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21-羟化酶缺陷导致先天性肾上腺皮质增生症患者青年期心血管功能障碍风险。

Cardiovascular dysfunction risk in young adults with congenital adrenal hyperplasia caused by 21-hydroxylase enzyme deficiency.

机构信息

Laboratory of Growth and Development (LabCreD), Center for Investigation in Pediatrics (CIPED), School of Medical Sciences (FCM), State University of Campinas (UNICAMP), Campinas, Brazil.

Department of Pediatrics, FCM, UNICAMP, Campinas, Brazil.

出版信息

Int J Clin Pract. 2021 Jul;75(7):e14233. doi: 10.1111/ijcp.14233. Epub 2021 May 1.

DOI:10.1111/ijcp.14233
PMID:33884716
Abstract

BACKGROUND

The association of congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase enzyme (21OHase) deficiency, duration of treatment and dosage with cardiovascular dysfunction in young adults remains unclear. We aimed to evaluate myocardial function, vascular structures and epicardial fat thickness in young adults with CAH as a result of 21OHase deficiency. Correlations between the duration and dose of glucocorticoid therapy and cardiovascular parameters were analysed.

METHODS

This case-control study of young adults (18-31 years old) included 20 patients (5 men and 15 women) and 16 control subjects (8 men and 8 women). Echocardiographic analysis was performed using high-resolution ultrasound.

RESULTS

No ultrasonographic changes in any indices of myocardial function, vascular structures and epicardial fat thickness were found in patients, except for an impaired left ventricular end-diastolic diameter in female patients (28.1 ± 1.6 vs 26.0 ± 2.4 mm/m , P = .021), compared with those in individuals in the control group. Nevertheless, the individual patient values were within the normal range. Multiple linear regression analysis in female patients demonstrated that an elevated daily dose of glucocorticoids correlated with increased indices of left ventricular posterior wall thickness (Partial r = 0.68, P = .007), left ventricular end-diastolic diameter (Partial r = 0.62, P = .017), aortic diameter (Partial r = 0.60, P = .022) and left carotid artery intima-media thickness (Partial r = 0.61, P = .021), independently of treatment duration.

CONCLUSION

No signs of cardiovascular dysfunction were observed in any patient. The daily dose of glucocorticoids may play a role in the mechanisms of some markers of cardiac hypertrophy, left ventricular and aortic dilation and subclinical atherosclerosis.

摘要

背景

21-羟化酶(21OHase)缺乏导致的先天性肾上腺皮质增生症(CAH)与心血管功能障碍的关联,包括治疗持续时间和剂量,在年轻成年人中仍不清楚。我们旨在评估由于 21OHase 缺乏而导致 CAH 的年轻成年人的心肌功能、血管结构和心外膜脂肪厚度。分析糖皮质激素治疗的持续时间和剂量与心血管参数之间的相关性。

方法

这项年轻成年人(18-31 岁)的病例对照研究包括 20 名患者(5 名男性和 15 名女性)和 16 名对照者(8 名男性和 8 名女性)。使用高分辨率超声进行超声心动图分析。

结果

除了女性患者的左心室舒张末期直径受损(28.1±1.6 比 26.0±2.4mm/m,P=0.021)外,患者的心肌功能、血管结构和心外膜脂肪厚度的任何指标均无超声改变,与对照组个体相比。然而,个体患者的值仍在正常范围内。在女性患者中,多元线性回归分析显示,糖皮质激素的日剂量升高与左心室后壁厚度(偏相关系数 r=0.68,P=0.007)、左心室舒张末期直径(偏相关系数 r=0.62,P=0.017)、主动脉直径(偏相关系数 r=0.60,P=0.022)和左颈动脉内中膜厚度(偏相关系数 r=0.61,P=0.021)的增加独立相关,而与治疗持续时间无关。

结论

在任何患者中均未观察到心血管功能障碍的迹象。糖皮质激素的日剂量可能在心脏肥大、左心室和主动脉扩张以及亚临床动脉粥样硬化的一些标志物的机制中发挥作用。

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