Cardiovascular imaging in Turner syndrome: state-of-the-art practice across the lifespan

Mortensen, Kristian H.; Young, Luciana; Backer, Julie De; Silberbach, Michael; Collins, R. Thomas; Duijnhouwer, Anthonie L.; Pandya, Bejal; Gravholt, Claus Højbjerg; Lopez, Leo; Roos‐Hesselink, Jolien W.

doi:10.1136/heartjnl-2017-312658

Cited by 28 publications

(17 citation statements)

References 53 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…MRI was not included in the latter study (20). This low number of MRIs performed in our study is particularly alarming, as the importance of cardiac MRI has already been described in a number of studies over several years (41, 42, 44).…”

Section: Discussionmentioning

confidence: 72%

Deficient knowledge in adult Turner syndrome care as an incentive to found Turner centers in Germany

Kahlert

Blaschke

Brockmann³

et al. 2019

Endocrine Connections

View full text Add to dashboard Cite

ObjectiveTurner syndrome (TS) is characterized by the complete or partial loss of the second sex chromosome and associated with a wide range of clinical manifestations. We aimed to assess the medical care of adult patients with TS in Germany.DesignRetrospective multicenter observational study.MethodsData were collected from medical records of 258 women with TS treated between 2001 and 2017 in five non-university endocrinologic centers in Germany.ResultsMean age was 29.8 ± 11.6 years, mean height 152 ± 7.7 cm, and mean BMI 26.6 ± 6.3 kg/m2. The karyotype was known in 50% of patients. Information on cholesterol state, liver enzymes, and thyroid status was available in 81–98% of women with TS; autoimmune thyroiditis was diagnosed in 37%. Echocardiography was performed in 42% and cardiac MRI in 8.5%, resulting in a diagnosis of cardiovascular disorder in 28%. Data on growth hormone therapy were available for 40 patients (15%) and data concerning menarche in 157 patients (61%).ConclusionIn 258 women with TS, retrospective analysis of healthcare data indicated that medical management was focused on endocrine manifestations. Further significant clinical features including cardiovascular disease, renal malformation, liver involvement, autoimmune diseases, hearing loss, and osteoporosis were only marginally if at all considered. Based on this evaluation and in accordance with recent guidelines, we compiled a documentation form facilitating the transition from pediatric to adult care and further medical management of TS patients. The foundation of Turner Centers in March 2019 will improve the treatment of TS women in Germany.

show abstract

Section: Discussionmentioning

confidence: 72%

Deficient knowledge in adult Turner syndrome care as an incentive to found Turner centers in Germany

Kahlert

Blaschke

Brockmann³

et al. 2019

Endocrine Connections

View full text Add to dashboard Cite

show abstract

“…All patients in our centre of expertise are seen according to a new value‐based healthcare clinical pathway which includes annual visits to the dedicated Turner outpatient clinic . Adult women with Turner syndrome who visited the outpatient clinic between December 2015 and October 2018 were prospectively included.…”

Section: Methodsmentioning

confidence: 99%

A value‐based healthcare approach: Health‐related quality of life and psychosocial functioning in women with Turner syndrome

Hoven

Bons

Dykgraaf

et al. 2020

Clinical Endocrinology

View full text Add to dashboard Cite

Objective: As part of the value-based healthcare programme in our hospital, a set of patient-reported outcome measures was developed together with patients and implemented in the dedicated Turner Syndrome (TS) outpatient clinic. This study aims to investigate different aspects of health-related quality of life (HR-QoL) and psychosocial functioning in women with TS in order to establish new possible targets for therapy.Design/Participants: A comprehensive set of questionnaires (EQ-5D, PSS-10, CIS-20, Ferti-QoL, FSFI) was developed and used to capture different aspects of HR-QoL and psychosocial functioning in a large cohort of adult women with Turner syndrome. All consecutive women, ≥18 years, who visited the outpatient clinic of our tertiary centre were eligible for inclusion. Results:Of the eligible 201 women who were invited to participate, 177 women (age 34 ± 12 years, mean ± SD) completed at least one of the validated questionnaires (88%). Women with TS reported a lower health-related quality of life (EQ-5D: 0.857 vs 0.892, P = .003), perceived more stress (PSS-10:14.7 vs 13.3; P = .012) and experienced increased fatigue (CIS-20: P < .001) compared to the general Dutch population. A relationship between noncardiac comorbidities (eg diabetes, orthopaedic complaints) and HR-QoL was found (R = .508). Conclusions:We showed that TS women suffer from impaired HR-QoL, more perceived stress and increased fatigue compared to healthy controls. A relationship between noncardiac comorbidities and HR-QoL was found. Especially perceived stress and increased fatigue can be considered targets for improvement of HR-QoL in TS women. | 435van den HOven et al.

show abstract

“…All patients in our centre of expertise are seen according to a new value-based healthcare clinical pathway which includes annual visits to the dedicated Turner outpatient clinic. 5,19 Adult women with Turner syndrome who visited the outpatient clinic between December 2015 and October 2018 were prospectively included.…”

Section: Setting and Study Designmentioning

confidence: 99%

“…Age, y 33 [18] Height, cm 157 [11] Weight, kg 64 [19] Systolic BP, mm Hg 125 [21] Diastolic BP, mm Hg 76 [17] Body ear and/or hearing problems (n = 57; 32%) and cardiovascular disease (n = 56; 32%). In the 56 women with cardiac disease, several different structural heart defects were identified, of which a bicuspid aortic valve (BAV) was the most frequent (19%).…”

Section: Women With Ts Median [Iqr]mentioning

confidence: 99%

A value-based healthcare approach: Health-related quality of life and psychosocial functioning in women with Turner syndrome

At¹,

Lr²,

Rhm³

et al. 2020

ESPE

View full text Add to dashboard Cite

show abstract

Cardiovascular imaging in Turner syndrome: state-of-the-art practice across the lifespan

Cited by 28 publications

References 53 publications

Deficient knowledge in adult Turner syndrome care as an incentive to found Turner centers in Germany

Deficient knowledge in adult Turner syndrome care as an incentive to found Turner centers in Germany

A value‐based healthcare approach: Health‐related quality of life and psychosocial functioning in women with Turner syndrome

A value-based healthcare approach: Health-related quality of life and psychosocial functioning in women with Turner syndrome

Contact Info

Product

Resources

About