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马凡综合征患者身高控制治疗的适宜年龄。

Appropriate Age for Height Control Treatment in Patients With Marfan Syndrome.

机构信息

Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

出版信息

Front Endocrinol (Lausanne). 2021 Oct 21;12:708931. doi: 10.3389/fendo.2021.708931. eCollection 2021.

Abstract

OBJECTIVE

This study aimed to determine the most appropriate age for height control treatment in patients with Marfan syndrome (MFS).

MATERIALS AND METHODS

This retrospective study included patients with MFS who underwent height control treatment with estradiol valerate. The estrogen dose was increased according to the height change. The cut-off age for the maximum difference between the expected height and actual final height was evaluated.

RESULTS

Seventeen patients were included in this study. The difference between the height predicted by the growth curve and the final height (gcHtD) and that predicted by the bone age and the final height (baHtD) was the largest in the 10.5 years age group (p=0.0045 and p=0.0237, respectively). The gcHtD was 10.6 (10.2, 13.5) cm for patients aged ≤10.5 years, whereas it was 0.6 (-3.65, 5.85) cm for patients aged >10.5 years. The baHtD was 10.1 (7.31, 11.42) cm for patients aged ≤10.5 years, while it was 3.83 (0.84, 6.4) cm for patients aged >10.5 years. When height change was observed for a minimum of 6 months after completion of estrogen treatment, the average growth was 0.6 (0.2, 2.1) cm.

CONCLUSION

Initiating height control treatment before the age of 10.5 years is effective in female patients with MFS.

摘要

目的

本研究旨在确定马凡综合征(MFS)患者进行身高控制治疗的最佳年龄。

材料与方法

本回顾性研究纳入了接受戊酸雌二醇进行身高控制治疗的 MFS 患者。根据身高变化增加雌激素剂量。评估预测身高与最终身高之间最大差值的截止年龄。

结果

本研究纳入了 17 例患者。生长曲线预测身高与最终身高的差值(gcHtD)和骨龄与最终身高的差值(baHtD)在 10.5 岁年龄组最大(分别为 p=0.0045 和 p=0.0237)。≤10.5 岁患者的 gcHtD 为 10.6(10.2,13.5)cm,而>10.5 岁患者的 gcHtD 为 0.6(-3.65,5.85)cm。≤10.5 岁患者的 baHtD 为 10.1(7.31,11.42)cm,而>10.5 岁患者的 baHtD 为 3.83(0.84,6.4)cm。完成雌激素治疗后至少观察 6 个月的身高变化时,平均生长速度为 0.6(0.2,2.1)cm。

结论

在 10.5 岁之前开始身高控制治疗对女性 MFS 患者有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/934a/8567100/a610b502932e/fendo-12-708931-g001.jpg

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