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意料之外的高患病率:安德森-法布里病患者中的冠状动脉痉挛性心绞痛。

Unexpectedly High Prevalence of Coronary Spastic Angina in Patients With Anderson-Fabry Disease.

机构信息

Division of Cardiology, Nephrology, Pulmonology and Neurology, Department of Internal Medicine, Asahikawa Medical University.

Department of Cardiology, Rumoi City Hospital.

出版信息

Circ J. 2019 Jan 25;83(2):481-484. doi: 10.1253/circj.CJ-18-0734. Epub 2018 Dec 18.

DOI:10.1253/circj.CJ-18-0734
PMID:30568064
Abstract

BACKGROUND

Although we and others have reported cases of patients with Anderson-Fabry disease (AFD) complicated by coronary spastic angina (CSA), the prevalence of CSA in these patients remains unknown.

METHODS AND RESULTS

We performed the acetylcholine-induced provocation test, according to the Japanese guidelines for the diagnosis and treatment of patients with CSA, in 9 consecutive patients having 5 independent AFD pedigrees. Coronary spasms were provoked in conjunction with symptoms and ECG ischemic changes in 8 of 9 (89%) patients with AFD.

CONCLUSIONS

We found an unexpectedly high prevalence of CSA in patients with AFD.

摘要

背景

虽然我们和其他人已经报告了伴有安德森-法布里病(AFD)的患者发生冠状动脉痉挛性心绞痛(CSA)的病例,但这些患者中 CSA 的患病率尚不清楚。

方法和结果

我们按照日本 CSA 患者诊断和治疗指南,对 9 例具有 5 个独立 AFD 家系的患者进行了乙酰胆碱激发试验。9 例 AFD 患者中的 8 例(89%)在出现症状和心电图缺血改变的同时诱发了冠状动脉痉挛。

结论

我们发现 AFD 患者中 CSA 的患病率出乎意料地高。

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