ECE2023 Eposter Presentations Late Breaking (91 abstracts)
Prevalence, patterns, and characteristics of hypertension diagnosis and management in patients with Turner syndrome; Descriptive analysis of real-world data from the International-Turner Syndrome registry
Shani Apsara Dilrukshi Mathara Diddhenipothage 1 , Katharina Beck 1 , C G K Amiyangoda 1 , Jillian Bryce 2 , Luminita Cima 3 , Katya De Groote 4 , Yana Deyanova 5 , Eugenia Globa 6 , Gloria Herrmann 7 , Anders Juul 8 , Anna Sophie. L Kjaer 9 , Anette Tønnes Pedersen Pedersen 10 , Sukran Poyrazoglu 11 , Ursina Probst-Scheidegger 12 , Theo C.J Sas 13 , Sumudu Nimali Seneviratne 14 , Justyna Witczak 15 , Elizabeth Orchard 16 , Jeremy Tomlinson 17 , Faisal Ahmed 2 & Helen Turner 1
1Oxford University Hospitals NHS Trust, Oxford Centre for Diabetes, Endocrinology and Metabolism, Oxford, United Kingdom, 2School of Medicine, University of Glasgow, Royal Hospital For Children, Developmental Endocrinology Research Group, Glasgow, United Kingdom, 3Elias University Hospital, Carol Davila University of Medicine and Pharmacy, Endocrinology Department, Bucharest, Romania, 4Ghent University Hospital, Department of Pediatrics, Paediatric Cardiology, Ghent, Belgium, 5Medical University of Varna, Department of Pediatrics, Varna, Bulgaria, 6Ukrainian Research Center of Endocrine Surgery, Endocrine Organs and Tissue Transplantation, Department of Pediatric Endocrinology, Kyiv, Ukraine, 7University Hospital Ulm, Department of Pediatrics and Adolescent Medicine, Division of Pediatric Endocrinology and Diabetes, Ulm, Germany, 8Copenhagen University Hospital—Rigshospitalet, Department of Growth and Reproduction and EDMaRC, Copenhagen, Denmark, 9Copenhagen University Hospital—Rigshospitalet, Department of Growth and Reproduction and EDMaRC, Copenhagen, Denmark, 10Copenhagen University Hospital - Rigshospitalet, University of Copenhagen, Department of Gynaecology, Copenhagen, Denmark, 11Istanbul Faculty of Medicine, Istanbul University, Paediatric Endocrinology Unit, Istanbul, Turkey, 12Kantonsspital Winterthur, Paediatric Department, Winterthur, Switzerland, 13Erasmus University Medical Center, Sophia Children’s Hospital, Department of Pediatrics, Division of Pediatric Endocrinology, Rotterdam, Netherlands, 14Faculty of Medicine, University of Colombo, Department of Paediatrics, Colombo, Sri Lanka, 15University Hospital of Wales, Centre for Endocrine and Diabetes Sciences, Cardiff, United Kingdom, 16Oxford University Hospitals NHS Foundation Trust, Department of Cardiology, Oxford, United Kingdom, 17University of Oxford, Oxford Centre for Diabetes, Endocrinology, and Metabolism, NIHR Oxford Biomedical Research Centre, Oxford, United Kingdom
Introduction: Hypertension is common in patients with Turner Syndrome (TS), and they appear to have an increased predisposition with various proposed disease related factors; sex hormone imbalance, aortopathy, increased activation of the renin-angiotensin-aldosterone system, insulin resistance, growth hormone deficiency/resistance, adverse imprinting during fetal life and renal malformations. Despite high susceptibility and unique challenges in managing hypertension in this group, there exists no consensus on screening, classification, diagnosis, and management of hypertension, predominantly due to scarcity of evidence. Undiagnosed and/or poorly controlled blood pressure (BP) can be detrimental/lethal in patients with aortopathy. In 2020, the International-TS(I-TS) registry was established within the International-Disorders of sexual differentiation (I-DSD) platform to fill gaps in the knowledge of TS and has a network reaching 260 centres in 63 countries.
Methods: All the centres registered under I-TS registry with patients≥18 years were invited to participate in this retrospective multi-centre observational study. Pseudoanonymised data relevant to the study[karyotype, hormone-replacement(HRT), hypertension diagnosis, risk factors, management, morbidity] were either directly extracted from the registry or clinicians of relevent centres were requested to complete a formatted spreadsheet using routine clinic records.
Results: Complete data were available for 12/15 accepted-to-participate centres, from 11 countries, January 2022-2023. A total 181 patients [median-age 28.5 (range:18-71)years, median-age at TS diagnois 11(range:0-34)years, and median BMI 25.2(range:18-44)Kgm2] were included. Monosomy X(45,X) was the commonest (42%) karyotype and 64% of all patients were receiving HRT. Twenty-four(13.3%) patients had hypertension [Characteristics at diagnosis; median age 27(range:10-56) years, median systolic-BP 150(range:125-270)mmHg and median diastolic-BP 90(60-136)mmHg)]. The majority(18/24) had primary hypertension, and 4/6 with secondary hypertension had coarctation of the aorta. The majority of those with hypertension (62%) were 45,X [X2(1,n=181)=4.779, P0.029], and 91.6% were on HRT[median daily oetrogen dose:1(range:0-2) mg]. Many patients had coexisiting vascular risk factors; type 2 diabetes:16.7%, dyslipidaemia:33.3%, family-history:8.3%. The incidence of comorbidities potentially exacerbated by hypertension were; aortic disease:37.5%, congenital cardiac anomalies:12.5%, cardiac surgery:12.5%, renal anomalies:20.8%. The majority (21/24) received angiotensin-converting-enzyme inhibitors(ACEi)/ angiotensin-receptor blocker(ARB), and 6/24 were on beta-blockers(BB).
Conclusions: Hypertension is common in TS, with a high prevalence of associated risk factors and co-morbidities, highlighting the importance of development of specific guidance. In most cases, hypertension was diagnosed as primary hypertension, despite a young age of onset. ACEi/ARB and BB were the most commonly used medications. Furthermore, this study demonatrates, the potential advantage of inclusion of a large number of individuals, which is international in applicability, with capture of local site specific details.
Article tools
My recent searches
My recently viewed abstracts
Authors
Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
© Bioscientifica 2024 |
Privacy policy |
Cookie settings
BiosciAbstracts
Bioscientifica Abstracts is the gateway to a series of products that provide a permanent, citable record of abstracts for biomedical and life science conferences.
Find out more