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法布里病的心电图

Electrocardiograms in Fabry's disease.

作者信息

Sheth K J, Thomas J P

出版信息

J Electrocardiol. 1982 Apr;15(2):153-6. doi: 10.1016/s0022-0736(82)80010-1.

DOI:10.1016/s0022-0736(82)80010-1
PMID:6461709
Abstract

We studied the ECGs of 47 kindred members from three families with Fabry's disease. The control group (19) with normal plasma alpha-galactosidase levels had normal ECGs. Of the 12 affected males (mean age 15 years, plasma alpha-galactosidase levels less than 10% of normals), ten had ECG evidence of LVH, and five had ST-T wave changes. The conduction time of the cardiac impulse was accelerated (decreased PR index) in one male. Of the 16 heterozygous females (mean age 19 years, plasma alpha-galactosidase level and alpha/beta galactosidase ratio in the carrier range) none had ECG evidence of LVH, but six had conduction abnormalities--decreased PR index (four) and delayed conduction (two). The ECG abnormalities were uniformly distributed in different age groups.

摘要

我们研究了来自三个患有法布里病家族的47名亲属的心电图。血浆α-半乳糖苷酶水平正常的对照组(19人)心电图正常。12名受影响男性(平均年龄15岁,血浆α-半乳糖苷酶水平低于正常水平的10%)中,10人有左心室肥厚的心电图证据,5人有ST-T波改变。一名男性的心脏冲动传导时间加快(PR指数降低)。16名杂合子女性(平均年龄19岁,血浆α-半乳糖苷酶水平和α/β半乳糖苷酶比率在携带者范围内)均无左心室肥厚的心电图证据,但6人有传导异常——PR指数降低(4人)和传导延迟(2人)。心电图异常在不同年龄组中分布均匀。

相似文献

1
Electrocardiograms in Fabry's disease.法布里病的心电图
J Electrocardiol. 1982 Apr;15(2):153-6. doi: 10.1016/s0022-0736(82)80010-1.
2
Molecular genetic, biochemical, and clinical studies in three families with cardiac Fabry's disease.对三个患有心脏法布里病的家族进行的分子遗传学、生物化学和临床研究。
Am J Cardiol. 2001 Jan 1;87(1):71-5. doi: 10.1016/s0002-9149(00)01275-3.
3
Cardiopulmonary involvement in Fabry's disease.法布里病的心肺受累情况。
Acta Cardiol. 2010 Apr;65(2):185-92. doi: 10.2143/AC.65.2.2047052.
4
Accelerated atrioventricular conduction in Fabry's disease: a case report.法布里病中的加速性房室传导:一例报告
Angiology. 1978 Jul;29(7):562-8. doi: 10.1177/000331977802900709.
5
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.
6
A case of heterozygous Fabry's disease with a short PR interval and giant negative T waves.一例伴有短PR间期和巨大负向T波的杂合子法布里病。
Br Heart J. 1987 Mar;57(3):296-9. doi: 10.1136/hrt.57.3.296.
7
New insights in cardiac structural changes in patients with Fabry's disease.法布里病患者心脏结构变化的新见解。
Am Heart J. 2000 Jun;139(6):1101-8. doi: 10.1067/mhj.2000.105105.
8
Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome.一名伴有预激综合征的杂合性法布里病患者的15年随访
Intern Med. 1999 Jun;38(6):476-81. doi: 10.2169/internalmedicine.38.476.
9
Ocular manifestations of Fabry's disease: data from the Fabry Outcome Survey.法布里病的眼部表现:来自法布里病结局调查的数据。
Br J Ophthalmol. 2007 Feb;91(2):210-4. doi: 10.1136/bjo.2006.100602. Epub 2006 Sep 14.
10
Fabry's disease in a black kindred.一个黑人家族中的法布里病。
Am J Dis Child. 1979 Nov;133(11):1178-81. doi: 10.1001/archpedi.1979.02130110086017.

引用本文的文献

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Storage diseases with hypertrophic cardiomyopathy phenotype.具有肥厚型心肌病表型的贮积病。
Glob Cardiol Sci Pract. 2018 Aug 12;2018(3):28. doi: 10.21542/gcsp.2018.28.
2
Fabry disease.法布里病。
Orphanet J Rare Dis. 2010 Nov 22;5:30. doi: 10.1186/1750-1172-5-30.
3
Twenty-four-month alpha-galactosidase A replacement therapy in Fabry disease has only minimal effects on symptoms and cardiovascular parameters.法布里病患者接受为期24个月的α-半乳糖苷酶A替代疗法,对症状和心血管参数仅有极小的影响。
J Inherit Metab Dis. 2008 Jun;31(3):432-41. doi: 10.1007/s10545-008-0848-3. Epub 2008 May 23.
4
Improvement in serial cardiopulmonary exercise testing following enzyme replacement therapy in Fabry disease.法布里病患者接受酶替代治疗后连续心肺运动试验的改善情况。
J Inherit Metab Dis. 2006 Aug;29(4):572-9. doi: 10.1007/s10545-006-0361-5.
5
Cardiac manifestations in Fabry disease.法布里病的心脏表现。
J Inherit Metab Dis. 2001;24 Suppl 2:75-83; discussion 65. doi: 10.1023/a:1012428009627.