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Long term psychological outcome for women with congenital adrenal hyperplasia: cross sectional survey

BMJ 2005; 330 doi: https://doi.org/10.1136/bmj.38334.427361.D3 (Published 10 February 2005) Cite this as: BMJ 2005;330:340
  1. John F Morgan, senior lecturer (jmorgan{at}sghms.ac.uk)1,
  2. Helen Murphy, clinical lecturer1,
  3. J Hubert Lacey, professor1,
  4. Gerard Conway, senior lecturer2
  1. 1 Department of Mental Health, St George's Hospital Medical School, University of London, London SW17 0RE
  2. 2 Department of Endocrinology, The Middlesex Hospital, London W1T 3AA
  1. Correspondence to: J F Morgan
  • Accepted 1 December 2004

Introduction

Management of conditions associated with intersex, such as congenital adrenal hyperplasia, is controversial. A recent editorial in the BMJ called for long term studies of outcome.1 Females (genotype XX) with congenital adrenal hyperplasia are born with ambiguous genitalia, have feminising genitoplasty soon after birth, and often have repeated genital surgery and genital examinations in adolescence. This has raised fears that the condition and its management adversely affect psychosexual development or psychosocial adjustment.2 The “serious deficiency of any evidence base” on long term outcome prompted us to investigate the hypothesis that psychiatric morbidity is increased and that social and psychological adjustment is impaired in women with congenital adrenal hyperplasia.1

Participants, methods, and results

We did a cross sectional study of a clinic based sample of women with congenital adrenal hyperplasia. All were genetically female, some had feminising genitoplasty soon after birth, and all were raised as female. We recruited 18 sequential female patients (aged 18-36) with congenital adrenal hyperplasia from an endocrinology clinic at the Middlesex Hospital, London, in 1997-8. We assessed participants with validated structured interviews (the Eating Disorder Examination, 12th edition, and the Structured Clinical Interview for DMS-IV-R (Diagnostic and Statistical Manual of Mental Disorders, fourth edition, revised)) and questionnaires (the General Health Questionnaire 30, the modified Social Adjustment Scale,3 and Rosenberg's Self Esteem Inventory). All patients approached agreed to participate (table).4

Psychiatric morbidity as defined in DSM-IV and psychosocial function in 18 women with congenital adrenal hyperplasia compared with normative data

View this table:

Two participants met diagnostic criteria for past mood disorder, five for past anxiety disorder, and one for past alcohol misuse. General health questionnaire scores were consistent with these findings, with a sensitivity of 100% and specificity of 88% for detection of depression. Social adjustment scores fell within the normal range for all role areas examined (table), and self esteem scores (mean 4.83, standard deviation 4.57) were above average (possible range −15 to +15).

No women met DSM-IV criteria for an eating disorder. Reflecting eating behaviours and attitudes, Eating Disorder Examination scores were comparable to normative data on subscales of restraint (0.70, 0.87 v 0.79, 0.97), shape concern (1.17, 0.96 v 1.14, 0.98), and weight concern (1.02, 1.28 v 1.00, 0.87), but eating concern was raised (0.72, 0.60 v 0.20, 0.51).

Only four participants rated themselves as sexually active, of whom two rated nil sexual enjoyment. Two participants categorised themselves as homosexual and two as bisexual, and sexual orientation was not associated with differences in any measure of psychosocial adjustment.

Comment

Women with congenital adrenal hyperplasia are psychologically well adjusted and do not show substantially increased psychiatric disorder or deficits of social adjustment compared with population data. Low levels of sexual activity and higher levels of non-heterosexual orientation are indicated, although legitimate normative data is lacking. Levels of disordered eating are not significantly different from population norms, despite increased exposure to sociocultural risk factors for eating disorders, though this may reflect a type II error.

Limitations of the study include the small sample size and the lack of a control group, which would have the same stresses as the congenital adrenal hyperplasia group but lack the stigmata of the illness. As such a control group does not exist, a descriptive comparison was made with the general population.

Although a previous study of childhood psychiatric morbidity in intersex people found increased prevalence of mental disorder,5 and accepting that some individuals with congenital adrenal hyperplasia may struggle to adapt, we conclude that the women with congenital adrenal hyperplasia in our small study are psychologically robust. Future studies must consider factors contributing to good psychological outcome, including timing of surgery and communication with parents and patients.

What is already known on this topic

Intersex children may have increased prevalence of psychiatric morbidity; research on adult psychological outcomes is limited despite fears that the condition may adversely affect psychosocial adjustment

What this study adds

Most women with congenital adrenal hyperplasia have good long term psychological outcome, with no dramatic increase in psychological morbidity, good social adjustment, and no deficit in self esteem

This article was posted on bmj.com on 26 January 2005:http://bmj.com/cgi/doi/10.1136/bmj.38334.427361.D3

Footnotes

  • Contributors JFM, JHL, and GC originated and designed the study. JFM collected the data. JFM and HM analysed and interpreted the data and drafted the manuscript. GC, JHL, and JFM critically revised the manuscript. JHL supervised the study. JFM is guarantor.

    Funding: No additional funding.

  • Funding No additional funding.

  • Competing interests None declared

  • Ethical approval Research ethics committee for the Central Middlesex Hospital.

References

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