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including short stature and gonadal failure &#40;the cardinal features&#41; and congenital cardiovascular &#40;CV&#41; defects as well as an abnormal karyotype&#46;<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The most serious consequences of X-chromosome haploinsufficiency involve the CV system&#46; Indeed&#44; CV defects&#44; present in up to 50&#37; of the TS population&#44; are the major cause of premature death and contribute to a standardized mortality ratio three times higher than in the general female population&#46; Therefore&#44; imaging studies &#40;both echocardiography and cardiovascular magnetic resonance &#91;CMR&#93;&#41; are crucial for the timely detection of often subclinical CV disease and should preferably be ordered before the onset of symptomatic and irreversible organ damage&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;4&#44;5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">A thorough cardiac examination is recommended for all women with TS every 5 to 10 years&#44; at the time of transition from pediatric to adult care&#44; before attempting pregnancy or if hypertension &#40;HTN&#41; develops&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The aim of this paper is to review both congenital and acquired cardiovascular diseases and the role of clinical imaging in TS CV risk stratification with recommendations for CV screening&#46; Moreover&#44; particular attention will be given to the special risk of CV complications in pregnancy with this syndrome&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Methods</span><p id="par0035" class="elsevierStylePara elsevierViewall">This review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses &#40;PRISMA&#41; guidelines&#44; thus providing a comprehensive framework that objectively assesses quality indicators and risk of bias in the studies included&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">All original studies investigating Turner syndrome and cardiovascular risk were eligible for this systematic review&#46; Further selection criteria were&#58; &#40;i&#41; publication date between January 2000 and September 2016&#44; &#40;ii&#41; written in English&#44; &#40;iii&#41; published in a scholarly peer-reviewed journal&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Studies were found by searching the PubMed&#44; EMBASE and Cochrane Library electronic databases&#44; using the following search strategy&#58; &#8220;Turner syndrome AND Cardiovascular disease AND Risk factors AND Female&#8221;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Studies were identified by searching relevant papers via PubMed&#47;MEDLINE &#40;<a id="intr0005" class="elsevierStyleInterRef" href="http://www.ncbi.nlm.nih.gov/pubmed">http&#58;&#47;&#47;www&#46;ncbi&#46;nlm&#46;nih&#46;gov&#47;pubmed</a>&#41;&#44; the Cochrane Library and EMBASE using the following search strategy&#58; &#8220;Turner syndrome AND Cardiovascular disease AND Risk factors&#46; Finally&#44; reference lists of the studies retrieved were manually searched in order to detect any additional relevant studies&#46; Keywords and combinations of keywords were used to search the electronic databases &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">After performing the initial literature searches&#44; each study title and abstract was screened for eligibility by the first author&#46; The full texts of all potentially relevant studies were subsequently retrieved and examined further for eligibility&#46; The PRISMA flow diagram &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41; provides more detailed information regarding the process for selecting studies&#46; Sixty articles were included in this review and the information from the studies included was then analyzed and recorded&#46; <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> shows a summary of the literature consulted&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Congenital cardiovascular disorders</span><p id="par0060" class="elsevierStylePara elsevierViewall">Variations in the CV anatomy of patients with TS&#44; the frequencies of which are summarized in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#44; are major factors in their reduced life expectancy&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a></p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">Major defects in cardiac and aortic development during fetal life are associated with miscarriage in most cases of fetuses with 45X karyotype&#46; Fetuses with CV failure almost always demonstrate obstructed jugular lymphatics with increased nuchal translucency or nuchal cystic hygromas&#44; the typical intrauterine presentation of TS&#46; The residual postnatal webbing of the neck predicts CV defects&#46; This association suggests a pathogenetic connection between fetal lymphatic obstruction and defective CV development due to the compression or obstruction of the outflow tracts&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;8&#44;9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">However&#44; this is just a theory regarding a pathogenetic mechanism for congenital CV defects&#44; because the real causes are currently unknown&#46;<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">1</span></a></p><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">A&#46; Valvular anomaly</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">A&#46;1&#46; Bicuspid aortic valve</span><p id="par0075" class="elsevierStylePara elsevierViewall">A bicuspid aortic valve &#40;BAV&#41; is a congenital valvular anomaly found in approximately 30&#37; of TS patients and it is not often clinically apparent&#46;<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">14</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Identifying a BAV in asymptomatic individuals is important because they are at increased risk of infective endocarditis&#44; hemodynamically significant stenosis &#40;promoted by accelerated valve calcification&#41;&#44; valve regurgitation &#40;secondary to poor leaflet coaptation&#41; and aortic aneurysm &#40;due to the vicious cycle of increased stroke volume that promotes aortic root dilatation&#41;&#46; For that reason&#44; the anomaly requires regular medical evaluation and possibly surgery to prevent aortic dissection or rupture&#46;<a class="elsevierStyleCrossRefs" href="#bib0370"><span class="elsevierStyleSup">14&#44;16</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Indeed&#44; the aortic valve leaflet must be clearly visualized and echocardiography is the primary test for BAV that has been reported to be quite sensitive in diagnosing this anomaly in 89&#37; of women with TS&#46; However&#44; echocardiography can be inadequate to view the aortic valve and&#44; in these cases&#44; CMR should be the next approach&#46;<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;14</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">In almost all the cases&#44; BAV results from fusion of the right and left coronary leaflets&#44; while the fused right coronary and non-coronary leaflets variant is much less common<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;14</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">B&#46; Aortic abnormalities</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">B&#46;1&#46; Coarctation of the aorta</span><p id="par0095" class="elsevierStylePara elsevierViewall">Coarctation of the aorta &#40;CoA&#41; affects around 12&#37; of women with TS<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a> and is usually diagnosed in infancy&#44; often with congestive heart failure in critical cases<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">17</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0100" class="elsevierStylePara elsevierViewall">Indeed&#44; in contrast to BAV&#44; which is detected by screening&#44; CoA is usually diagnosed based on clinical grounds &#8211; HTN and brachial-femoral delay are common features&#46; However&#44; since many cases are detected later in life&#44; any woman with suspected CoA should have a CMR or computed tomography &#40;CT&#41; angiography with three-dimensional reconstruction of the thoracic aorta&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;11</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Concentric narrowing of the aortic lumen leads to the development of pressure gradients across the lesion&#44; with the possibility of arterial collaterals becoming a detour for oxygenated blood traveling to the lower segments of the body&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">If left untreated&#44; complications such as HTN&#44; congestive heart failure&#44; aortic dissection and aortic rupture may occur&#46; Surgical intervention is required when peak-to-peak coarctation gradient is &#8805;20 mmHg&#44; or &#60;20 mmHg with radiological evidence of significant collateral flow&#46; Even after surgical repair&#44; there is an increased risk of HTN&#44; coronary artery disease &#40;CAD&#41;&#44; cerebrovascular disease&#44; aortic dissection and restenosis&#46;<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">18</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">B&#46;2&#46; Elongated transverse arch</span><p id="par0115" class="elsevierStylePara elsevierViewall">Elongated transverse arch &#40;ETA&#41; is a silent anomaly&#44; detected by CMR&#44; defined as an increased distance between the origin of the left common carotid and left subclavian arteries&#44; with flattening of the arch and kinking along the inferior curvature&#46; This distinct anatomy&#44; sometimes referred to as &#8220;pseudocoarctation&#8221;&#44; is embryologically similar to coarctation and may predict aortic complications&#44; such as aortic dilation&#44; aortic dissection or progression to overt coarctation&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;11</span></a> However&#44; ETA differs from true coarctation in that there is no true luminal narrowing&#44; nor are there pressure gradients or collateral circulation&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">ETA has been significantly associated with BAV&#44; CoA and also with aortic sinus dilatation&#46;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a></p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">B&#46;3&#46; Aortic dilatation</span><p id="par0125" class="elsevierStylePara elsevierViewall">Aortic dilatation is reported in 23&#37; of women with TS&#46;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall">Risk factors for this and&#44; secondarily&#44; dissection include HTN&#44; BAV&#44; CoA&#44; 45&#44;X karyotype and pregnancy&#46; Aortic dilatation may less commonly occur alone in approximately 5&#37; of women with TS&#46; This indicates that all women with TS should be periodically monitored&#46;<a class="elsevierStyleCrossRefs" href="#bib0325"><span class="elsevierStyleSup">5&#44;19&#8211;21</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">In TS patients with enlargement of the ascending aorta&#44; a general aortopathy is present&#44; which is accelerated by the presence of a BAV&#46;<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">22</span></a></p><p id="par0140" class="elsevierStylePara elsevierViewall">It has been suggested that patients with an enlarged aortic root should undergo echocardiographic evaluation every year and that patients with normal aortic root dimensions should be evaluated every 2 to 3 years&#46;<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">23</span></a></p><p id="par0145" class="elsevierStylePara elsevierViewall">Aortic diameter&#44; corrected for age and body size&#44; is accurate for predicting aortic events&#44; and is the principal risk marker for aortic dissection&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;5</span></a> An ascending to descending aortic diameter ratio above 1&#46;5 signifies ascending aorta dilatation if descending aorta diameter is normal&#46; There is another alternative to identifying aortic dilatation that correlates more closely with aortic diameter&#44; because it adjusts aortic dimensions for body surface area &#40;BSA&#41;&#59; this is important due to the relatively short stature of patients with TS&#46; This latter method involves estimating the aortic size index &#40;ASI&#41;&#58; aortic diameter &#40;at the ascending aorta or at the site with the largest dimension&#41;&#47;BSA&#46; It has been proposed that an ASI &#8805;2 cm&#47;m<span class="elsevierStyleSup">2</span> identifies those who require close monitoring and an ASI &#8805;2&#46;5 cm&#47;m<span class="elsevierStyleSup">2</span> &#40;as in <a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41; requires aortic surgery to prevent aortic dissection&#46;<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;21&#44;24&#44;25</span></a></p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">B&#46;4&#46; Aortic dissection</span><p id="par0150" class="elsevierStylePara elsevierViewall">Although rare&#44; aortic dissection is a major concern in TS&#46; It has an estimated incidence of 40 cases per 10000 patients&#44; but is often fatal&#46; It occurs at a much earlier age&#59; in the general population&#44; the peak incidence of dissection occurs between ages 50 and 80 and in TS population median age is 35&#44; with higher incidence rates at ages 20&#8211;29 and 30&#8211;39&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;26</span></a></p><p id="par0155" class="elsevierStylePara elsevierViewall">Up to 90&#37; of aortic dissections have predisposing CV risk factors &#40;BAV&#44; aortic dilatation and CoA&#41; that can be identified with CMR imaging&#46; HTN and pregnancy are associated with a higher risk of dissection&#44; the former due to an association with increased aortic root diameter and the latter due to physiological adaptation&#44; which imposes a higher cardiac workload&#46;<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;19</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">However&#44; up to 25&#37; of cases have no apparent risk factors other than TS itself&#44; suggesting that TS phenotype may also include a primary aortopathy&#44; which can be present as early as 9 years of age&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">18&#44;26&#8211;28</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall">Indeed&#44; histological evidence of cystic medial necrosis in aortic tissue taken from patients with BAV&#44; Marfan syndrome and TS indicates a shared etiology despite genetically diverse backgrounds&#46;<a class="elsevierStyleCrossRef" href="#bib0445"><span class="elsevierStyleSup">29</span></a></p><p id="par0170" class="elsevierStylePara elsevierViewall">Aortic dissection is sometimes overlooked at onset because it could include apparently minor complaints such as abdominal pain&#44; heartburn&#44; back or shoulder pain or a voice change due to traction of the recurrent laryngeal nerve&#46; Therefore&#44; patients should be advised to go to the emergency department soon after experiencing sustained chest pain &#40;&#62;30<span class="elsevierStyleHsp" style=""></span>min&#41;&#44; regardless of the severity of symptoms&#46;<a class="elsevierStyleCrossRefs" href="#bib0385"><span class="elsevierStyleSup">17&#44;29</span></a></p></span></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">C&#46; Venous anomalies</span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">C&#46;1&#46; Partial anomalous pulmonary venous connection</span><p id="par0175" class="elsevierStylePara elsevierViewall">In TS&#44; partial anomalous pulmonary venous connection may be right-sided&#44; which is the most typical in the general population&#46; Nevertheless&#44; it frequently involves the left upper pulmonary vein&#44; which makes echocardiographic detection more challenging&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;30</span></a></p><p id="par0180" class="elsevierStylePara elsevierViewall">When the pulmonary veins connect to the right atrium or with one of its venous tributaries&#44; blood from the pulmonary circulation returns to the right atrium and this may become clinically significant in adult life&#46; Indeed&#44; if sufficiently severe&#44; pulmonary HTN and right ventricular volume overload can lead to congestive heart failure&#44; which has been related to death in middle-aged TS patients secondary to undiagnosed partial anomalous pulmonary venous return lesions&#46; Echocardiography and ideally CMR imaging should therefore be performed to assess pulmonary vein drainage patterns&#46;<a class="elsevierStyleCrossRefs" href="#bib0450"><span class="elsevierStyleSup">30&#44;31</span></a></p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">C&#46;2&#46; Persistent left superior vena cava</span><p id="par0185" class="elsevierStylePara elsevierViewall">The venous return from a persistent left superior vena cava &#40;PLSVC&#59; <a class="elsevierStyleCrossRef" href="#fig0025">Figure 5</a>&#41; has a prevalence of around 13&#37;&#46; In approximately 80&#37; to 92&#37; of cases&#44; the PLSVC drains into the right atrium via the coronary sinus&#44; resulting in no hemodynamic consequences&#46; However&#44; in about 10&#37; to 20&#37; of cases&#44; it drains into the left atrium and results in venous blood returning to the left atrium&#46;<a class="elsevierStyleCrossRefs" href="#bib0355"><span class="elsevierStyleSup">11&#44;32</span></a></p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0190" class="elsevierStylePara elsevierViewall">It is important to always report this irregular anatomy&#44; as it needs to be taken into account during interventional procedures&#46;<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">32</span></a></p></span></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">D&#46; Other congenital cardiovascular disorders</span><p id="par0195" class="elsevierStylePara elsevierViewall">Cardiac conduction defects are also common&#44; caused by left ventricular hypertrophy&#44; myocardial ischemia&#44; previous myocardial infarction and congenital cardiac malformations&#46;<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">33</span></a></p><p id="par0200" class="elsevierStylePara elsevierViewall">Other associated lesions can be found in TS&#44; such as ventricular and atrial septal defects&#44; hypoplastic left heart syndrome&#44; single ventricle&#44; mitral valve abnormalities&#44; coronary artery abnormalities and aberrant right subclavian artery<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">4</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0030">Figure 6</a>&#41;&#46;</p><elsevierMultimedia ident="fig0030"></elsevierMultimedia></span></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Cardiovascular risk</span><p id="par0205" class="elsevierStylePara elsevierViewall">In addition to potentially harmful CV malformations&#44; women with TS display evidence of adverse CV risk profiles that expose them to greater risk of CAD with a relative risk &#40;RR&#41; of 2-11&#44; cerebrovascular disease with a RR of 2-7&#44; HTN and types 1 and 2 diabetes mellitus&#46;<a class="elsevierStyleCrossRefs" href="#bib0380"><span class="elsevierStyleSup">16&#44;18&#44;34&#44;35</span></a></p><p id="par0210" class="elsevierStylePara elsevierViewall">The precise mechanisms of increased CV risk in TS are unclear&#44; but estrogen deficiencies &#40;ensuing from premature ovarian failure due to gonadal dysgenesis&#41; and haploinsufficiency of the X chromosome seem to be major factors that distinguish this metabolic phenotype&#46;<a class="elsevierStyleCrossRefs" href="#bib0475"><span class="elsevierStyleSup">35&#8211;37</span></a></p><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">A&#46; Specific aspects of vascular structure and physiology</span><p id="par0215" class="elsevierStylePara elsevierViewall">Women with TS have greater intima-media thickness and conduit artery diameters than normal controls&#46;<a class="elsevierStyleCrossRef" href="#bib0480"><span class="elsevierStyleSup">36</span></a> The stiffness of the ascending aorta is also increased in these patients&#46;<a class="elsevierStyleCrossRef" href="#bib0490"><span class="elsevierStyleSup">38</span></a></p><p id="par0220" class="elsevierStylePara elsevierViewall">Estrogen deficiency may contribute to this&#44; as hormone replacement therapy &#40;HRT&#41; appears to reduce aortic stiffness&#44;<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">39</span></a> decrease intima-media thickness<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">40</span></a> and improve endothelial function&#46;<a class="elsevierStyleCrossRef" href="#bib0505"><span class="elsevierStyleSup">41</span></a> Indeed&#44; gonadal dysgenesis may be the key determinant of neointimal hyperplasia in these subjects&#44; because it has been shown that increasing doses of HRT results in a reduction in carotid intima-media thickness in young hypogonadal women&#44; which raises the possibility that exogenous estrogen may be cardioprotective<a class="elsevierStyleCrossRefs" href="#bib0480"><span class="elsevierStyleSup">36&#44;40</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0225" class="elsevierStylePara elsevierViewall">Relative resistance to growth hormone &#40;GH&#41; effects and an imbalance in the GH&#47;insulin-like growth factor I axis could also occur&#44; which may have a negative impact on cardiovascular prognosis&#46; In fact&#44; GH replacement &#40;the accepted treatment for the short stature of TS girls&#41; decreases intima-media thickness at major arteries and improves endothelial dysfunction&#44; which are considered surrogate markers of atherosclerosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0380"><span class="elsevierStyleSup">16&#44;42</span></a></p></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">B&#46; Glucose metabolism&#44; body composition and physical activity</span><p id="par0230" class="elsevierStylePara elsevierViewall">TS girls have a greater risk of developing diabetes&#44; which is related to both the impaired fasting glucose and the impaired glucose tolerance detected among these girls&#46;<a class="elsevierStyleCrossRef" href="#bib0515"><span class="elsevierStyleSup">43</span></a> Moreover&#44; those who have normal fasting glucose concentrations are more insulin resistant than controls&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">44</span></a></p><p id="par0235" class="elsevierStylePara elsevierViewall">The effect of HRT on insulin sensitivity remains unclear&#44; but insulin resistance is a karyotype-dependent factor&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">44</span></a> Furthermore&#44; GH therapy has been shown to reduce insulin sensitivity&#44; antagonizing the effects of insulin on glucose and lipid metabolism via GH receptors&#46; Thus&#44; high GH levels induce peripheral and hepatic insulin resistance&#46; However&#44; patients with TS develop an adaptation to long periods of GH therapy&#44; so insulin sensitivity increases&#46; Upon therapy cessation&#44; insulin sensitivity also increases and returns to pre-treatment levels&#46;<a class="elsevierStyleCrossRef" href="#bib0525"><span class="elsevierStyleSup">45</span></a></p><p id="par0240" class="elsevierStylePara elsevierViewall">Additionally&#44; compared to normal controls&#44; TS patients have a greater waist circumference and higher absolute body mass index&#44; which are associated with an increased risk of type 2 diabetes and metabolic dysfunction&#46;<a class="elsevierStyleCrossRef" href="#bib0515"><span class="elsevierStyleSup">43</span></a> They also tend to be less physically active&#44; thereby contributing to the increased frequency of obesity&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">18&#44;46</span></a></p></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">C&#46; Lipid profile</span><p id="par0245" class="elsevierStylePara elsevierViewall">TS patients have an abnormal lipid profile with higher total cholesterol levels&#44; which contributes to the onset of atherosclerosis and also confers an increased risk for CV disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0515"><span class="elsevierStyleSup">43&#44;44&#44;47&#44;48</span></a></p><p id="par0250" class="elsevierStylePara elsevierViewall">A dose-related relationship between HRT and high-density lipoprotein concentrations has been observed&#44;<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">40</span></a> elucidating the benefits of HRT on CV outcomes by improving the lipid profile&#46; Moreover&#44; the impact of estrogens on lipid metabolism is also determined by diminishing lipid oxidation and increasing triglyceride synthesis&#46; However&#44; in one study&#44; the application of HRT in women with TS had no effect on lipid metabolism&#46;<a class="elsevierStyleCrossRef" href="#bib0545"><span class="elsevierStyleSup">49</span></a></p></span><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">D&#46; Hypertension</span><p id="par0255" class="elsevierStylePara elsevierViewall">HTN can be present in TS patients of any age and has been reported in around 42&#37; of patients&#46; As TS alone carries with it an increased intrinsic risk for HTN&#44; related to the morbidity and mortality of the syndrome&#44; HTN should be carefully diagnosed and treated&#46;<a class="elsevierStyleCrossRefs" href="#bib0470"><span class="elsevierStyleSup">34&#44;50</span></a></p><p id="par0260" class="elsevierStylePara elsevierViewall">HTN in TS is commonly seen in the absence of cardiac or renal malformations and its pathophysiology is poorly understood and complex&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">19</span></a> Some of the mechanisms that seem to be involved in HTN are increased set risk factors for HTN which are often present in TS&#44; such as obesity&#44; type-1 and type-2 diabetes&#44; dyslipidemia&#44; oxidative stress and inflammation&#44; impaired insulin-mediated vasodilatation and abnormal sodium processing by the kidneys&#46; The role of the inappropriate activation of the renin-angiotensin-aldosterone &#40;RAA&#41; system remains uncertain&#44; but overactivation of the sympathetic nervous system likely plays a major part&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">34</span></a> Estrogen deficiency contributes to blood-pressure &#40;BP&#41; imbalance&#44; because estrogens regulate vascular function by modulating some biological cascades&#44; such as the RAA and endothelin systems&#46; They also have an antioxidant effect and a positive correlation with renal function&#46;<a class="elsevierStyleCrossRefs" href="#bib0470"><span class="elsevierStyleSup">34&#44;51&#8211;53</span></a></p><p id="par0265" class="elsevierStylePara elsevierViewall">Aortic root dilatation is closely dependent on BP&#44; suggesting that rigorous treatment of HTN may reduce the risk of aortic dilatation progression&#46; In addition to that&#44; a significant association between abnormal aortic arch morphology and HTN has been demonstrated&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">19&#44;54</span></a></p><p id="par0270" class="elsevierStylePara elsevierViewall">CoA&#44; even if repaired in the neonatal period&#44; is also a significant risk factor for HTN in TS&#44; suggesting that prenatal or early postnatal hypoperfusion of the kidneys may play a pathogenic role&#46; Haploinsufficiency of critical genes on the X chromosome may be related to the established TS vasculopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0550"><span class="elsevierStyleSup">50</span></a></p><p id="par0275" class="elsevierStylePara elsevierViewall">There is no evidence to support specific HTN treatments in TS&#44; and the choice of therapeutic agent should follow general guidelines when BP reduction is the primary objective&#46;<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">16</span></a> Target BP should be less than 135&#47;85 mmHg&#44; and in those who have congenital heart defects&#44; such as BAV&#44; CoA and dilatation of the aorta &#40;ASI &#8805;2&#46;0 cm&#47;m<span class="elsevierStyleSup">2</span>&#41;&#44; should be less than 130&#47;80 mmHg&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">34</span></a></p></span></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Recommendations for cardiovascular risk screening</span><p id="par0280" class="elsevierStylePara elsevierViewall">Awareness of considerable CV risk requires effective preventive medicine from the earliest years of life&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">35</span></a> Therefore&#44; an appropriate and rigorous CV evaluation with the spectrum of CV issues encountered in TS should be performed &#40;<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>&#41;&#46; The best cardiac imaging modalities for visualizing each of the congenital CV anomalies are shown in <a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a>&#46;</p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia><elsevierMultimedia ident="tbl0025"></elsevierMultimedia><p id="par0285" class="elsevierStylePara elsevierViewall">At diagnosis&#44; all infants and children with TS should undergo comprehensive CV evaluation&#44; including clinical examination&#44; electrocardiogram &#40;ECG&#41; to detect conduction abnormalities&#44; BP measurement and echocardiography&#44; which are currently the standard of care to assess the cardiac anatomy of girls with TS&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;55</span></a></p><p id="par0290" class="elsevierStylePara elsevierViewall">CMR is recommended in TS children from the age of 12 &#40;when it can be performed without sedation&#41;&#44; even if no cardiac anomalies are detected on echocardiography&#46; Indeed&#44; CMR is the gold-standard imaging test for the diagnosis and monitoring of morphological anomalies of the thoracic aorta in TS&#44; since echocardiography underestimates the size of both the ascending and the descending aortas&#46;<a class="elsevierStyleCrossRefs" href="#bib0325"><span class="elsevierStyleSup">5&#44;18</span></a></p><p id="par0295" class="elsevierStylePara elsevierViewall">For outpatients&#44; alternating echocardiography and CMR may be helpful&#46; Aortic diameter in TS patients with no risk factors for aortic dissection &#40;BAV&#44; CoA and&#47;or increased aortic dimensions&#41; should be re-evaluated every 5-10 years&#46; In patients considered to be at high risk&#44; CMR should be repeated sooner&#46;<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">21</span></a><a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a> summarizes a reasonable imaging approach for these patients&#46;</p><p id="par0300" class="elsevierStylePara elsevierViewall">In the acute setting&#44; CMR is not useful due to the relatively long examination time and difficulty in monitoring unstable patients&#46; ECG-gated CT aortography is a valuable alternative for obtaining images of the entire aorta and aortic branch vessels including coronary arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Pregnancy</span><p id="par0305" class="elsevierStylePara elsevierViewall">As mentioned above&#44; ovarian failure is a typical feature in TS with no or incomplete development of secondary sexual characteristics and primary amenorrhea&#46; The X chromosome locus responsible for primary ovarian insufficiency in TS is probably on the short arm &#40;Xq&#41;&#46; However&#44; 2-8&#37; experience spontaneous pregnancy&#44; and advances in assisted reproductive medicine with donated oocytes have been used&#44; enabling successful pregnancies to be achieved&#46;<a class="elsevierStyleCrossRefs" href="#bib0315"><span class="elsevierStyleSup">3&#44;56</span></a></p><p id="par0310" class="elsevierStylePara elsevierViewall">These pregnancies carry substantial fetal and maternal risks&#44; exerting significant stress on the heart and aorta &#40;with an increase in stroke volume and rise in estrogen level&#41; and hypertensive disorders such as pre-eclampsia that may directly promote vascular damage and aneurysm formation&#46; If uncontrolled&#44; this can lead to aortic rupture and dissection&#46;<a class="elsevierStyleCrossRefs" href="#bib0315"><span class="elsevierStyleSup">3&#44;18</span></a></p><p id="par0315" class="elsevierStylePara elsevierViewall">The risk of death during pregnancy from aortic dissection and rupture may be 2&#37; or higher in women with TS&#46;<a class="elsevierStyleCrossRef" href="#bib0585"><span class="elsevierStyleSup">57</span></a></p><p id="par0320" class="elsevierStylePara elsevierViewall">Thus&#44; careful follow-up&#44; including echocardiography and CMR performed by trained specialists familiar with TS&#44; is essential for patients who intend to become pregnant&#46;<a class="elsevierStyleCrossRef" href="#bib0590"><span class="elsevierStyleSup">58</span></a></p><p id="par0325" class="elsevierStylePara elsevierViewall">According to the 2008 publication of American Society of Reproductive Medicine&#44; TS is a relative contraindication for pregnancy&#44; but an absolute contraindication if there is a documented cardiac anomaly&#46; However&#44; the French task force 2011 recommendations&#44; summarized in <a class="elsevierStyleCrossRef" href="#tbl0030">Table 6</a>&#44; do not list an isolated BAV as a contraindication&#44; but rather a risk factor&#46;<a class="elsevierStyleCrossRefs" href="#bib0595"><span class="elsevierStyleSup">59&#44;60</span></a></p><elsevierMultimedia ident="tbl0030"></elsevierMultimedia><p id="par0330" class="elsevierStylePara elsevierViewall">If the ASI is less than 2&#46;5 cm&#47;m<span class="elsevierStyleSup">2</span> or the aortic diameter is less than 3&#46;5 cm&#44; and if there is no associated CoA&#44; pregnancy can be authorized although careful CV monitoring is required&#44; with an echocardiography suggested at the end of the first and the second trimesters and monthly during the third&#46; If a patient is pending oocyte donation and the aorta increases by 10&#37; or more in the annual echocardiography examination&#44; said increase must be confirmed via a second imaging technique &#40;CMR&#44; cardiac CT or transesophageal echocardiography&#41; and&#44; if confirmed&#44; pregnancy is contraindicated&#46;<a class="elsevierStyleCrossRef" href="#bib0595"><span class="elsevierStyleSup">59</span></a></p></span><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Final remarks</span><p id="par0335" class="elsevierStylePara elsevierViewall">In TS&#44; the detection of congenital CV anomalies and their continuous monitoring with the use of cardiac imaging methods&#44; particularly CMR &#40;now recommended for optimal cardiac risk assessment&#41;&#44; is of vital importance&#46;</p><p id="par0340" class="elsevierStylePara elsevierViewall">Aortic dissection&#44; an extremely common event in these women&#44; can occur at a remarkably young age and in pregnancy&#46; Therefore&#44; special attention to prevention and treatment should be given by monitoring the standard CV risk factors in TS&#46; This approach is crucial to avoid life-threatening complications&#46;</p><p id="par0345" class="elsevierStylePara elsevierViewall">Pregnancy&#44; which is not often spontaneous and can be achieved through assisted reproduction&#44; increases risk in women with TS&#46; Contraindications to pregnancy are defined&#44; but even if none are present&#44; patients should be carefully monitored before and during pregnancy&#46;</p><p id="par0350" class="elsevierStylePara elsevierViewall">To sum up&#44; it is clear that TS is associated with several CV anomalies and risk factors present at an early age&#44; which contribute to high TS mortality and morbidity&#46; However&#44; there are numerous unresolved issues that require further research&#46;</p><p id="par0355" class="elsevierStylePara elsevierViewall">Firstly&#44; studies are warranted to clarify the pathophysiology of CV risk and the development of congenital CV anomalies&#46; TS aortopathy has been postulated as a connective disorder&#44; a theory that has not yet been confirmed&#46; Determining the etiology will enable offering potential therapies to prevent aortic dissection&#46; It will also provide information about potential risk stratification and&#44; therefore&#44; help determine the frequency of cardiovascular screening &#40;which is currently not appropriately addressed&#41;&#46;</p><p id="par0360" class="elsevierStylePara elsevierViewall">Furthermore&#44; the cut-off for aortic dilatation that puts an individual at high risk of aortic dissection in TS remains unclear&#46; As such&#44; the relationship between aortic dimensions and aortic dissection needs to be defined&#44; and the adjustment of aortic dimensions for younger patients and those with lower BSA needs clarification&#46;</p><p id="par0365" class="elsevierStylePara elsevierViewall">Finally&#44; the predictive values of aortic dimensions for prophylactic aortic surgery to prevent aortic dissection are undefined&#44; as are the efficacy and safety of the procedure&#46;</p></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Conflicts of interest</span><p id="par0370" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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          "titulo" => "Introduction"
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        5 => array:2 [
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          "titulo" => "Methods"
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        6 => array:3 [
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          "titulo" => "Congenital cardiovascular disorders"
          "secciones" => array:4 [
            0 => array:3 [
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              "titulo" => "A&#46; Valvular anomaly"
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                  "identificador" => "sec0025"
                  "titulo" => "A&#46;1&#46; Bicuspid aortic valve"
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            1 => array:3 [
              "identificador" => "sec0030"
              "titulo" => "B&#46; Aortic abnormalities"
              "secciones" => array:4 [
                0 => array:2 [
                  "identificador" => "sec0035"
                  "titulo" => "B&#46;1&#46; Coarctation of the aorta"
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                1 => array:2 [
                  "identificador" => "sec0040"
                  "titulo" => "B&#46;2&#46; Elongated transverse arch"
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                2 => array:2 [
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                  "titulo" => "B&#46;3&#46; Aortic dilatation"
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                  "titulo" => "B&#46;4&#46; Aortic dissection"
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                  "titulo" => "C&#46;1&#46; Partial anomalous pulmonary venous connection"
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                  "titulo" => "C&#46;2&#46; Persistent left superior vena cava"
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              "identificador" => "sec0070"
              "titulo" => "D&#46; Other congenital cardiovascular disorders"
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          "titulo" => "Cardiovascular risk"
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              "titulo" => "A&#46; Specific aspects of vascular structure and physiology"
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              "identificador" => "sec0085"
              "titulo" => "B&#46; Glucose metabolism&#44; body composition and physical activity"
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              "identificador" => "sec0090"
              "titulo" => "C&#46; Lipid profile"
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              "titulo" => "D&#46; Hypertension"
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          "titulo" => "Recommendations for cardiovascular risk screening"
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          "titulo" => "Pregnancy"
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    "fechaAceptado" => "2017-08-16"
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          "clase" => "keyword"
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            0 => "Turner syndrome"
            1 => "Cardiovascular diseases"
            2 => "Risk factors"
            3 => "Female"
          ]
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          "clase" => "keyword"
          "titulo" => "Palavras-chave"
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            0 => "S&#237;ndrome de Turner"
            1 => "Doen&#231;as cardiovasculares"
            2 => "Fatores de risco"
            3 => "Mulher"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Turner syndrome is a relatively common genetic disorder of female development&#44; characterized by partial or complete absence of an X chromosome&#44; with a variable clinical presentation&#46; Congenital or acquired cardiovascular disease is highly prevalent and a major cause of early death in this syndrome&#46; The most feared complication is aortic dissection&#44; which can occur at a very young age and requires careful assessment of its risk factors&#46; A systematic literature search identified sixty relevant publications&#46; These were reviewed with regard to the increased risk of cardiovascular disease in women with Turner syndrome&#44; especially in pregnancy&#46; The most common congenital cardiovascular defects are presented and illustrated with appropriate iconography&#46; The current recommendations regarding the screening and monitoring of cardiovascular disease in these patients are discussed&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A s&#237;ndrome de Turner &#233; uma condi&#231;&#227;o gen&#233;tica relativamente comum&#44; caracterizada pela perda total ou parcial de um cromossoma X&#44; com uma apresenta&#231;&#227;o cl&#237;nica vari&#225;vel e que afeta o desenvolvimento das mulheres&#46; Nessa s&#237;ndrome h&#225; uma preval&#234;ncia elevada de doen&#231;a cardiovascular&#44; cong&#233;nita e adquirida&#44; que condiciona um risco aumentado de morte prematura&#46; A complica&#231;&#227;o mais temida &#233; a disse&#231;&#227;o da aorta&#44; que tende a ocorrer em idades mais precoces&#44; implica uma avalia&#231;&#227;o cuidada dos seus fatores de risco&#46; A pesquisa sistem&#225;tica da literatura permitiu encontrar 60 artigos relevantes sobre o tema&#46; Foi feita uma revis&#227;o sobre o risco aumentado de doen&#231;a cardiovascular nas doentes com s&#237;ndrome de Turner&#44; em particular na gravidez&#46; Foram apresentados e ilustrados com iconografia pr&#243;pria os defeitos cardiovasculares cong&#233;nitos mais prevalentes&#46; Foram discutidas as recomenda&#231;&#245;es atuais relativas ao rastreio e &#224; vigil&#226;ncia da doen&#231;a cardiovascular nessas doentes&#46;</p></span>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Flow diagram of search strategy and study selection&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Systolic cine magnetic resonance image of a 36-year-old TS patient&#44; with fusion of the right and left coronary leaflets&#44; with an anterior raphe &#40;white arrow&#41;&#46;</p>"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Volume-rendered magnetic resonance aortography &#40;posterior-oblique view&#41; showing an aberrant right subclavian artery &#40;white arrow&#41; in a 37-year-old TS woman&#46;</p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Reference number&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Year of publication&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Type of study&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Number of patients&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Objective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">1</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To describe the spectrum of cardiovascular defects with particular attention to identifying risk factors for aortic dissection&#47;rupture&#46; X-chromosome genetic pathways implicated in Turner cardiovascular disease&#44; including premature coronary artery disease&#44; are discussed&#46; Recent guidelines for diagnosis and treatment of girls and women with TS are reviewed&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">2</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To provide updated guidelines for the evaluation and treatment of girls and women with Turner syndrome &#40;TS&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">3</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review publications since 2000 concerning maternal outcomes for pregnancies in women with TS to determine if specific risk factors such as type of pregnancy&#44; age or presence of underlying congenital cardiovascular disease may identify women at special risk&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Marin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">4</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review cardiovascular risks in this group of patients and discuss a systematic imaging approach for early identification of cardiovascular disorders in these patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mortensen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">5</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the state of the art of cardiovascular imaging in Turner syndrome&#44; emphasizing unresolved issues in the care of these patients with links to appropriate multimodality imaging strategies&#44; both in acute and chronic presentations and in symptomatic and asymptomatic patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hiratzka et al&#46;<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">6</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2010&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Guidelines&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Granger et al&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To summarize the cardiovascular&#44; neurological&#44; genitourinary&#44; otolaryngological&#44; craniofacial&#44; and skeletal defects associated with TS&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Surerus et al&#46;<a class="elsevierStyleCrossRef" href="#bib0340"><span class="elsevierStyleSup">8</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2003&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">53&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare the incidence and type of heart disease found in association with 45X karyotype in fetal life with postnatal life and to examine the outcome after fetal diagnosis&#46;<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Loscalzo et al&#46;<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">9</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2005&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">134&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine whether there is a specific association between a history of fetal lymphedema and congenital cardiovascular defects in monosomy X&#44; or TS&#44; independent of karyotype or general severity of the phenotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Spectrum et al&#46;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">51&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To use cardiac MRI to describe the spectrum and frequency of cardiovascular abnormalities and to evaluate aortic dilatation and associated abnormalities in pediatric patients with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ho et al&#46;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2004&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">85 adults with TS and 27 normal female adult volunteers<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To better understand the prevalence and pathogenesis of cardiovascular defects in TS by evaluating a group of asymptomatic adult volunteers with TS using magnetic resonance angiography&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hjerrild et al&#46;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2010&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cross-sectional<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">102&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate aortic dimensions in women with Turner syndrome in relation to aortic valve morphology&#44; blood pressure&#44; karyotype and clinical characteristics&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">13</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To focus on emerging knowledge of the characteristics of aortic disease in TS in comparison with Marfan-like syndromes and isolated aortic valve disease&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sachdev et al&#46;<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">14</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">253&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence and characteristics of aortic valve disease in girls and women with monosomy for the X chromosome&#44; or Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Gutmark-Little et al&#46;<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">15</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">39&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence and hemodynamic significance of partial anomalous pulmonary venous return in adolescents and young adults with TS using transthoracic echocardiogram and cardiac magnetic resonance&#44; and to study the association with phenotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mortensen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">16</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To provide insight into pathogenesis of Turner syndrome with perspectives to advances in the understanding of genetics of the X-chromosome&#46; The review also incorporates important endocrine features&#44; in order to comprehensively explain the cardiovascular phenotype and to highlight how increased attention to endocrinology and genetics is important in the identification and modification of cardiovascular risk&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Turtle et al&#46;<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">17</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the literature on the key risk factors for aortic dissection&#44; and to propose recommendations on the optimal imaging for congenital heart defects and aortic dilatation&#44; and on the assessment and management of blood pressure in this patient group&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Turtle et al&#46;<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">18</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the literature on cardiovascular disease in women with TS and make recommendations based on relatively limited high-quality evidence&#44; together with our experience&#44; on the optimal timing of cardiovascular screening&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Elsheikh et al&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">19</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2001&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">38&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the prevalence of aortic root dilatation in a group of women with TS and to investigate the factors contributing to its development&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sharma et al&#46;<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">20</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2009&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective cross-sectional&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess utility of aortic distensibility as a measure of aortic elasticity for the stratification of the risk for aortic dilation&#44; and its relationship with age&#44; karyotype and hormonal therapy&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Wong et al&#46;<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">21</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To raise awareness of the risk of aortic dissection in groups of adolescents and young adults amongst clinicians of all backgrounds &#40;pediatric and adult endocrinologists&#44; pediatric and adult cardiologists&#44; general pediatricians and adolescent physicians&#44; adult physicians&#44; emergency specialists and general practitioners&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mortensen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">22</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">80 adult TS patients and 67 healthy-age and gender-matched controls<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To prospectively assess aortic dimensions in TS&#46;<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lanzarini et al&#46;<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">23</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">78&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the prevalence of aortic root and proximal thoracic aorta enlargement by 2-dimensional echocardiography and the evolution of aortic diameters over time&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Carlson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0420"><span class="elsevierStyleSup">24</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">20&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To describe the signs&#44; symptoms and echocardiographic features preceding aortic dissection in 20 women with TS&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Matura et al&#46;<a class="elsevierStyleCrossRef" href="#bib0425"><span class="elsevierStyleSup">25</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">166 adult volunteers with TS who were not selected for cardiovascular disease and 26 healthy female control subjects&#46;<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate aortic diameters measured by magnetic resonance in a large group of women with TS to elucidate factors associated with variation in ascending aortic size and to compare methods to identify potentially ominous dilatation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Gravhoolt et al&#46;<a class="elsevierStyleCrossRef" href="#bib0430"><span class="elsevierStyleSup">26</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To describe in more detail aortic dissection as encountered in Turner&#39;s syndrome&#44; giving attention to clinical&#44; histological and epidemiological aspects&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lopez et al&#46;<a class="elsevierStyleCrossRef" href="#bib0435"><span class="elsevierStyleSup">27</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">138&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To study the prevalence&#44; magnitude&#44; and determinants of aortic dilation in a large group of girls and young women with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lawson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0440"><span class="elsevierStyleSup">28</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">49 Turner syndrome patients and 76 lean and 52 obese controls&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether vasculopathy can be detected in young TS patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Carlson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0445"><span class="elsevierStyleSup">29</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review &#43; case report&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Two case reports and a review of 85 cases&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To discuss two cases in which only the diagnosis of TS was present and other predisposing risk factors&#44; such as congenital heart disease or systemic hypertension&#44; were expressly absent&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Gutmark-Little et al&#46;<a class="elsevierStyleCrossRef" href="#bib0450"><span class="elsevierStyleSup">30</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">39&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence and hemodynamic significance of partial anomalous pulmonary venous return in adolescents and young adults with TS using transthoracic echocardiogram and cardiac magnetic resonance&#44; and to study the association with phenotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sears et al&#46;<a class="elsevierStyleCrossRef" href="#bib0455"><span class="elsevierStyleSup">31</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Case report&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To discuss two adult patients who presented with pulmonary hypertension&#44; and evidence of right ventricular hypertrophy and dysfunction&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Povoski et al&#46;<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">32</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Case report with literature review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the available literature as it pertains to the specific congenital venous anomaly of the thoracic systemic venous return&#44; persistent left superior vena cava&#44; and to discuss the clinical implications and relevance of congenital aberrancies&#44; as well as of treatment-induced or disease-induced alterations in the anatomy of the thoracic central venous system&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Proprawski et al&#46;<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">33</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2009&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">34&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the cardiovascular system by clinical examination&#44; echocardiography and electrocardiography in female patients with Turner syndrome&#44; depending on karyotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">De Groote et al&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">34</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare current treatment strategies and also propose an integrated practical approach for the diagnosis and treatment of hypertension in Turner syndrome applicable in daily practice&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Kozlowka-Wojciechowska et al&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">35</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence of classic risk factors of atherosclerosis in a group of young Polish women with Turner syndrome in comparison to a representative group of young Polish women from the general population&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ostberg et al&#46;<a class="elsevierStyleCrossRef" href="#bib0480"><span class="elsevierStyleSup">36</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2005&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">93 TS women&#44; 25 normal controls and 11 women with 46&#44;XX primary amenorrhea&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether TS women have a fundamental arterial wall defect that may be due to genetic factors or estrogen deficiency&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Trolle et al&#46;<a class="elsevierStyleCrossRef" href="#bib0485"><span class="elsevierStyleSup">37</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To discuss the effects of estrogen and androgen insufficiency as well as the effects of sex hormone replacement therapy &#40;HRT&#41; on morbidity and mortality with special emphasis on evidence-based research and areas needing further studies&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Groote et al&#46;<a class="elsevierStyleCrossRef" href="#bib0490"><span class="elsevierStyleSup">38</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective case &#8211; control&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15 prepubertal<br>Turner syndrome girls and 31 sex-&#44; age- and height-matched healthy controls<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To examine whether abnormal rigidity of the aorta is present from a young age and if it is associated with an increased carotid artery diameter or intima-media thickness&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Elsheikh et al&#46;<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">39</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2000&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">21&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the degree to which HRT may be protective against ischemic heart disease in women with TS by measuring arterial stiffness&#44; blood pressure&#44; insulin sensitivity and lipids&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ostberg et al&#46;<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">40</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To study the influence of increasing doses of HRT on markers of metabolism and vascular physiology&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Chan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0505"><span class="elsevierStyleSup">41</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2002&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To examine the effect of HRT on endothelial function of forearm resistance vessels in women with Turner&#39;s syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Colao et al&#46;<a class="elsevierStyleCrossRef" href="#bib0510"><span class="elsevierStyleSup">42</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the role of the growth hormone &#40;GH&#41; and insulin-like growth factor I system on the cardiovascular system&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">O&#8217;Gorman et al&#46;<a class="elsevierStyleCrossRef" href="#bib0515"><span class="elsevierStyleSup">43</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cross-sectional cohort&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">19 TS and 17 control girls<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare cardiometabolic risk factors and measures of subcutaneous&#44; visceral adipose tissue and intra-myocellular lipid between young TS girls and an age- and body mass index-standard deviation scores-matched healthy female cohort&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Salgin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">44</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16 TS women and 16 normal healthy women&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine whether the defect in insulin sensitivity is a primary intrinsic defect in TS or dependent on variation in body composition&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mazzanti et al&#46;<a class="elsevierStyleCrossRef" href="#bib0525"><span class="elsevierStyleSup">45</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Mixed longitudinal and cross-sectional&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">46&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To analyze carbohydrate tolerance and insulin sensitivity in patients with TS who reached their final height after long-term &#40;10 years&#41; treatment with high-dose GH&#44; and in those who continued to be followed-up after the cessation of GH therapy&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ladin-Wilhelmsen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0530"><span class="elsevierStyleSup">46</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2001&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">100&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To analyze the prevalence of cardiovascular risk factors&#44; especially diabetes mellitus&#44; blood lipids&#44; fibrinogen&#44; and cardiovascular malformations&#44; as well as sex hormones in a large group of adult TS women and compare them with a random population sample of similar age from the same region&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Akyrek et al&#46;<a class="elsevierStyleCrossRef" href="#bib0535"><span class="elsevierStyleSup">47</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">29 TS and 29 healthy children and adolescents&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To evaluate the relationship between periaortic adipose thickness and metabolic data in a group of children with TS&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Pirgon et al&#46;<a class="elsevierStyleCrossRef" href="#bib0540"><span class="elsevierStyleSup">48</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24 TS and 24 healthy children&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine whether there are risk factors for atherosclerosis in children with TS&#44; carotid artery intima-media thickness&#44; anthropometric and metabolic parameters were compared between children with TS and healthy controls&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Irzyniec et al&#46;<a class="elsevierStyleCrossRef" href="#bib0545"><span class="elsevierStyleSup">49</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">165&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess lipid and carbohydrate metabolism in TS women in the context of HRT as well as GH treatment during childhood&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Los et al&#46;<a class="elsevierStyleCrossRef" href="#bib0550"><span class="elsevierStyleSup">50</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">168&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To identify the factors associated with hypertension in girls with Turner syndrome by studying the prevalence of elevated systemic blood pressure&#44; awareness of the problem&#44; and its clinical associations in a large cohort of girls with Turner syndrome&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Zuckerman-Levin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0555"><span class="elsevierStyleSup">51</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10 young women with Turner syndrome and 10 age-matched healthy women&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether women with Turner syndrome have reduced catecholaminergic and physiological response to sympathetic stimulation&#44; and whether changes in blood pressure and heart rate are related to their catecholamine response to sympathetic stimulation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Maric-Bilkan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0560"><span class="elsevierStyleSup">52</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To summarize the current understanding of the mechanisms by which estrogens regulate blood pressure and the potential use of hormone therapy in prevention of hypertension and consequent cardiovascular risk&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lastra et al&#46;<a class="elsevierStyleCrossRef" href="#bib0565"><span class="elsevierStyleSup">53</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">An update of type 2 diabetes mellitus and hypertension&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Herck et al&#46;<a class="elsevierStyleCrossRef" href="#bib0570"><span class="elsevierStyleSup">54</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">74&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether TS patients with an abnormal arch morphology are more at risk for hypertension&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Somerville et al&#46;<a class="elsevierStyleCrossRef" href="#bib0575"><span class="elsevierStyleSup">55</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare the detection of cardiac lesions with the use of cardiac magnetic resonance imaging and conventional echocardiography in children with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Nwosu et al&#46;<a class="elsevierStyleCrossRef" href="#bib0580"><span class="elsevierStyleSup">56</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Case report with literature review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To present the case of a woman with Turner syndrome achieving a successful pregnancy from a donor oocyte and review the relevant literature&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><a class="elsevierStyleCrossRef" href="#bib0585"><span class="elsevierStyleSup">57</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To evaluate the impact of Turner syndrome on pregnancy outcomes after oocyte donation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Boissonnas et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0590"><span class="elsevierStyleSup">58</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To evaluate the follow-up &#40;mainly cardiovascular&#41; of women with TS requesting oocyte donation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cabanes et al&#46;<a class="elsevierStyleCrossRef" href="#bib0595"><span class="elsevierStyleSup">59</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2010&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Recommendations for pregnancy in Turner syndrome patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Karnis et al&#46;<a class="elsevierStyleCrossRef" href="#bib0600"><span class="elsevierStyleSup">60</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Guidelines for pre-conception screening and counseling now exist that may mitigate the maternal and fetal risks associated with pregnancy in women with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Summary of the literature consulted&#46;</p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Congenital heart defect&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Frequency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Aortic anomalies&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Elongated transverse arch&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">31&#46;4&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a> 49&#37;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Aortic dilatation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">23&#37;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Aortic dissection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">100-fold increased risk<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">13</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Coarctation of the aorta&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a> 15&#46;7&#37;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Valvular anomaly&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Bicuspid aortic valve&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a> 30&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">14</span></a> 39&#46;2&#37;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Venous anomalies&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Partial anomalous venous connection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a> 15&#46;7&#37;&#44;<span class="elsevierStyleSup">10</span>18&#37;<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">15</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Persistent left superior vena cava&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&#37;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Arch vessel anomaly&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Aberrant right subclavian artery&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&#37;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Frequency of the main congenital cardiovascular anomalies in TS&#46;</p>"
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                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Estrogen action&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Consequence&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stimulation of the release of nitric oxide by vascular endothelial cellules&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Inhibition of platelet aggregation and monocyte adhesion to the vascular wall&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Vasodilatation &#40;also induced by bradykinin through the bradykinin B2 receptors&#41;<br>Inhibition of vascular smooth muscle proliferation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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          "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Antiatherogenic effect of estrogen&#46; Adapted from Elsheikh et al&#46;<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">39</span></a>&#59; Chan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0505"><span class="elsevierStyleSup">41</span></a></p>"
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          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Screening and monitoring algorithm for cardiovascular imaging in TS girls and women&#46; Adapted from Marin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">4</span></a></p>"
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hepatic contraindications&nbsp;\t\t\t\t\t\t\n
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Review Article
Cardiovascular risk in Turner syndrome
Risco cardiovascular na síndrome de Turner
Beatriz Donatoa,
Autor para correspondência
beatrizcamposdonato@gmail.com

Corresponding author.
, Maria João Ferreiraa,b
a Faculdade de Medicina, Universidade de Coimbra, Coimbra, Portugal
b Cardiology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
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6474
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        "titulo" => "Risco cardiovascular na s&#237;ndrome de Turner"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Long-axis three-chamber-view CMR of a 24-year-old patient with TS&#44; showing dilatation of the ascending aorta&#44; with ASI &#62;2&#46;5 cm&#47;m<span class="elsevierStyleSup">2</span> indicating a high risk of dissection&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Turner syndrome &#40;TS&#41; is a genetic disorder with a characteristic phenotype that occurs as a result of a structurally abnormal or absent X chromosome&#46; TS is the only monosomy compatible with life and affects around 1 in 2000 newborn females&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">There are variable Turner karyotypes&#44; such as monosomy X &#40;45&#44;X&#59; the most prevalent karyotype&#41;&#44; mosaic karyotype&#44; isochromosome X&#44; ring X chromosome or deletions&#46; In all these cases&#44; significant portions of the X chromosome are deleted&#46;<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The diagnosis of TS requires having key clinical features&#44; including short stature and gonadal failure &#40;the cardinal features&#41; and congenital cardiovascular &#40;CV&#41; defects as well as an abnormal karyotype&#46;<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The most serious consequences of X-chromosome haploinsufficiency involve the CV system&#46; Indeed&#44; CV defects&#44; present in up to 50&#37; of the TS population&#44; are the major cause of premature death and contribute to a standardized mortality ratio three times higher than in the general female population&#46; Therefore&#44; imaging studies &#40;both echocardiography and cardiovascular magnetic resonance &#91;CMR&#93;&#41; are crucial for the timely detection of often subclinical CV disease and should preferably be ordered before the onset of symptomatic and irreversible organ damage&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;4&#44;5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">A thorough cardiac examination is recommended for all women with TS every 5 to 10 years&#44; at the time of transition from pediatric to adult care&#44; before attempting pregnancy or if hypertension &#40;HTN&#41; develops&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The aim of this paper is to review both congenital and acquired cardiovascular diseases and the role of clinical imaging in TS CV risk stratification with recommendations for CV screening&#46; Moreover&#44; particular attention will be given to the special risk of CV complications in pregnancy with this syndrome&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Methods</span><p id="par0035" class="elsevierStylePara elsevierViewall">This review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses &#40;PRISMA&#41; guidelines&#44; thus providing a comprehensive framework that objectively assesses quality indicators and risk of bias in the studies included&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">All original studies investigating Turner syndrome and cardiovascular risk were eligible for this systematic review&#46; Further selection criteria were&#58; &#40;i&#41; publication date between January 2000 and September 2016&#44; &#40;ii&#41; written in English&#44; &#40;iii&#41; published in a scholarly peer-reviewed journal&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Studies were found by searching the PubMed&#44; EMBASE and Cochrane Library electronic databases&#44; using the following search strategy&#58; &#8220;Turner syndrome AND Cardiovascular disease AND Risk factors AND Female&#8221;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Studies were identified by searching relevant papers via PubMed&#47;MEDLINE &#40;<a id="intr0005" class="elsevierStyleInterRef" href="http://www.ncbi.nlm.nih.gov/pubmed">http&#58;&#47;&#47;www&#46;ncbi&#46;nlm&#46;nih&#46;gov&#47;pubmed</a>&#41;&#44; the Cochrane Library and EMBASE using the following search strategy&#58; &#8220;Turner syndrome AND Cardiovascular disease AND Risk factors&#46; Finally&#44; reference lists of the studies retrieved were manually searched in order to detect any additional relevant studies&#46; Keywords and combinations of keywords were used to search the electronic databases &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">After performing the initial literature searches&#44; each study title and abstract was screened for eligibility by the first author&#46; The full texts of all potentially relevant studies were subsequently retrieved and examined further for eligibility&#46; The PRISMA flow diagram &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41; provides more detailed information regarding the process for selecting studies&#46; Sixty articles were included in this review and the information from the studies included was then analyzed and recorded&#46; <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> shows a summary of the literature consulted&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Congenital cardiovascular disorders</span><p id="par0060" class="elsevierStylePara elsevierViewall">Variations in the CV anatomy of patients with TS&#44; the frequencies of which are summarized in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#44; are major factors in their reduced life expectancy&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a></p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0065" class="elsevierStylePara elsevierViewall">Major defects in cardiac and aortic development during fetal life are associated with miscarriage in most cases of fetuses with 45X karyotype&#46; Fetuses with CV failure almost always demonstrate obstructed jugular lymphatics with increased nuchal translucency or nuchal cystic hygromas&#44; the typical intrauterine presentation of TS&#46; The residual postnatal webbing of the neck predicts CV defects&#46; This association suggests a pathogenetic connection between fetal lymphatic obstruction and defective CV development due to the compression or obstruction of the outflow tracts&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;8&#44;9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">However&#44; this is just a theory regarding a pathogenetic mechanism for congenital CV defects&#44; because the real causes are currently unknown&#46;<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">1</span></a></p><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">A&#46; Valvular anomaly</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">A&#46;1&#46; Bicuspid aortic valve</span><p id="par0075" class="elsevierStylePara elsevierViewall">A bicuspid aortic valve &#40;BAV&#41; is a congenital valvular anomaly found in approximately 30&#37; of TS patients and it is not often clinically apparent&#46;<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">14</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Identifying a BAV in asymptomatic individuals is important because they are at increased risk of infective endocarditis&#44; hemodynamically significant stenosis &#40;promoted by accelerated valve calcification&#41;&#44; valve regurgitation &#40;secondary to poor leaflet coaptation&#41; and aortic aneurysm &#40;due to the vicious cycle of increased stroke volume that promotes aortic root dilatation&#41;&#46; For that reason&#44; the anomaly requires regular medical evaluation and possibly surgery to prevent aortic dissection or rupture&#46;<a class="elsevierStyleCrossRefs" href="#bib0370"><span class="elsevierStyleSup">14&#44;16</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Indeed&#44; the aortic valve leaflet must be clearly visualized and echocardiography is the primary test for BAV that has been reported to be quite sensitive in diagnosing this anomaly in 89&#37; of women with TS&#46; However&#44; echocardiography can be inadequate to view the aortic valve and&#44; in these cases&#44; CMR should be the next approach&#46;<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;14</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">In almost all the cases&#44; BAV results from fusion of the right and left coronary leaflets&#44; while the fused right coronary and non-coronary leaflets variant is much less common<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;14</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">B&#46; Aortic abnormalities</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">B&#46;1&#46; Coarctation of the aorta</span><p id="par0095" class="elsevierStylePara elsevierViewall">Coarctation of the aorta &#40;CoA&#41; affects around 12&#37; of women with TS<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a> and is usually diagnosed in infancy&#44; often with congestive heart failure in critical cases<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">17</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0100" class="elsevierStylePara elsevierViewall">Indeed&#44; in contrast to BAV&#44; which is detected by screening&#44; CoA is usually diagnosed based on clinical grounds &#8211; HTN and brachial-femoral delay are common features&#46; However&#44; since many cases are detected later in life&#44; any woman with suspected CoA should have a CMR or computed tomography &#40;CT&#41; angiography with three-dimensional reconstruction of the thoracic aorta&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;11</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">Concentric narrowing of the aortic lumen leads to the development of pressure gradients across the lesion&#44; with the possibility of arterial collaterals becoming a detour for oxygenated blood traveling to the lower segments of the body&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">If left untreated&#44; complications such as HTN&#44; congestive heart failure&#44; aortic dissection and aortic rupture may occur&#46; Surgical intervention is required when peak-to-peak coarctation gradient is &#8805;20 mmHg&#44; or &#60;20 mmHg with radiological evidence of significant collateral flow&#46; Even after surgical repair&#44; there is an increased risk of HTN&#44; coronary artery disease &#40;CAD&#41;&#44; cerebrovascular disease&#44; aortic dissection and restenosis&#46;<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">18</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">B&#46;2&#46; Elongated transverse arch</span><p id="par0115" class="elsevierStylePara elsevierViewall">Elongated transverse arch &#40;ETA&#41; is a silent anomaly&#44; detected by CMR&#44; defined as an increased distance between the origin of the left common carotid and left subclavian arteries&#44; with flattening of the arch and kinking along the inferior curvature&#46; This distinct anatomy&#44; sometimes referred to as &#8220;pseudocoarctation&#8221;&#44; is embryologically similar to coarctation and may predict aortic complications&#44; such as aortic dilation&#44; aortic dissection or progression to overt coarctation&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;11</span></a> However&#44; ETA differs from true coarctation in that there is no true luminal narrowing&#44; nor are there pressure gradients or collateral circulation&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">ETA has been significantly associated with BAV&#44; CoA and also with aortic sinus dilatation&#46;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a></p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">B&#46;3&#46; Aortic dilatation</span><p id="par0125" class="elsevierStylePara elsevierViewall">Aortic dilatation is reported in 23&#37; of women with TS&#46;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall">Risk factors for this and&#44; secondarily&#44; dissection include HTN&#44; BAV&#44; CoA&#44; 45&#44;X karyotype and pregnancy&#46; Aortic dilatation may less commonly occur alone in approximately 5&#37; of women with TS&#46; This indicates that all women with TS should be periodically monitored&#46;<a class="elsevierStyleCrossRefs" href="#bib0325"><span class="elsevierStyleSup">5&#44;19&#8211;21</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">In TS patients with enlargement of the ascending aorta&#44; a general aortopathy is present&#44; which is accelerated by the presence of a BAV&#46;<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">22</span></a></p><p id="par0140" class="elsevierStylePara elsevierViewall">It has been suggested that patients with an enlarged aortic root should undergo echocardiographic evaluation every year and that patients with normal aortic root dimensions should be evaluated every 2 to 3 years&#46;<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">23</span></a></p><p id="par0145" class="elsevierStylePara elsevierViewall">Aortic diameter&#44; corrected for age and body size&#44; is accurate for predicting aortic events&#44; and is the principal risk marker for aortic dissection&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">1&#44;5</span></a> An ascending to descending aortic diameter ratio above 1&#46;5 signifies ascending aorta dilatation if descending aorta diameter is normal&#46; There is another alternative to identifying aortic dilatation that correlates more closely with aortic diameter&#44; because it adjusts aortic dimensions for body surface area &#40;BSA&#41;&#59; this is important due to the relatively short stature of patients with TS&#46; This latter method involves estimating the aortic size index &#40;ASI&#41;&#58; aortic diameter &#40;at the ascending aorta or at the site with the largest dimension&#41;&#47;BSA&#46; It has been proposed that an ASI &#8805;2 cm&#47;m<span class="elsevierStyleSup">2</span> identifies those who require close monitoring and an ASI &#8805;2&#46;5 cm&#47;m<span class="elsevierStyleSup">2</span> &#40;as in <a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41; requires aortic surgery to prevent aortic dissection&#46;<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;21&#44;24&#44;25</span></a></p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">B&#46;4&#46; Aortic dissection</span><p id="par0150" class="elsevierStylePara elsevierViewall">Although rare&#44; aortic dissection is a major concern in TS&#46; It has an estimated incidence of 40 cases per 10000 patients&#44; but is often fatal&#46; It occurs at a much earlier age&#59; in the general population&#44; the peak incidence of dissection occurs between ages 50 and 80 and in TS population median age is 35&#44; with higher incidence rates at ages 20&#8211;29 and 30&#8211;39&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;26</span></a></p><p id="par0155" class="elsevierStylePara elsevierViewall">Up to 90&#37; of aortic dissections have predisposing CV risk factors &#40;BAV&#44; aortic dilatation and CoA&#41; that can be identified with CMR imaging&#46; HTN and pregnancy are associated with a higher risk of dissection&#44; the former due to an association with increased aortic root diameter and the latter due to physiological adaptation&#44; which imposes a higher cardiac workload&#46;<a class="elsevierStyleCrossRefs" href="#bib0320"><span class="elsevierStyleSup">4&#44;19</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">However&#44; up to 25&#37; of cases have no apparent risk factors other than TS itself&#44; suggesting that TS phenotype may also include a primary aortopathy&#44; which can be present as early as 9 years of age&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">18&#44;26&#8211;28</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall">Indeed&#44; histological evidence of cystic medial necrosis in aortic tissue taken from patients with BAV&#44; Marfan syndrome and TS indicates a shared etiology despite genetically diverse backgrounds&#46;<a class="elsevierStyleCrossRef" href="#bib0445"><span class="elsevierStyleSup">29</span></a></p><p id="par0170" class="elsevierStylePara elsevierViewall">Aortic dissection is sometimes overlooked at onset because it could include apparently minor complaints such as abdominal pain&#44; heartburn&#44; back or shoulder pain or a voice change due to traction of the recurrent laryngeal nerve&#46; Therefore&#44; patients should be advised to go to the emergency department soon after experiencing sustained chest pain &#40;&#62;30<span class="elsevierStyleHsp" style=""></span>min&#41;&#44; regardless of the severity of symptoms&#46;<a class="elsevierStyleCrossRefs" href="#bib0385"><span class="elsevierStyleSup">17&#44;29</span></a></p></span></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">C&#46; Venous anomalies</span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">C&#46;1&#46; Partial anomalous pulmonary venous connection</span><p id="par0175" class="elsevierStylePara elsevierViewall">In TS&#44; partial anomalous pulmonary venous connection may be right-sided&#44; which is the most typical in the general population&#46; Nevertheless&#44; it frequently involves the left upper pulmonary vein&#44; which makes echocardiographic detection more challenging&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;30</span></a></p><p id="par0180" class="elsevierStylePara elsevierViewall">When the pulmonary veins connect to the right atrium or with one of its venous tributaries&#44; blood from the pulmonary circulation returns to the right atrium and this may become clinically significant in adult life&#46; Indeed&#44; if sufficiently severe&#44; pulmonary HTN and right ventricular volume overload can lead to congestive heart failure&#44; which has been related to death in middle-aged TS patients secondary to undiagnosed partial anomalous pulmonary venous return lesions&#46; Echocardiography and ideally CMR imaging should therefore be performed to assess pulmonary vein drainage patterns&#46;<a class="elsevierStyleCrossRefs" href="#bib0450"><span class="elsevierStyleSup">30&#44;31</span></a></p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">C&#46;2&#46; Persistent left superior vena cava</span><p id="par0185" class="elsevierStylePara elsevierViewall">The venous return from a persistent left superior vena cava &#40;PLSVC&#59; <a class="elsevierStyleCrossRef" href="#fig0025">Figure 5</a>&#41; has a prevalence of around 13&#37;&#46; In approximately 80&#37; to 92&#37; of cases&#44; the PLSVC drains into the right atrium via the coronary sinus&#44; resulting in no hemodynamic consequences&#46; However&#44; in about 10&#37; to 20&#37; of cases&#44; it drains into the left atrium and results in venous blood returning to the left atrium&#46;<a class="elsevierStyleCrossRefs" href="#bib0355"><span class="elsevierStyleSup">11&#44;32</span></a></p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0190" class="elsevierStylePara elsevierViewall">It is important to always report this irregular anatomy&#44; as it needs to be taken into account during interventional procedures&#46;<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">32</span></a></p></span></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">D&#46; Other congenital cardiovascular disorders</span><p id="par0195" class="elsevierStylePara elsevierViewall">Cardiac conduction defects are also common&#44; caused by left ventricular hypertrophy&#44; myocardial ischemia&#44; previous myocardial infarction and congenital cardiac malformations&#46;<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">33</span></a></p><p id="par0200" class="elsevierStylePara elsevierViewall">Other associated lesions can be found in TS&#44; such as ventricular and atrial septal defects&#44; hypoplastic left heart syndrome&#44; single ventricle&#44; mitral valve abnormalities&#44; coronary artery abnormalities and aberrant right subclavian artery<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">4</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0030">Figure 6</a>&#41;&#46;</p><elsevierMultimedia ident="fig0030"></elsevierMultimedia></span></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Cardiovascular risk</span><p id="par0205" class="elsevierStylePara elsevierViewall">In addition to potentially harmful CV malformations&#44; women with TS display evidence of adverse CV risk profiles that expose them to greater risk of CAD with a relative risk &#40;RR&#41; of 2-11&#44; cerebrovascular disease with a RR of 2-7&#44; HTN and types 1 and 2 diabetes mellitus&#46;<a class="elsevierStyleCrossRefs" href="#bib0380"><span class="elsevierStyleSup">16&#44;18&#44;34&#44;35</span></a></p><p id="par0210" class="elsevierStylePara elsevierViewall">The precise mechanisms of increased CV risk in TS are unclear&#44; but estrogen deficiencies &#40;ensuing from premature ovarian failure due to gonadal dysgenesis&#41; and haploinsufficiency of the X chromosome seem to be major factors that distinguish this metabolic phenotype&#46;<a class="elsevierStyleCrossRefs" href="#bib0475"><span class="elsevierStyleSup">35&#8211;37</span></a></p><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">A&#46; Specific aspects of vascular structure and physiology</span><p id="par0215" class="elsevierStylePara elsevierViewall">Women with TS have greater intima-media thickness and conduit artery diameters than normal controls&#46;<a class="elsevierStyleCrossRef" href="#bib0480"><span class="elsevierStyleSup">36</span></a> The stiffness of the ascending aorta is also increased in these patients&#46;<a class="elsevierStyleCrossRef" href="#bib0490"><span class="elsevierStyleSup">38</span></a></p><p id="par0220" class="elsevierStylePara elsevierViewall">Estrogen deficiency may contribute to this&#44; as hormone replacement therapy &#40;HRT&#41; appears to reduce aortic stiffness&#44;<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">39</span></a> decrease intima-media thickness<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">40</span></a> and improve endothelial function&#46;<a class="elsevierStyleCrossRef" href="#bib0505"><span class="elsevierStyleSup">41</span></a> Indeed&#44; gonadal dysgenesis may be the key determinant of neointimal hyperplasia in these subjects&#44; because it has been shown that increasing doses of HRT results in a reduction in carotid intima-media thickness in young hypogonadal women&#44; which raises the possibility that exogenous estrogen may be cardioprotective<a class="elsevierStyleCrossRefs" href="#bib0480"><span class="elsevierStyleSup">36&#44;40</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0225" class="elsevierStylePara elsevierViewall">Relative resistance to growth hormone &#40;GH&#41; effects and an imbalance in the GH&#47;insulin-like growth factor I axis could also occur&#44; which may have a negative impact on cardiovascular prognosis&#46; In fact&#44; GH replacement &#40;the accepted treatment for the short stature of TS girls&#41; decreases intima-media thickness at major arteries and improves endothelial dysfunction&#44; which are considered surrogate markers of atherosclerosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0380"><span class="elsevierStyleSup">16&#44;42</span></a></p></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">B&#46; Glucose metabolism&#44; body composition and physical activity</span><p id="par0230" class="elsevierStylePara elsevierViewall">TS girls have a greater risk of developing diabetes&#44; which is related to both the impaired fasting glucose and the impaired glucose tolerance detected among these girls&#46;<a class="elsevierStyleCrossRef" href="#bib0515"><span class="elsevierStyleSup">43</span></a> Moreover&#44; those who have normal fasting glucose concentrations are more insulin resistant than controls&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">44</span></a></p><p id="par0235" class="elsevierStylePara elsevierViewall">The effect of HRT on insulin sensitivity remains unclear&#44; but insulin resistance is a karyotype-dependent factor&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">44</span></a> Furthermore&#44; GH therapy has been shown to reduce insulin sensitivity&#44; antagonizing the effects of insulin on glucose and lipid metabolism via GH receptors&#46; Thus&#44; high GH levels induce peripheral and hepatic insulin resistance&#46; However&#44; patients with TS develop an adaptation to long periods of GH therapy&#44; so insulin sensitivity increases&#46; Upon therapy cessation&#44; insulin sensitivity also increases and returns to pre-treatment levels&#46;<a class="elsevierStyleCrossRef" href="#bib0525"><span class="elsevierStyleSup">45</span></a></p><p id="par0240" class="elsevierStylePara elsevierViewall">Additionally&#44; compared to normal controls&#44; TS patients have a greater waist circumference and higher absolute body mass index&#44; which are associated with an increased risk of type 2 diabetes and metabolic dysfunction&#46;<a class="elsevierStyleCrossRef" href="#bib0515"><span class="elsevierStyleSup">43</span></a> They also tend to be less physically active&#44; thereby contributing to the increased frequency of obesity&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">18&#44;46</span></a></p></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">C&#46; Lipid profile</span><p id="par0245" class="elsevierStylePara elsevierViewall">TS patients have an abnormal lipid profile with higher total cholesterol levels&#44; which contributes to the onset of atherosclerosis and also confers an increased risk for CV disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0515"><span class="elsevierStyleSup">43&#44;44&#44;47&#44;48</span></a></p><p id="par0250" class="elsevierStylePara elsevierViewall">A dose-related relationship between HRT and high-density lipoprotein concentrations has been observed&#44;<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">40</span></a> elucidating the benefits of HRT on CV outcomes by improving the lipid profile&#46; Moreover&#44; the impact of estrogens on lipid metabolism is also determined by diminishing lipid oxidation and increasing triglyceride synthesis&#46; However&#44; in one study&#44; the application of HRT in women with TS had no effect on lipid metabolism&#46;<a class="elsevierStyleCrossRef" href="#bib0545"><span class="elsevierStyleSup">49</span></a></p></span><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">D&#46; Hypertension</span><p id="par0255" class="elsevierStylePara elsevierViewall">HTN can be present in TS patients of any age and has been reported in around 42&#37; of patients&#46; As TS alone carries with it an increased intrinsic risk for HTN&#44; related to the morbidity and mortality of the syndrome&#44; HTN should be carefully diagnosed and treated&#46;<a class="elsevierStyleCrossRefs" href="#bib0470"><span class="elsevierStyleSup">34&#44;50</span></a></p><p id="par0260" class="elsevierStylePara elsevierViewall">HTN in TS is commonly seen in the absence of cardiac or renal malformations and its pathophysiology is poorly understood and complex&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">19</span></a> Some of the mechanisms that seem to be involved in HTN are increased set risk factors for HTN which are often present in TS&#44; such as obesity&#44; type-1 and type-2 diabetes&#44; dyslipidemia&#44; oxidative stress and inflammation&#44; impaired insulin-mediated vasodilatation and abnormal sodium processing by the kidneys&#46; The role of the inappropriate activation of the renin-angiotensin-aldosterone &#40;RAA&#41; system remains uncertain&#44; but overactivation of the sympathetic nervous system likely plays a major part&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">34</span></a> Estrogen deficiency contributes to blood-pressure &#40;BP&#41; imbalance&#44; because estrogens regulate vascular function by modulating some biological cascades&#44; such as the RAA and endothelin systems&#46; They also have an antioxidant effect and a positive correlation with renal function&#46;<a class="elsevierStyleCrossRefs" href="#bib0470"><span class="elsevierStyleSup">34&#44;51&#8211;53</span></a></p><p id="par0265" class="elsevierStylePara elsevierViewall">Aortic root dilatation is closely dependent on BP&#44; suggesting that rigorous treatment of HTN may reduce the risk of aortic dilatation progression&#46; In addition to that&#44; a significant association between abnormal aortic arch morphology and HTN has been demonstrated&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">19&#44;54</span></a></p><p id="par0270" class="elsevierStylePara elsevierViewall">CoA&#44; even if repaired in the neonatal period&#44; is also a significant risk factor for HTN in TS&#44; suggesting that prenatal or early postnatal hypoperfusion of the kidneys may play a pathogenic role&#46; Haploinsufficiency of critical genes on the X chromosome may be related to the established TS vasculopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0550"><span class="elsevierStyleSup">50</span></a></p><p id="par0275" class="elsevierStylePara elsevierViewall">There is no evidence to support specific HTN treatments in TS&#44; and the choice of therapeutic agent should follow general guidelines when BP reduction is the primary objective&#46;<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">16</span></a> Target BP should be less than 135&#47;85 mmHg&#44; and in those who have congenital heart defects&#44; such as BAV&#44; CoA and dilatation of the aorta &#40;ASI &#8805;2&#46;0 cm&#47;m<span class="elsevierStyleSup">2</span>&#41;&#44; should be less than 130&#47;80 mmHg&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">34</span></a></p></span></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Recommendations for cardiovascular risk screening</span><p id="par0280" class="elsevierStylePara elsevierViewall">Awareness of considerable CV risk requires effective preventive medicine from the earliest years of life&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">35</span></a> Therefore&#44; an appropriate and rigorous CV evaluation with the spectrum of CV issues encountered in TS should be performed &#40;<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>&#41;&#46; The best cardiac imaging modalities for visualizing each of the congenital CV anomalies are shown in <a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a>&#46;</p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia><elsevierMultimedia ident="tbl0025"></elsevierMultimedia><p id="par0285" class="elsevierStylePara elsevierViewall">At diagnosis&#44; all infants and children with TS should undergo comprehensive CV evaluation&#44; including clinical examination&#44; electrocardiogram &#40;ECG&#41; to detect conduction abnormalities&#44; BP measurement and echocardiography&#44; which are currently the standard of care to assess the cardiac anatomy of girls with TS&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">2&#44;55</span></a></p><p id="par0290" class="elsevierStylePara elsevierViewall">CMR is recommended in TS children from the age of 12 &#40;when it can be performed without sedation&#41;&#44; even if no cardiac anomalies are detected on echocardiography&#46; Indeed&#44; CMR is the gold-standard imaging test for the diagnosis and monitoring of morphological anomalies of the thoracic aorta in TS&#44; since echocardiography underestimates the size of both the ascending and the descending aortas&#46;<a class="elsevierStyleCrossRefs" href="#bib0325"><span class="elsevierStyleSup">5&#44;18</span></a></p><p id="par0295" class="elsevierStylePara elsevierViewall">For outpatients&#44; alternating echocardiography and CMR may be helpful&#46; Aortic diameter in TS patients with no risk factors for aortic dissection &#40;BAV&#44; CoA and&#47;or increased aortic dimensions&#41; should be re-evaluated every 5-10 years&#46; In patients considered to be at high risk&#44; CMR should be repeated sooner&#46;<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">21</span></a><a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a> summarizes a reasonable imaging approach for these patients&#46;</p><p id="par0300" class="elsevierStylePara elsevierViewall">In the acute setting&#44; CMR is not useful due to the relatively long examination time and difficulty in monitoring unstable patients&#46; ECG-gated CT aortography is a valuable alternative for obtaining images of the entire aorta and aortic branch vessels including coronary arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Pregnancy</span><p id="par0305" class="elsevierStylePara elsevierViewall">As mentioned above&#44; ovarian failure is a typical feature in TS with no or incomplete development of secondary sexual characteristics and primary amenorrhea&#46; The X chromosome locus responsible for primary ovarian insufficiency in TS is probably on the short arm &#40;Xq&#41;&#46; However&#44; 2-8&#37; experience spontaneous pregnancy&#44; and advances in assisted reproductive medicine with donated oocytes have been used&#44; enabling successful pregnancies to be achieved&#46;<a class="elsevierStyleCrossRefs" href="#bib0315"><span class="elsevierStyleSup">3&#44;56</span></a></p><p id="par0310" class="elsevierStylePara elsevierViewall">These pregnancies carry substantial fetal and maternal risks&#44; exerting significant stress on the heart and aorta &#40;with an increase in stroke volume and rise in estrogen level&#41; and hypertensive disorders such as pre-eclampsia that may directly promote vascular damage and aneurysm formation&#46; If uncontrolled&#44; this can lead to aortic rupture and dissection&#46;<a class="elsevierStyleCrossRefs" href="#bib0315"><span class="elsevierStyleSup">3&#44;18</span></a></p><p id="par0315" class="elsevierStylePara elsevierViewall">The risk of death during pregnancy from aortic dissection and rupture may be 2&#37; or higher in women with TS&#46;<a class="elsevierStyleCrossRef" href="#bib0585"><span class="elsevierStyleSup">57</span></a></p><p id="par0320" class="elsevierStylePara elsevierViewall">Thus&#44; careful follow-up&#44; including echocardiography and CMR performed by trained specialists familiar with TS&#44; is essential for patients who intend to become pregnant&#46;<a class="elsevierStyleCrossRef" href="#bib0590"><span class="elsevierStyleSup">58</span></a></p><p id="par0325" class="elsevierStylePara elsevierViewall">According to the 2008 publication of American Society of Reproductive Medicine&#44; TS is a relative contraindication for pregnancy&#44; but an absolute contraindication if there is a documented cardiac anomaly&#46; However&#44; the French task force 2011 recommendations&#44; summarized in <a class="elsevierStyleCrossRef" href="#tbl0030">Table 6</a>&#44; do not list an isolated BAV as a contraindication&#44; but rather a risk factor&#46;<a class="elsevierStyleCrossRefs" href="#bib0595"><span class="elsevierStyleSup">59&#44;60</span></a></p><elsevierMultimedia ident="tbl0030"></elsevierMultimedia><p id="par0330" class="elsevierStylePara elsevierViewall">If the ASI is less than 2&#46;5 cm&#47;m<span class="elsevierStyleSup">2</span> or the aortic diameter is less than 3&#46;5 cm&#44; and if there is no associated CoA&#44; pregnancy can be authorized although careful CV monitoring is required&#44; with an echocardiography suggested at the end of the first and the second trimesters and monthly during the third&#46; If a patient is pending oocyte donation and the aorta increases by 10&#37; or more in the annual echocardiography examination&#44; said increase must be confirmed via a second imaging technique &#40;CMR&#44; cardiac CT or transesophageal echocardiography&#41; and&#44; if confirmed&#44; pregnancy is contraindicated&#46;<a class="elsevierStyleCrossRef" href="#bib0595"><span class="elsevierStyleSup">59</span></a></p></span><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Final remarks</span><p id="par0335" class="elsevierStylePara elsevierViewall">In TS&#44; the detection of congenital CV anomalies and their continuous monitoring with the use of cardiac imaging methods&#44; particularly CMR &#40;now recommended for optimal cardiac risk assessment&#41;&#44; is of vital importance&#46;</p><p id="par0340" class="elsevierStylePara elsevierViewall">Aortic dissection&#44; an extremely common event in these women&#44; can occur at a remarkably young age and in pregnancy&#46; Therefore&#44; special attention to prevention and treatment should be given by monitoring the standard CV risk factors in TS&#46; This approach is crucial to avoid life-threatening complications&#46;</p><p id="par0345" class="elsevierStylePara elsevierViewall">Pregnancy&#44; which is not often spontaneous and can be achieved through assisted reproduction&#44; increases risk in women with TS&#46; Contraindications to pregnancy are defined&#44; but even if none are present&#44; patients should be carefully monitored before and during pregnancy&#46;</p><p id="par0350" class="elsevierStylePara elsevierViewall">To sum up&#44; it is clear that TS is associated with several CV anomalies and risk factors present at an early age&#44; which contribute to high TS mortality and morbidity&#46; However&#44; there are numerous unresolved issues that require further research&#46;</p><p id="par0355" class="elsevierStylePara elsevierViewall">Firstly&#44; studies are warranted to clarify the pathophysiology of CV risk and the development of congenital CV anomalies&#46; TS aortopathy has been postulated as a connective disorder&#44; a theory that has not yet been confirmed&#46; Determining the etiology will enable offering potential therapies to prevent aortic dissection&#46; It will also provide information about potential risk stratification and&#44; therefore&#44; help determine the frequency of cardiovascular screening &#40;which is currently not appropriately addressed&#41;&#46;</p><p id="par0360" class="elsevierStylePara elsevierViewall">Furthermore&#44; the cut-off for aortic dilatation that puts an individual at high risk of aortic dissection in TS remains unclear&#46; As such&#44; the relationship between aortic dimensions and aortic dissection needs to be defined&#44; and the adjustment of aortic dimensions for younger patients and those with lower BSA needs clarification&#46;</p><p id="par0365" class="elsevierStylePara elsevierViewall">Finally&#44; the predictive values of aortic dimensions for prophylactic aortic surgery to prevent aortic dissection are undefined&#44; as are the efficacy and safety of the procedure&#46;</p></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Conflicts of interest</span><p id="par0370" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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          "titulo" => "Introduction"
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          "titulo" => "Congenital cardiovascular disorders"
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              "titulo" => "A&#46; Valvular anomaly"
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                  "titulo" => "A&#46;1&#46; Bicuspid aortic valve"
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              "titulo" => "B&#46; Aortic abnormalities"
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                  "titulo" => "B&#46;1&#46; Coarctation of the aorta"
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                  "titulo" => "B&#46;2&#46; Elongated transverse arch"
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                  "titulo" => "B&#46;3&#46; Aortic dilatation"
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                  "titulo" => "B&#46;4&#46; Aortic dissection"
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                  "titulo" => "C&#46;1&#46; Partial anomalous pulmonary venous connection"
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                  "titulo" => "C&#46;2&#46; Persistent left superior vena cava"
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              "titulo" => "D&#46; Other congenital cardiovascular disorders"
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          "titulo" => "Cardiovascular risk"
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              "identificador" => "sec0080"
              "titulo" => "A&#46; Specific aspects of vascular structure and physiology"
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              "identificador" => "sec0085"
              "titulo" => "B&#46; Glucose metabolism&#44; body composition and physical activity"
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              "titulo" => "C&#46; Lipid profile"
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              "titulo" => "D&#46; Hypertension"
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          "titulo" => "Recommendations for cardiovascular risk screening"
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          "titulo" => "Pregnancy"
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            2 => "Risk factors"
            3 => "Female"
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            0 => "S&#237;ndrome de Turner"
            1 => "Doen&#231;as cardiovasculares"
            2 => "Fatores de risco"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Turner syndrome is a relatively common genetic disorder of female development&#44; characterized by partial or complete absence of an X chromosome&#44; with a variable clinical presentation&#46; Congenital or acquired cardiovascular disease is highly prevalent and a major cause of early death in this syndrome&#46; The most feared complication is aortic dissection&#44; which can occur at a very young age and requires careful assessment of its risk factors&#46; A systematic literature search identified sixty relevant publications&#46; These were reviewed with regard to the increased risk of cardiovascular disease in women with Turner syndrome&#44; especially in pregnancy&#46; The most common congenital cardiovascular defects are presented and illustrated with appropriate iconography&#46; The current recommendations regarding the screening and monitoring of cardiovascular disease in these patients are discussed&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A s&#237;ndrome de Turner &#233; uma condi&#231;&#227;o gen&#233;tica relativamente comum&#44; caracterizada pela perda total ou parcial de um cromossoma X&#44; com uma apresenta&#231;&#227;o cl&#237;nica vari&#225;vel e que afeta o desenvolvimento das mulheres&#46; Nessa s&#237;ndrome h&#225; uma preval&#234;ncia elevada de doen&#231;a cardiovascular&#44; cong&#233;nita e adquirida&#44; que condiciona um risco aumentado de morte prematura&#46; A complica&#231;&#227;o mais temida &#233; a disse&#231;&#227;o da aorta&#44; que tende a ocorrer em idades mais precoces&#44; implica uma avalia&#231;&#227;o cuidada dos seus fatores de risco&#46; A pesquisa sistem&#225;tica da literatura permitiu encontrar 60 artigos relevantes sobre o tema&#46; Foi feita uma revis&#227;o sobre o risco aumentado de doen&#231;a cardiovascular nas doentes com s&#237;ndrome de Turner&#44; em particular na gravidez&#46; Foram apresentados e ilustrados com iconografia pr&#243;pria os defeitos cardiovasculares cong&#233;nitos mais prevalentes&#46; Foram discutidas as recomenda&#231;&#245;es atuais relativas ao rastreio e &#224; vigil&#226;ncia da doen&#231;a cardiovascular nessas doentes&#46;</p></span>"
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        "descripcion" => array:1 [
          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Volume-rendered magnetic resonance aortography &#40;posterior-oblique view&#41; showing an aberrant right subclavian artery &#40;white arrow&#41; in a 37-year-old TS woman&#46;</p>"
        ]
      ]
      6 => array:8 [
        "identificador" => "tbl0005"
        "etiqueta" => "Table 1"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "detalles" => array:1 [
          0 => array:3 [
            "identificador" => "at1"
            "detalle" => "Table "
            "rol" => "short"
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        "tabla" => array:1 [
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Reference number&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Year of publication&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Type of study&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Number of patients&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Objective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">1</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To describe the spectrum of cardiovascular defects with particular attention to identifying risk factors for aortic dissection&#47;rupture&#46; X-chromosome genetic pathways implicated in Turner cardiovascular disease&#44; including premature coronary artery disease&#44; are discussed&#46; Recent guidelines for diagnosis and treatment of girls and women with TS are reviewed&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">2</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To provide updated guidelines for the evaluation and treatment of girls and women with Turner syndrome &#40;TS&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">3</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review publications since 2000 concerning maternal outcomes for pregnancies in women with TS to determine if specific risk factors such as type of pregnancy&#44; age or presence of underlying congenital cardiovascular disease may identify women at special risk&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Marin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">4</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review cardiovascular risks in this group of patients and discuss a systematic imaging approach for early identification of cardiovascular disorders in these patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mortensen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">5</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the state of the art of cardiovascular imaging in Turner syndrome&#44; emphasizing unresolved issues in the care of these patients with links to appropriate multimodality imaging strategies&#44; both in acute and chronic presentations and in symptomatic and asymptomatic patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hiratzka et al&#46;<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">6</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2010&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Guidelines&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Granger et al&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">7</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To summarize the cardiovascular&#44; neurological&#44; genitourinary&#44; otolaryngological&#44; craniofacial&#44; and skeletal defects associated with TS&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Surerus et al&#46;<a class="elsevierStyleCrossRef" href="#bib0340"><span class="elsevierStyleSup">8</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2003&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">53&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare the incidence and type of heart disease found in association with 45X karyotype in fetal life with postnatal life and to examine the outcome after fetal diagnosis&#46;<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Loscalzo et al&#46;<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">9</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2005&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">134&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine whether there is a specific association between a history of fetal lymphedema and congenital cardiovascular defects in monosomy X&#44; or TS&#44; independent of karyotype or general severity of the phenotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Spectrum et al&#46;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">51&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To use cardiac MRI to describe the spectrum and frequency of cardiovascular abnormalities and to evaluate aortic dilatation and associated abnormalities in pediatric patients with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ho et al&#46;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2004&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">85 adults with TS and 27 normal female adult volunteers<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To better understand the prevalence and pathogenesis of cardiovascular defects in TS by evaluating a group of asymptomatic adult volunteers with TS using magnetic resonance angiography&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hjerrild et al&#46;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2010&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cross-sectional<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">102&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate aortic dimensions in women with Turner syndrome in relation to aortic valve morphology&#44; blood pressure&#44; karyotype and clinical characteristics&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Bondy et al&#46;<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">13</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To focus on emerging knowledge of the characteristics of aortic disease in TS in comparison with Marfan-like syndromes and isolated aortic valve disease&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sachdev et al&#46;<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">14</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">253&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence and characteristics of aortic valve disease in girls and women with monosomy for the X chromosome&#44; or Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Gutmark-Little et al&#46;<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">15</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">39&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence and hemodynamic significance of partial anomalous pulmonary venous return in adolescents and young adults with TS using transthoracic echocardiogram and cardiac magnetic resonance&#44; and to study the association with phenotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mortensen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">16</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To provide insight into pathogenesis of Turner syndrome with perspectives to advances in the understanding of genetics of the X-chromosome&#46; The review also incorporates important endocrine features&#44; in order to comprehensively explain the cardiovascular phenotype and to highlight how increased attention to endocrinology and genetics is important in the identification and modification of cardiovascular risk&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Turtle et al&#46;<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">17</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the literature on the key risk factors for aortic dissection&#44; and to propose recommendations on the optimal imaging for congenital heart defects and aortic dilatation&#44; and on the assessment and management of blood pressure in this patient group&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Turtle et al&#46;<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">18</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the literature on cardiovascular disease in women with TS and make recommendations based on relatively limited high-quality evidence&#44; together with our experience&#44; on the optimal timing of cardiovascular screening&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Elsheikh et al&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">19</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2001&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">38&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the prevalence of aortic root dilatation in a group of women with TS and to investigate the factors contributing to its development&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sharma et al&#46;<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">20</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2009&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective cross-sectional&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess utility of aortic distensibility as a measure of aortic elasticity for the stratification of the risk for aortic dilation&#44; and its relationship with age&#44; karyotype and hormonal therapy&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Wong et al&#46;<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">21</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To raise awareness of the risk of aortic dissection in groups of adolescents and young adults amongst clinicians of all backgrounds &#40;pediatric and adult endocrinologists&#44; pediatric and adult cardiologists&#44; general pediatricians and adolescent physicians&#44; adult physicians&#44; emergency specialists and general practitioners&#41;&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mortensen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">22</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">80 adult TS patients and 67 healthy-age and gender-matched controls<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To prospectively assess aortic dimensions in TS&#46;<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lanzarini et al&#46;<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">23</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">78&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the prevalence of aortic root and proximal thoracic aorta enlargement by 2-dimensional echocardiography and the evolution of aortic diameters over time&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Carlson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0420"><span class="elsevierStyleSup">24</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">20&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To describe the signs&#44; symptoms and echocardiographic features preceding aortic dissection in 20 women with TS&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Matura et al&#46;<a class="elsevierStyleCrossRef" href="#bib0425"><span class="elsevierStyleSup">25</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">166 adult volunteers with TS who were not selected for cardiovascular disease and 26 healthy female control subjects&#46;<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate aortic diameters measured by magnetic resonance in a large group of women with TS to elucidate factors associated with variation in ascending aortic size and to compare methods to identify potentially ominous dilatation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Gravhoolt et al&#46;<a class="elsevierStyleCrossRef" href="#bib0430"><span class="elsevierStyleSup">26</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To describe in more detail aortic dissection as encountered in Turner&#39;s syndrome&#44; giving attention to clinical&#44; histological and epidemiological aspects&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lopez et al&#46;<a class="elsevierStyleCrossRef" href="#bib0435"><span class="elsevierStyleSup">27</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">138&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To study the prevalence&#44; magnitude&#44; and determinants of aortic dilation in a large group of girls and young women with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lawson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0440"><span class="elsevierStyleSup">28</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">49 Turner syndrome patients and 76 lean and 52 obese controls&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether vasculopathy can be detected in young TS patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Carlson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0445"><span class="elsevierStyleSup">29</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review &#43; case report&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Two case reports and a review of 85 cases&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To discuss two cases in which only the diagnosis of TS was present and other predisposing risk factors&#44; such as congenital heart disease or systemic hypertension&#44; were expressly absent&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Gutmark-Little et al&#46;<a class="elsevierStyleCrossRef" href="#bib0450"><span class="elsevierStyleSup">30</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">39&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence and hemodynamic significance of partial anomalous pulmonary venous return in adolescents and young adults with TS using transthoracic echocardiogram and cardiac magnetic resonance&#44; and to study the association with phenotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sears et al&#46;<a class="elsevierStyleCrossRef" href="#bib0455"><span class="elsevierStyleSup">31</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Case report&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To discuss two adult patients who presented with pulmonary hypertension&#44; and evidence of right ventricular hypertrophy and dysfunction&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Povoski et al&#46;<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">32</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Case report with literature review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the available literature as it pertains to the specific congenital venous anomaly of the thoracic systemic venous return&#44; persistent left superior vena cava&#44; and to discuss the clinical implications and relevance of congenital aberrancies&#44; as well as of treatment-induced or disease-induced alterations in the anatomy of the thoracic central venous system&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Proprawski et al&#46;<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">33</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2009&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">34&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the cardiovascular system by clinical examination&#44; echocardiography and electrocardiography in female patients with Turner syndrome&#44; depending on karyotype&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">De Groote et al&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">34</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare current treatment strategies and also propose an integrated practical approach for the diagnosis and treatment of hypertension in Turner syndrome applicable in daily practice&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Kozlowka-Wojciechowska et al&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">35</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine the prevalence of classic risk factors of atherosclerosis in a group of young Polish women with Turner syndrome in comparison to a representative group of young Polish women from the general population&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ostberg et al&#46;<a class="elsevierStyleCrossRef" href="#bib0480"><span class="elsevierStyleSup">36</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2005&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">93 TS women&#44; 25 normal controls and 11 women with 46&#44;XX primary amenorrhea&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether TS women have a fundamental arterial wall defect that may be due to genetic factors or estrogen deficiency&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Trolle et al&#46;<a class="elsevierStyleCrossRef" href="#bib0485"><span class="elsevierStyleSup">37</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To discuss the effects of estrogen and androgen insufficiency as well as the effects of sex hormone replacement therapy &#40;HRT&#41; on morbidity and mortality with special emphasis on evidence-based research and areas needing further studies&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Groote et al&#46;<a class="elsevierStyleCrossRef" href="#bib0490"><span class="elsevierStyleSup">38</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective case &#8211; control&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15 prepubertal<br>Turner syndrome girls and 31 sex-&#44; age- and height-matched healthy controls<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To examine whether abnormal rigidity of the aorta is present from a young age and if it is associated with an increased carotid artery diameter or intima-media thickness&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Elsheikh et al&#46;<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">39</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2000&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">21&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess the degree to which HRT may be protective against ischemic heart disease in women with TS by measuring arterial stiffness&#44; blood pressure&#44; insulin sensitivity and lipids&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ostberg et al&#46;<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">40</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2007&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To study the influence of increasing doses of HRT on markers of metabolism and vascular physiology&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Chan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0505"><span class="elsevierStyleSup">41</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2002&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To examine the effect of HRT on endothelial function of forearm resistance vessels in women with Turner&#39;s syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Colao et al&#46;<a class="elsevierStyleCrossRef" href="#bib0510"><span class="elsevierStyleSup">42</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2008&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To review the role of the growth hormone &#40;GH&#41; and insulin-like growth factor I system on the cardiovascular system&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">O&#8217;Gorman et al&#46;<a class="elsevierStyleCrossRef" href="#bib0515"><span class="elsevierStyleSup">43</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2013&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cross-sectional cohort&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">19 TS and 17 control girls<br>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare cardiometabolic risk factors and measures of subcutaneous&#44; visceral adipose tissue and intra-myocellular lipid between young TS girls and an age- and body mass index-standard deviation scores-matched healthy female cohort&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Salgin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">44</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16 TS women and 16 normal healthy women&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine whether the defect in insulin sensitivity is a primary intrinsic defect in TS or dependent on variation in body composition&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mazzanti et al&#46;<a class="elsevierStyleCrossRef" href="#bib0525"><span class="elsevierStyleSup">45</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Mixed longitudinal and cross-sectional&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">46&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To analyze carbohydrate tolerance and insulin sensitivity in patients with TS who reached their final height after long-term &#40;10 years&#41; treatment with high-dose GH&#44; and in those who continued to be followed-up after the cessation of GH therapy&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ladin-Wilhelmsen et al&#46;<a class="elsevierStyleCrossRef" href="#bib0530"><span class="elsevierStyleSup">46</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2001&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">100&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To analyze the prevalence of cardiovascular risk factors&#44; especially diabetes mellitus&#44; blood lipids&#44; fibrinogen&#44; and cardiovascular malformations&#44; as well as sex hormones in a large group of adult TS women and compare them with a random population sample of similar age from the same region&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Akyrek et al&#46;<a class="elsevierStyleCrossRef" href="#bib0535"><span class="elsevierStyleSup">47</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">29 TS and 29 healthy children and adolescents&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To evaluate the relationship between periaortic adipose thickness and metabolic data in a group of children with TS&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Pirgon et al&#46;<a class="elsevierStyleCrossRef" href="#bib0540"><span class="elsevierStyleSup">48</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24 TS and 24 healthy children&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To determine whether there are risk factors for atherosclerosis in children with TS&#44; carotid artery intima-media thickness&#44; anthropometric and metabolic parameters were compared between children with TS and healthy controls&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Irzyniec et al&#46;<a class="elsevierStyleCrossRef" href="#bib0545"><span class="elsevierStyleSup">49</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">165&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To assess lipid and carbohydrate metabolism in TS women in the context of HRT as well as GH treatment during childhood&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Los et al&#46;<a class="elsevierStyleCrossRef" href="#bib0550"><span class="elsevierStyleSup">50</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">168&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To identify the factors associated with hypertension in girls with Turner syndrome by studying the prevalence of elevated systemic blood pressure&#44; awareness of the problem&#44; and its clinical associations in a large cohort of girls with Turner syndrome&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Zuckerman-Levin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0555"><span class="elsevierStyleSup">51</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2006&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10 young women with Turner syndrome and 10 age-matched healthy women&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether women with Turner syndrome have reduced catecholaminergic and physiological response to sympathetic stimulation&#44; and whether changes in blood pressure and heart rate are related to their catecholamine response to sympathetic stimulation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Maric-Bilkan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0560"><span class="elsevierStyleSup">52</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2014&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To summarize the current understanding of the mechanisms by which estrogens regulate blood pressure and the potential use of hormone therapy in prevention of hypertension and consequent cardiovascular risk&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lastra et al&#46;<a class="elsevierStyleCrossRef" href="#bib0565"><span class="elsevierStyleSup">53</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">An update of type 2 diabetes mellitus and hypertension&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Herck et al&#46;<a class="elsevierStyleCrossRef" href="#bib0570"><span class="elsevierStyleSup">54</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2015&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">74&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To investigate whether TS patients with an abnormal arch morphology are more at risk for hypertension&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Somerville et al&#46;<a class="elsevierStyleCrossRef" href="#bib0575"><span class="elsevierStyleSup">55</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2016&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Prospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To compare the detection of cardiac lesions with the use of cardiac magnetic resonance imaging and conventional echocardiography in children with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Nwosu et al&#46;<a class="elsevierStyleCrossRef" href="#bib0580"><span class="elsevierStyleSup">56</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Case report with literature review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To present the case of a woman with Turner syndrome achieving a successful pregnancy from a donor oocyte and review the relevant literature&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><a class="elsevierStyleCrossRef" href="#bib0585"><span class="elsevierStyleSup">57</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To evaluate the impact of Turner syndrome on pregnancy outcomes after oocyte donation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Boissonnas et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0590"><span class="elsevierStyleSup">58</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2011&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Retrospective&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">To evaluate the follow-up &#40;mainly cardiovascular&#41; of women with TS requesting oocyte donation&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cabanes et al&#46;<a class="elsevierStyleCrossRef" href="#bib0595"><span class="elsevierStyleSup">59</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2010&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Recommendations for pregnancy in Turner syndrome patients&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Karnis et al&#46;<a class="elsevierStyleCrossRef" href="#bib0600"><span class="elsevierStyleSup">60</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2012&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Review&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Guidelines for pre-conception screening and counseling now exist that may mitigate the maternal and fetal risks associated with pregnancy in women with Turner syndrome&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Congenital heart defect&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Frequency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Aortic anomalies&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Elongated transverse arch&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">31&#46;4&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a> 49&#37;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Aortic dilatation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">23&#37;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Aortic dissection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">100-fold increased risk<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">13</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Coarctation of the aorta&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">12&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a> 15&#46;7&#37;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Valvular anomaly&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Bicuspid aortic valve&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">12</span></a> 30&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">14</span></a> 39&#46;2&#37;<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">10</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Venous anomalies&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Partial anomalous venous connection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&#37;&#44;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a> 15&#46;7&#37;&#44;<span class="elsevierStyleSup">10</span>18&#37;<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">15</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Persistent left superior vena cava&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&#37;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Arch vessel anomaly&#58;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Aberrant right subclavian artery&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&#37;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">11</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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                            0 => "C&#46;A&#46; Bondy"
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                      "doi" => "10.1111/j.1747-0803.2007.00163.x"
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