Department of Endocrinology, Shenzhen Children's Hospital, Shenzhen, China.
Department of Radiology, Shenzhen Children's Hospital, Shenzhen, China.
BMC Pediatr. 2024 May 8;24(1):317. doi: 10.1186/s12887-024-04783-2.
Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS.
This study included TS patients admitted to Shenzhen Children's Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada's method).
A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score.
The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.
特纳综合征（TS）患者发生主动脉扩张（AD）的风险增加，但与成人相比，儿童 AD 的诊断更为复杂。本研究旨在探讨 AD 的各种评估指标在患有 TS 的中国儿童和青少年中的应用。
本研究纳入了 2017 年至 2022 年在深圳市儿童医院就诊的 TS 患者。心血管病变由经验丰富的放射科医生诊断。无结构性心脏病的患者分为不同的体表面积组，然后计算升主动脉的中国 TS 人群 Z 评分（CHTSZ-score），并与主动脉大小指数（ASI）、升主动脉与降主动脉直径比（A/D 比）、TSZ 评分（Quezada 法）等其他指标进行比较。
共纳入 115 例 TS 患者，平均年龄为 10.0±3.7 岁。三种最严重心血管并发症的发生率分别为 9.6%（AD）、10.4%（主动脉缩窄，CoA）和 7.0%（二叶式主动脉瓣，BAV）。年龄≥10 岁的 TS 患者发生 AD 的比例高于年龄<10 岁的患者（16.6%比 1.8%，P=0.009），同时患有 CoA 或 BAV 且存在 AD 的患者比例高于无这些情况的患者（31.6%比 5.2%，P<0.001）。11 例 AD 患者的 ASI、A/D 比、TSZ 评分和 CHTSZ 评分分别为 2.27±0.40 cm/m、1.90±0.37、1.28±1.08 和 3.07±2.20。AD 患者中，仅 3 例 TSZ 评分≥2，2 例 TSZ 评分≥1。然而，根据 CHTSZ 评分评估，有 6 例得分≥2，5 例得分≥1。相比之下，TSZ 评分通常低估了中国 TS 儿童的主动脉 Z 评分，而 CHTSZ 评分则不然。
ASI 和 A/D 比在 TS 儿童中的适用性值得怀疑，种族差异可能会影响中国人群中 TSZ 评分的评估。因此，建立专门针对中国儿童和青少年的 CHTSZ 评分至关重要。
