Abstract:
BACKGROUND:Females with Ullrich-Turner syndrome (UTS) have typical clinical features such as short stature, ovarian failure, visible dysmorphic stigmata, and abnormalities in different organs such as kidney or heart. HYPOTHESIS:The aim of the present study was to analyze the distribution, prevalence, and relative risk of cardiovascular anomalies (CVA) in females with Ullrich-Turner syndrome (UTS) seen at one single center compared with that of the regional Bavarian population. METHODS:The associations between CVA and karyotype were determined. In all, 117 girls and women with UTS, aged between 3 and 43 years (median 17.4 years) were studied retrospectively. The detailed cardiologic status including echocardiography was available in all patients. The prevalences of each cardiovascular anomaly were determined. On the basis of published epidemiologic data of CVA in Bavarian children, we assessed the relative risks of each CVA. RESULTS:Thirty-five (29.9%) girls with UTS had at least one CVA. In all of these CVAs, coarctation of the aorta and bicuspid aortic valve occurred most often (18.5% each). The aortic malformations represented over two-thirds of all CVA (72.8%), whereas anomalies of the septum (8.6%), mitral valve (6.2%), pulmonary veins (4.9%), and other locations together accounted for the other third. Bicuspid aortic valve and partial anomalous pulmonary venous drainage were associated with the highest relative risk (RR) (3603 and 1293, respectively) compared with the Bavarian population. The overall RR of CVA was 48.7. Of the 117 girls and women examined, 64 (54.7%) had complete monosomy 45 X. CONCLUSIONS:Our data demonstrate that about every third female with UTS is affected with at least one CVA, mainly left sided and associated with aortic structures. Our results underline the necessity of thorough cardiologic evaluation.
journal_name
Clin Cardioljournal_title
Clinical cardiologyauthors
Völkl TM,Degenhardt K,Koch A,Simm D,Dörr HG,Singer Hdoi
10.1002/clc.4960280209subject
Has Abstractpub_date
2005-02-01 00:00:00pages
88-92issue
2eissn
0160-9289issn
1932-8737journal_volume
28pub_type
杂志文章abstract::In this editorial, I would like to share some thoughts about clinical studies, including value and limitations using as an example a recently published randomized clinical trial. Randomized and non-randomized clinical trials, epidemiologic studies, and registries guide our clinical practice. They are good but not perf...
journal_title:Clinical cardiology
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abstract:BACKGROUND:Takotsubo cardiomyopathy (TCM) is characterized by systolic ballooning of the left ventricular apex. It is triggered by emotional or physical stress, but the exact mechanism through which stress leads to TCM is not known. HYPOTHESIS:Coronary microvessel apoptosis is the missing link between stress and TCM. ...
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journal_title:Clinical cardiology
pub_type: 杂志文章,评审
doi:10.1002/clc.4960220409
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abstract::We report four patients who developed severe adverse reactions to protamine sulfate following cardiac surgery. Two types of reactions were seen. First, an immediate anaphylaxis which is a complement-dependent IgG antibody-mediated reaction. In the literature, 80% of patients who had similar reactions have had previous...
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doi:10.1002/clc.4960070305
更新日期:1984-03-01 00:00:00
abstract::Several investigators have documented the successful use of oral sustained-release theophylline in treating symptomatic bradycardia and sick sinus syndrome. This paper reports a case of chronotropic incompetence in which specific exercise indices, including the chronotropic response index, were used to measure the the...
journal_title:Clinical cardiology
pub_type: 临床试验,杂志文章,评审
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更新日期:2000-10-01 00:00:00
abstract::Flecainide is reported to be effective in patients with paroxysmal tachycardias, but its effect on rapid ventricular response over accessory atrioventricular pathway during atrial fibrillation is not known. The influence of flecainide on various electrophysiological properties of the accessory pathway with special emp...
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journal_title:Clinical cardiology
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:1991-01-01 00:00:00
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journal_title:Clinical cardiology
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journal_title:Clinical cardiology
pub_type: 杂志文章,随机对照试验
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更新日期:2010-03-01 00:00:00
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doi:10.1002/clc.4960210209
更新日期:1998-02-01 00:00:00
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pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1994-10-01 00:00:00
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更新日期:2009-06-01 00:00:00
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更新日期:2003-04-01 00:00:00
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journal_title:Clinical cardiology
pub_type: 杂志文章,评审
doi:
更新日期:1990-04-01 00:00:00
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pub_type: 杂志文章
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pub_type: 临床试验,杂志文章
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更新日期:1996-12-01 00:00:00
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更新日期:1999-01-01 00:00:00
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doi:10.1002/clc.4960080902
更新日期:1985-09-01 00:00:00