Self-Perception of Reproductive Health, Mental Health, and Quality of Life in Females With Classical Congenital Adrenal Hyperplasia: A Systematic Review.

BACKGROUND: Classical Congenital Adrenal Hyperplasia (C-CAH) is a congenital disorder characterized by a loss of 21-hydroxylase enzymes. Consequently, androgen levels increase, resulting in varying degrees of psychological and physiological masculinization. This systematic review aims to examine existing research on females with C-CAH and identify challenges related to reproductive self-perception, mental health, and overall quality of life. METHODOLOGY: This systematic review was conducted based on the PRISMA 2020 guidelines, studies were retrieved from PubMed, EBSCO, Science Direct, and ProQuest, and screened according to predefined eligibility criteria. Quality assessments of included studies were done using the Newcastle-Ottawa Scale (NOS) for case-control studies, adapted NOS for cross-sectional studies, and Risk of Bias in Non-randomized Studies of Intervention (ROBINS-I). RESULTS: Eleven studies determined to have low risk of bias involving 523 participants were included. All 11 studies addressed self-perception of reproductive health, evaluating gender and sexual-related issues, with outcomes such as atypical gender identity and sexual orientation, gender dysphoria, and sexual satisfaction impairment. Adverse perceptions and experiences related to virilized genitalia were commonly reported. Four studies examined mental health issues, comprising anxiety, social phobia, ADHD, sexual distress, and signs of depression. Finally, females with C-CAH have reduced QoL compared to healthy females, mainly due to impacted general health and pain. CONCLUSION: Females with C-CAH demonstrated impaired reproductive self-perception, including sexual satisfaction and gender-related difficulties, alongside psychological concerns including anxiety, social phobia, and maladaptive coping behaviors, with reduced quality of life related to general health and pain.