Dataset Information

Cardiometabolic and vascular risks in young and adolescent girls with Turner syndrome.

ABSTRACT:

Background

Turner syndrome (TS) is the most common chromosomal abnormality in females and is associated with several co-morbidities. It commonly results from X monosomy which is diagnosed on a 30 cell karyotype. Congenital heart disease is a clinical feature in 30% of cases. It is becoming evident that TS patients have an increased risk of cardiovascular and cerebrovascular diseases.

Scope of review

This review provides a detailed overview of the literature surrounding cardiometabolic health in childhood and adolescent TS. In addition, the review also summarises the current data on the impact of growth hormone (GH) therapy on cardiometabolic risk in paediatric TS patients.

Major conclusions

Current epidemiological evidence suggests that young women and girls with TS have unfavourable cardiometabolic risk factors which predispose them to adverse cardiac and cerebrovascular outcomes in young adulthood. It remains unclear whether this risk is the result of unidentified factors which are intrinsic to TS, or whether modifiable risk factors (obesity, hypertension, hyperglycaemia) are contributing to this risk.

General significance

From a clinical perspective, this review highlights the importance of regular screening and pro-active management of cardiometabolic risk from childhood in TS cohorts and that future research should aim to address whether modification of these variables at a young age can alter the disease process and atherosclerotic outcomes in adulthood.

SUBMITTER: Mavinkurve M

PROVIDER: S-EPMC4661589 | biostudies-literature | 2015 Jun

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Cardiometabolic and vascular risks in young and adolescent girls with Turner syndrome.

Mavinkurve Meenal M O'Gorman Clodagh S CS

BBA clinical 20150430

<h4>Background</h4>Turner syndrome (TS) is the most common chromosomal abnormality in females and is associated with several co-morbidities. It commonly results from X monosomy which is diagnosed on a 30 cell karyotype. Congenital heart disease is a clinical feature in 30% of cases. It is becoming evident that TS patients have an increased risk of cardiovascular and cerebrovascular diseases.<h4>Scope of review</h4>This review provides a detailed overview of the literature surrounding cardiometab ...[more]

PMID: 26673162

Similar Datasets

Project description:ObjectiveMost girls and women with Turner syndrome (TS) require estrogen replacement therapy (ERT) to initiate or maintain pubertal development. Most likely, the most fundamental effect of ERT in hypogonadism is the promotion of uterine growth. The optimal ERT model is still being discussed. The present study aimed to assess uterine size in girls with TS in the prepubertal state during and after the induction of puberty and compare it to a healthy population.MethodsThe analysis encompassed 40 TS girls. The prepubertal and postpubertal control groups contained 20 healthy girls each. All patients with TS were treated with 17-ß estradiol. Uterine imaging was performed with two-dimensional (2D) transabdominal ultrasound. The uterine volume (UV) and fundocervical antero-posterior ratio (FCR) were calculated in patients with TS before the pubertal induction, after 6-12 months of estrogen replacement therapy (ERT), after ≥ 36 months of ERT or ≥ 12 months after menarche.ResultsThe average age of TS patients at estrogen introduction and at the last control visit, when the uterus was considered mature, was 12.9 years and 16.1 years, respectively. The UV in patients with TS at the beginning of ERT was 1.55 ± 1.22 cm3 and was not significantly different from the UV in the prepubertal controls. The mature UV in patients with TS was 31.04 ± 11.78 cm3 and was significantly smaller than the UV of the postpubertal controls (45.68 ± 12.51 cm3, p<0.001). The FCR in girls with TS did not differ significantly from that in the prepubertal and postpubertal control groups, respectively. No prognostic factors could be established for the final UV. By the last control visit, thelarche had advanced in most patients to Tanner 4 and 5 (37.5% and 40%, respectively).ConclusionsBefore the onset of ERT, patients with TS have a uterus similar in size to that in prepubertal healthy girls. Pubertal induction in patients with TS causes a significant increase in the UV that is detectable after 6-12 months of ERT. The mature uterus is smaller in patients with TS than in the age-matched healthy population.

Project description:IntroductionHigh incidence of HIV infection among adolescent girls and young women (AGYW) has been attributed to the numerous and often layered vulnerabilities that they encounter including violence against women, unfavourable power relations that are worsened by age-disparate sexual relations, and limited access to sexual and reproductive health information and services. For AGYW living in urban informal settlements (slums), these vulnerabilities are compounded by pervasive poverty, fragmented social networks, and limited access to social services including health and education. In this paper, we assess sexual risk behaviours and their correlates among AGYW in two slum settlements in Nairobi, Kenya, prior to the implementation of interventions under the Determined Resilient Empowered AIDS-free Mentored and Safe (DREAMS) Partnership.MethodsWe drew on secondary data from the Transition to Adulthood study, the most recent representative study on adolescent sexual behaviour in the two settlements. The study was nested within the Nairobi Urban Health and Demographic Surveillance System (NUHDSS). Data were collected in 2009 from 1,390 AGYW aged 12-23 years. We estimated the proportions of AGYW reporting ever tested for HIV, condom use, multiple sexual partners and age-disparate sex by socio-demographic characteristics. "High risk" sexual behaviour was defined as a composite of these four variables and age at first sex. Multivariable regression analyses were performed to identify factors associated with risk behaviours.ResultsFifty-one percent of AGYW reported that they had ever tested for HIV and received results of their last test, with the proportion rising steeply by age (from 15% to 84% among those <15 years and 20-23 years, respectively). Of 578 AGYW who were sexually active in the 12 months preceding the survey, 26% reported using a condom at last sex, 4% had more than one sexual partner, and 26% had sex with men who were at least 5 years older or younger. All girls aged below 15 years who had sex (n = 9) had not used condoms at last sex. The likelihood of engaging in "high risk" sexual risk behaviour was higher among older AGYW (19-23 years), those in marital unions, of Luo ethnicity, out of school, living alone or with a friend (versus parents), living with spouse (versus parents), and those whose friends engaged in risky/anti-social behaviours. In contrast, Muslim faith, co-residence with both parents, and belonging to an organised social group were associated with lower odds of risky sexual behaviours.ConclusionOur study findings suggest that multifaceted approaches addressing the educational and social mediators of AGYW's vulnerability and that also reach the people with whom AGYW live and interact, are needed to reduce the rapid onset of sexual risk during the adolescent years. There is a particular need to reach the youngest adolescent girls in poor urban settings, among whom condom use and awareness of HIV status is rare.

Project description:BACKGROUND:The final adult height of untreated girls aged up to 18 years with Turner syndrome (TS) is approximately 20 cm shorter compared with healthy females. Treatment with growth hormone (GH) increases the adult height of people with TS. The effects of adding the androgen, oxandrolone, in addition to GH are unclear. Therefore, we conducted this systematic review to investigate the benefits and harms of oxandrolone as an adjuvant therapy for people with TS treated with GH. OBJECTIVES:To assess the effects of oxandrolone on growth hormone-treated girls aged up to 18 years with Turner syndrome. SEARCH METHODS:We searched CENTRAL, MEDLINE, Embase, the ICTRP Search Portal and ClinicalTrials.gov. The date of the last search was October 2018. We applied no language restrictions. SELECTION CRITERIA:We included randomised controlled clinical trials (RCTs) that enrolled girls aged up to 18 years with TS who were treated with GH and oxandrolone compared with GH only treatment. DATA COLLECTION AND ANALYSIS:Three review authors independently screened titles and abstracts for relevance, selected trials, extracted data and assessed risk of bias. We resolved disagreements by consensus, or by consultation with a fourth review author. We assessed trials for overall certainty of the evidence using the GRADE instrument. MAIN RESULTS:We included six trials with 498 participants with TS, 267 participants were randomised to oxandrolone plus GH treatment and 231 participants were randomised to GH only treatment. The individual trial sample size ranged between 22 and 133 participants. The included trials were conducted in 65 different paediatric endocrinology healthcare facilities including clinics, centres, hospitals and academia in the USA and Europe. The duration of interventions ranged between 3 and 7.6 years. The mean age of participants at start of therapy ranged from 9 to 12 years. Overall, we judged only one trial at low risk of bias in all domains and another trial at high risk of bias in most domains. We downgraded the level of evidence mainly because of imprecision (low number of trials, low number of participants or both). Comparing oxandrolone plus GH with GH only for final adult height showed a mean difference (MD) of 2.7 cm in favour of oxandrolone plus GH treatment (95% confidence interval (CI) 1.3 to 4.1; P < 0.001; 5 trials, 270 participants; moderate-quality evidence). The 95% prediction interval ranged between 0.3 cm and 5.1 cm. For adverse events, we based our main analysis on reliable date from two trials with overall low risk of bias. There was no evidence of a difference between oxandrolone plus GH and GH for adverse events (RR 1.81, 95% CI 0.83 to 3.96; P = 0.14; 2 trials, 170 participants; low-quality evidence). Six out of 86 (18.6%) participants receiving oxandrolone plus GH compared with 8/84 (9.5%) participants receiving GH only reported adverse events, mainly signs of virilisation (e.g. deepening of the voice). One trial each investigated the effects of treatments on speech (voice frequency; 88 participants), cognition (51 participants) and psychological status (106 participants). The overall results for these comparisons were inconclusive (very low-quality evidence). No trial reported on health-related quality of life or all-cause mortality. AUTHORS' CONCLUSIONS:Addition of oxandrolone to the GH therapy led to a modest increase in the final adult height of girls aged up to 18 years with TS. Adverse effects identified included virilising effects such as deepening of the voice, but reporting was inadequate in some trials.

Dataset Information

Cardiometabolic and vascular risks in young and adolescent girls with Turner syndrome.

Background

Scope of review

Major conclusions

General significance

Publications

Cardiometabolic and vascular risks in young and adolescent girls with Turner syndrome.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets