, Kuwako K, Yamaguchi T

机构信息

Division of Cardiology, Mitsui Memorial Hospital, Tokyo, Japan.

出版信息

Br Heart J. 1992 Sep;68(3):323-5. doi: 10.1136/hrt.68.9.323.

DOI:10.1136/hrt.68.9.323
PMID:1389767
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1025080/
Abstract

A 63 year old man with complete atrioventricular block was diagnosed as having Fabry's disease. A short PR interval is a common electrocardiographic finding in Fabry's disease, but complete atrioventricular block is a very rare complication. Necropsy indicated that lipid accumulation in the atrioventricular conduction system was the probable cause of this patient's atrioventricular block.

摘要

一名63岁的完全性房室传导阻滞男性被诊断为法布里病。短PR间期是法布里病常见的心电图表现，但完全性房室传导阻滞是一种非常罕见的并发症。尸检表明，房室传导系统中的脂质积聚可能是该患者房室传导阻滞的原因。

相似文献

1
Fabry's disease with complete atrioventricular block: histological evidence of involvement of the conduction system.伴有完全性房室传导阻滞的法布里病：传导系统受累的组织学证据。
Br Heart J. 1992 Sep;68(3):323-5. doi: 10.1136/hrt.68.9.323.
2
Sisters with atypical Fabry's disease with complete atrioventricular block.患有完全性房室传导阻滞的非典型法布里病姐妹。
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First-degree atrioventricular block and restrictive physiology as cardiac manifestations of Fabry's disease.一度房室传导阻滞和限制性生理学表现作为法布里病的心脏表现。
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4
[Complete atrioventricular block disclosing Fabry's disease].
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[Echocardiographic findings in a case of Fabry's disease with aortic regurgitation and complete AV block, and in his family members].[1例伴有主动脉反流和完全性房室传导阻滞的法布里病患者及其家庭成员的超声心动图表现]
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The abbreviated PR interval of Fabry's disease.
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Combined atrioventricular block and sinus node dysfunction in Fabry's disease.
Am Heart J. 1990 Aug;120(2):438-40. doi: 10.1016/0002-8703(90)90096-g.

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本文引用的文献

1
The abbreviated PR interval of Fabry's disease.
N Engl J Med. 1973 Aug 16;289(7):357-8. doi: 10.1056/NEJM197308162890709.
2
Cardiovascular manifestations in Fabry's disease. A high incidence of mitral valve prolapse in hemizygotes and heterozygotes.法布里病的心血管表现。半合子和杂合子中二尖瓣脱垂的高发病率。
Clin Genet. 1986 Apr;29(4):276-83.
3
Combined atrioventricular block and sinus node dysfunction in Fabry's disease.
Am Heart J. 1990 Aug;120(2):438-40. doi: 10.1016/0002-8703(90)90096-g.
4
Cardiac valvular anomalies in Fabry disease. Clinical, morphologic, and biochemical studies.
Circulation. 1976 Nov;54(5):818-25. doi: 10.1161/01.cir.54.5.818.
5
Cardiac manifestations of Fabry's disease. Report of a case with mitral insufficiency and electrocardiographic evidence of myocardial infarction.法布里病的心脏表现。二尖瓣关闭不全及心肌梗死心电图证据病例报告。
Am J Cardiol. 1975 Nov;36(6):829-35. doi: 10.1016/0002-9149(75)90468-3.
6
Cardiac manifestations of Fabry's disease. Report of a case with pulmonary regurgitation diagnosed on the basis of endomyocardial biopsy findings.
Jpn Circ J. 1977 Sep;41(9):1023-36.
7
Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease.法布里病的心电图和向量心电图异常
Am Heart J. 1977 Jun;93(6):699-705. doi: 10.1016/s0002-8703(77)80064-1.