Self-Esteem and Social Adjustment in Young Women with Turner Syndrome—Influence of Pubertal Management and Sexuality: Population-Based Cohort Study

Carel, Jean‐Claude; Elie, Caroline; Écosse, Emmanuel; Tauber, Maïthé; Léger, Juliane; Cabrol, Sylvie; Nicolino, Marc; Brauner, Raja; Chaussain, Jean‐Louis; Coste, Joël

doi:10.1210/jc.2005-2652

Cited by 134 publications

(107 citation statements)

References 41 publications

(46 reference statements)

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“…It also indicates that the phenotype of the TS persons diagnosed late is not as affected as those diagnosed early, as shown previously in a Swedish cohort (12). Nevertheless, we assume that a puberty as similar to peers as possible in onset and timing is important for the individual TS person, in line with previous findings of an increased quality of life in those with spontaneous pubertal timing (13).…”

Section: Discussionsupporting

confidence: 89%

Socioeconomic parameters and mortality in Turner syndrome

Stochholm

Hjerrild

Mortensen

et al. 2012

European Journal of Endocrinology

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Background: Turner syndrome (TS) is characterized by hypogonadism, short adult height, increased morbidity and mortality, contrasted by self-reported normal quality of life and perception of health. Small studies have indicated a similar level of education compared with the background population. Aim: To study the socioeconomic profile in TS and the impact of these factors on mortality. Materials and methods: Register study using Danish nationwide registries. Nine hundred and seventynine TS females and 94 850 controls were included. Information concerning cohabitation, motherhoods, level of education (bachelor degree), income, retirement, and death were obtained. One hundred and three TS and 5989 controls died during the study period. For the socioeconomic parameters, median age at first relevant episode was calculated. Income was analyzed using conditional logistic regression and the other parameters using Cox regression. Results: In comparison with controls, TS had significantly fewer partnerships (hazard ratio (HR): 0.45), fewer motherhoods (HR: 0.18), and retired earlier (HR: 1.8). After the diagnosis of TS, the risk of retiring was increased. Educational attainment (HR: 1.0) as well as risk of unemployment was similar. Before the age of 30, low income was significantly more frequent; hereafter, it was similar to controls. Mortality was significantly increased (HR: 2.9) and slightly lower after adjustment for cohabitation and education (HR: 2.7). Conclusions: A divergent socioeconomic profile is apparent, with a reduced proportion of TS persons finding a partner and becoming mothers. The educational level was similar to controls. The increased mortality in TS was not materially affected after adjustment for cohabitation and education.

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Section: Discussionsupporting

confidence: 89%

Socioeconomic parameters and mortality in Turner syndrome

Stochholm

Hjerrild

Mortensen

et al. 2012

European Journal of Endocrinology

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“…However, in recent years, studies have shown that initiation of very low-dose estrogen does not seem to limit growth. Moreover, more attention is now focused on the psychosocial adjustment of these girls, which seems to be improved when they mature through puberty in concert with their peers [13]. The literature on this evolving issue, in addition to the current consensus recommendations have been summarized recently [14].…”

mentioning

confidence: 99%

Turner syndrome in childhood and adolescence

McCarthy

Bondy²

2008

Expert Review of Endocrinology & Metabolism

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This article reviews current patterns of ascertainment, clinical characteristics and quality of care for girls with Turner syndrome, based on a cohort of 100 girls (aged 7-17 years) prospectively evaluated at the National Institute of Child Health since 2001. Approximately 25% were diagnosed prenatally or at birth owing to webbed neck and other features typical of fetal lymphedema, few were diagnosed during early childhood, with the majority undiagnosed until age 9 years or older. Major clinical features included thyroid autoimmunity (51%), congenital cardiovascular anomalies (44%), liver abnormalities (36%), hypertension (34%), hearing loss (30%) and renal anomalies (18%). Of the group, 75% were being or had been treated with growth hormone. These girls were an average of 5 cm taller and significantly less obese than the untreated group. We discuss new guidelines for the initiation of puberty and urgent research needed to promote the health and longevity of girls suffering from Turner syndrome as they become adults. Keywords aorta; bicuspid aortic valve; lymphedema; phenotype; short stature; Turner syndrome; X chromosome Turner syndrome (TS) is a relatively common disorder of female development (affecting one out of 2000 births) caused by complete or partial monosomy for the X chromosome during embryonic development. The most consistent features are short stature and premature ovarian failure. Full-scale IQ is usually normal, with verbal scores typically greater than performance scores [1]. With timely diagnosis and intervention, girls with TS may gain improved height, benefit from attention to nonverbal learning difficulties early in school and have puberty at an age similar to their peers. Importantly, all these girls need comprehensive cardiovascular evaluation, since over a third have an underlying congenital cardiovascular defect that places them at an increased risk for life-threatening complications, such as pulmonary hypertension (e.g., partial anomalous pulmonary venous connection) or aortic dissection (i.e., bicuspid aortic valve or dilated ascending aorta). This article focuses on patterns of diagnosis and growth hormone (GH) (59%) are 45,X. The average age of our pediatric study subjects is 12.5 years, with a median of 13 years. The age of diagnosis demonstrates a distinct biphasic pattern ( Figure 1). Four girls were diagnosed prenatally, either incidentally during routine screening or because of an abnormal fetal ultrasound [2]. In total, 18 were diagnosed at birth because of neck webbing and associated signs of lymphedema and, in many cases, severe congenital heart disease. Surprisingly, very few girls were diagnosed during early childhood, despite the fact that growth failure is usually evident from shortly after birth [3], with 60% of girls not diagnosed until age 9 years or later. PhenotypeCommon phenotypic features of the NIH pediatric TS study group are summarized in Table 1. Only 22% of the group had neck webbing, or pterygium colli. This feature is defined as the presence of (u...

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“…[25][26][27][28][29][30][31] Of these, three had used Short Form questionnaire-36 items (SF-36) as a tool to measure QoL. [27][28][29] All but one of the studies had evaluated the impact of growth hormone on QoL.…”

Section: Resultsmentioning

confidence: 99%

Growth monitoring for short stature: update of a systematic review and economic model

Craig

Fayter²,

Stirk³

et al. 2011

Health Technol Assess

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An electronic version of this title, in Adobe Acrobat format, is available for downloading free of charge for personal use from the HTA website (www.hta.ac.uk). A fully searchable DVD is also available (see below).Printed copies of HTA journal series issues cost £20 each (post and packing free in the UK) to both public and private sector purchasers from our despatch agents.Non-UK purchasers will have to pay a small fee for post and packing. For European countries the cost is £2 per issue and for the rest of the world £3 per issue. How to order:-fax (with credit card details) -post (with credit card details or cheque) -phone during office hours (credit card only).Additionally the HTA website allows you to either print out your order or download a blank order form. Contact details are as follows:Synergie UK (HTA Department) Digital House, The Loddon Centre Wade Road Basingstoke Hants RG24 8QW Email: orders@hta.ac.uk Tel: 0845 812 4000 -ask for 'HTA Payment Services' (out-of-hours answer-phone service) Fax: 0845 812 4001 -put 'HTA Order' on the fax header Payment methods Paying by chequeIf you pay by cheque, the cheque must be in pounds sterling, made payable to University of Southampton and drawn on a bank with a UK address.Paying by credit card You can order using your credit card by phone, fax or post. SubscriptionsNHS libraries can subscribe free of charge. Public libraries can subscribe at a reduced cost of £100 for each volume (normally comprising 40-50 titles). The commercial subscription rate is £400 per volume (addresses within the UK) and £600 per volume (addresses outside the UK). Please see our website for details. Subscriptions can be purchased only for the current or forthcoming volume.How do I get a copy of HTA on DVD?Please use the form on the HTA website (www.hta.ac.uk/htacd/index.shtml). HTA on DVD is currently free of charge worldwide.The website also provides information about the HTA programme and lists the membership of the various committees. ii ii HTA NIHR Health Technology Assessment programmeThe Health Technology Assessment (HTA) programme, part of the National Institute for Health Research (NIHR), was set up in 1993. It produces high-quality research information on the effectiveness, costs and broader impact of health technologies for those who use, manage and provide care in the NHS. 'Health technologies' are broadly defined as all interventions used to promote health, prevent and treat disease, and improve rehabilitation and long-term care. The research findings from the HTA programme directly influence decision-making bodies such as the National Institute for Health and Clinical Excellence (NICE) and the National Screening Committee (NSC). HTA findings also help to improve the quality of clinical practice in the NHS indirectly in that they form a key component of the 'National Knowledge Service' . The HTA programme is needs led in that it fills gaps in the evidence needed by the NHS. There are three routes to the start of projects. First is the commissioned route. Suggestions for resea...

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Self-Esteem and Social Adjustment in Young Women with Turner Syndrome—Influence of Pubertal Management and Sexuality: Population-Based Cohort Study

Cited by 134 publications

References 41 publications

Socioeconomic parameters and mortality in Turner syndrome

Socioeconomic parameters and mortality in Turner syndrome

Turner syndrome in childhood and adolescence

Growth monitoring for short stature: update of a systematic review and economic model

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