Mathara Diddhenipothage Shani A D, Beck Katharina J, Amiyangoda Gayana, Bryce Jillian, Cima Luminita, De Groote Katya, Deyanova Yana, Globa Evgenia, Herrmann Gloria, Juul Anders, Kjaer Anna Sophie L, Pedersen Anette Tønnes, Poyrazoglu Sukran, Probst-Scheidegger Ursina, Sas Theo C J, Fica Simona, Seneviratne Sumudu Nimali, Witczak Justyna Karolina, Orchard Elizabeth, Tomlinson Jeremy W, Ahmed S Faisal, Turner Helen E
Endocr Connect. 2025 Jan 18;14(2). doi: 10.1530/EC-24-0477. Print 2025 Feb 1.
Cardiovascular disease is the most common cause of death in Turner syndrome (TS) for which arterial hypertension has a direct influence and is a key modifiable risk factor.
To investigate the prevalence and patterns of hypertension diagnosis and management in adult patients with TS who are registered in a large international multicentre database (TS-HTN study).
Retrospective multicentre observational study of patients aged ≥18 years included in the I-TS (International-TS) registry (2020-2022), using registry and participating centre-collected data.
Twelve international centres participated, including 182 patients with a median age of 28 years (IQR 23-37.2). Arterial hypertension was recorded in 13.2% (n = 24). The median age at hypertension diagnosis was 27 years (range 10-56), with 92% aged less than 50 years at diagnosis. The majority (75%) were classified as primary hypertension (n = 18). In binomial regression analysis, higher body mass index was the only parameter significantly associated with the occurrence of hypertension (B = 1.487, P = 0.004). Among patients with aortic disease (n = 9), 50% had systolic BP ≥ 130 mmHg and 66.6% had diastolic BP ≥ 80 mmHg during the last clinic review. Angiotensin-converting enzyme inhibitors were the most common (n = 16) medication prescribed, followed by angiotensin receptor blockers (n = 6), beta-blockers (n = 6) and calcium channel blockers (n = 6).
Arterial hypertension is common in TS and occurs at a young age. Overweight/obesity was a notable risk factor for hypertension. The frequency of suboptimal BP control among high-risk patients highlights the importance of increased awareness and TS-specific consensus guidance on management.
心血管疾病是特纳综合征（TS）最常见的死亡原因，动脉高血压对其有直接影响，且是一个关键的可改变风险因素。
调查在一个大型国际多中心数据库中登记的成年TS患者高血压诊断和管理的患病率及模式（TS-HTN研究）。
对纳入I-TS（国际TS）登记处（2020 - 2022年）的年龄≥18岁的患者进行回顾性多中心观察性研究，使用登记处和参与中心收集的数据。
12个国际中心参与，包括182例患者，中位年龄为28岁（四分位间距23 - 37.2）。记录到动脉高血压的患者占13.2%（n = 24）。高血压诊断时的中位年龄为27岁（范围10 - 56岁），92%的患者在诊断时年龄小于50岁。大多数（75%）被归类为原发性高血压（n = 18）。在二项回归分析中，较高的体重指数是与高血压发生显著相关的唯一参数（B = 1.487，P = 0.004）。在患有主动脉疾病的患者（n = 9）中，在最后一次临床复查时，50%的患者收缩压≥130 mmHg，66.6%的患者舒张压≥80 mmHg。血管紧张素转换酶抑制剂是最常用的处方药物（n = 16），其次是血管紧张素受体阻滞剂（n = 6）、β受体阻滞剂（n = 6）和钙通道阻滞剂（n = 6）。
动脉高血压在TS中很常见，且发病年龄较轻。超重/肥胖是高血压的一个显著风险因素。高危患者血压控制不佳的频率凸显了提高认识以及制定TS特异性管理共识指南的重要性。
