Abstract
The aim of this study is to determine the behavioural problems of children with 46XY disorders of sex development (DSD) with genital ambiguity and to identify the risk factors that may influence behaviour. The 27 participants (aged 6–18 years) consisted of 21 patients raised as boys and 6 patients raised as girls. Control data were obtained from a representative sibling of each patient who was matched for age and gender. The study tool used was the Child Behaviour Checklist (CBCL), which is a parent-administered questionnaire. The analysis of the behavioural scores revealed that the patient group had poorer scores in the total, externalizing, and internalizing realms. This group also had poorer scores in the anxious-depressed, social, and rule-breaking realms as compared to the control group. In addition, the XY-F group had higher scores (more pathological) than the XY-M group, although the difference in the scores was not statistically significant. A comparison of the prevalence of patients with scores in the clinical range with that of the control group was not statistically significant. These findings support the current recommendations that psychological counselling should be an integral part of the professional support offered to patients with DSD.
Highlights
Disorders of sex development (DSD) are defined as congenital conditions in which the development of chromosomal, gonadal, and anatomic sex is atypical [1]
Psychological distress, self-harming behaviour, and suicidal tendencies are prevalent in some samples of women with complete androgen insensitivity syndrome (CAIS) recruited via physicians or support groups [8]
Similar patterns of psychological distress have been identified from responses to the Brief Symptom Inventory (BSI), and suicidal ideation has been observed in 46XY women with 5-alpha reductase deficiency and 17-beta hydroxyprogesterone deficiency [4, 8]
Summary
Disorders of sex development (DSD) are defined as congenital conditions in which the development of chromosomal, gonadal, and anatomic sex is atypical [1]. It has been estimated that the incidence of patients presenting with ambiguous genitalia at birth is approximately 1 in 4500– 5500 [2, 3] These individuals are often exposed to numerous diagnostic procedures, including surgery, hormonal treatments, and long-term follow-up, which, in addition to the disorder itself, may cause serious distress [4]. Outcome studies on the psychological well-being of individuals with DSD have tended to focus on the group with congenital adrenal hyperplasia [5,6,7]. Among those with 46XY DSD, the largest group studied has partial androgen insensitivity syndrome (PAIS) [8]. Similar patterns of psychological distress have been identified from responses to the BSI, and suicidal ideation has been observed in 46XY women with 5-alpha reductase deficiency and 17-beta hydroxyprogesterone deficiency [4, 8]
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