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先天性肾上腺皮质增生女性的行为评估。

Behavioral assessment of females with congenital adrenal hyperplasia.

机构信息

Department of Neurology and Psychiatry, Assiut University Hospital, P. O. Box 71516, Assiut, Egypt.

Department of Internal Medicine, Assiut University Hospital, Assiut, Egypt.

出版信息

Hormones (Athens). 2021 Mar;20(1):131-141. doi: 10.1007/s42000-020-00232-8. Epub 2020 Aug 1.

Abstract

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.

摘要

目的

先天性肾上腺皮质增生症(CAH)患者存在心理/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的风险变量。

方法

这是一项横断面研究,纳入了 55 例由于 21-羟化酶缺乏导致的 CAH 女孩(平均年龄 12.64±1.52 岁;失盐型[SW]20 例,单纯男性化型[SV]35 例)。采用精神科访谈和长处和困难问卷(SDQ)(家长报告问卷)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),且存在情绪、品行和多动/注意力问题、同伴关系问题(所有 P 值均<0.001)和亲社会行为(P=0.01)亚量表评分高,提示存在外化和内化行为问题。与 SV 型相比,SW 型患者存在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-羟孕酮(17-OHP)(β=0.408,t=4.220;P=0.001)、睾酮(β=0.348,t=3.220;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

CAH 女性存在频繁和严重的行为症状。部分由于类固醇治疗不理想导致的雄激素过多是行为问题的原因。因此,应强调早期优化 CAH 管理以预防心理后果。

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