Behavioral assessment of females with congenital adrenal hyperplasia

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.

Keywords: Behavioral symptoms; Congenital adrenal hyperplasia; Girls; Salt-wasting; Simple virilizing.

MeSH terms

  • Adolescent
  • Adrenal Hyperplasia, Congenital / diagnosis
  • Adrenal Hyperplasia, Congenital / psychology*
  • Child
  • Cross-Sectional Studies
  • Female
  • Humans
  • Social Behavior

Supplementary concepts

  • Congenital adrenal hyperplasia due to 21 hydroxylase deficiency