Purpose

Patients with congenital adrenal hyperplasia (CAH) have an increased risk of psychological/psychiatric symptoms and disorders. This study aimed to assess the behavior of girls with CAH and its independent risk variables.

Methods

This cross-sectional study included 55 girls with CAH due to 21-hydroxylase deficiency (mean age 12.64 ± 1.52 years; salt-wasting (SW) form = 20, simple virilizing (SV) form = 35). Psychiatric interviewing and the Strength and Difficulties Questionnaire (SDQ) (parent-reporting questionnaires) were used to assess behavior.

Results

Compared to controls (n = 60), patients had a high total SDQ score (P = 0.001) and emotional, conduct, and hyperactivity-inattention symptoms, peer relationship problems (P = 0.001 for all), and prosocial behavior (p = 0.01) subscale scores, indicating externalizing and internalizing behavioral problems. Severe emotional symptoms and poor disease control were found with SW compared to the SV form. Multiple linear regression showed that bone age (BA) (β = 0.331, t = 3.608; P = 0.001) and 17-OHP (β = 0.408, t = 4.220; P = 0.001), testosterone (β = 0.348, t = 3.220; P = 0.001), and androstenedione (β = 0.238, t = 2.487; P = 0.015) levels were independently associated with behavioral problems.

Conclusion

Females with CAH had frequent and severe behavioral symptoms. Excess androgenization, which was in part due to suboptimal steroid therapy, was the cause of the behavioral problems. Therefore, early optimization of CAH management should be stressed to prevent psychological consequences.

中文翻译：

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先天性肾上腺增生女性的行为评估。

目的

先天性肾上腺皮质增生症 (CAH) 患者出现心理/精神症状和障碍的风险增加。本研究旨在评估 CAH 女孩的行为及其独立风险变量。

方法

这项横断面研究包括 55 名因 21-羟化酶缺乏而患有 CAH 的女孩（平均年龄 12.64 ± 1.52 岁；盐浪费 (SW) 型 = 20，简单男性化 (SV) 型 = 35）。精神病学访谈和力量和困难问卷（SDQ）（家长报告问卷）用于评估行为。

结果

与对照组 ( n  = 60) 相比，患者的 SDQ 总分较高 ( P  = 0.001) 以及情绪、行为和多动-注意力不集中症状、同伴关系问题 ( P  = 0.001 ) 和亲社会行为 ( p  = 0.01 ) 分量表分数，表明行为问题的外化和内化。与 SV 形式相比，SW 发现严重的情绪症状和疾病控制不佳。多元线性回归显示骨龄 (BA) (β = 0.331, t  = 3.608; P  = 0.001) 和 17-OHP (β = 0.408, t  = 4.220; P  = 0.001), 睾酮 (β = 0.348, t  = 3.222) ; P = 0.001) 和雄烯二酮 (β = 0.238, t  = 2.487; P  = 0.015) 水平与行为问题独立相关。

结论

患有 CAH 的女性有频繁和严重的行为症状。过度雄激素化，部分是由于类固醇治疗不理想，是行为问题的原因。因此，应强调早期优化 CAH 管理，以防止心理后果。