Psychological aspects of the treatment of patients with Disorders of Sex Development

Seminars in Reproductive Medicine

Volumn 30, Issue 5, 2012, Pages 443-452

  Sandberg, Davide ^a   Gardner, Melissa ^a   Cohen Kettenis, Peggyt ^b  

^a UNIVERSITY OF MICHIGAN   (United States)

^b VU UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

disorders of sex development; DSD; gender identity; gender role; psychosexual; psychosocial; sexual orientation

Indexed keywords

ADOLESCENCE; ADOLESCENT; ANDROGEN INSENSITIVITY SYNDROME; ARTICLE; CHILD; CHILDHOOD; FEMALE; GENETIC SCREENING; HUMAN; INFANCY; INFANT; MALE; MEDICAL INFORMATION SYSTEM; PATIENT CARE; PHYSICIAN; PRENATAL PERIOD; PSYCHOLOGICAL ASPECT; PSYCHOSEXUAL DEVELOPMENT; QUALITY OF LIFE; ROKITANSKY SYNDROME; SEX DIFFERENTIATION DISORDER; SEX REASSIGNMENT; SEXUALITY; ULTRASOUND;

ADOLESCENT; ADOLESCENT DEVELOPMENT; ADOLESCENT PSYCHOLOGY; ANIMALS; CHILD; CHILD DEVELOPMENT; CHILD PSYCHOLOGY; DISORDERS OF SEX DEVELOPMENT; FEMALE; FETAL DEVELOPMENT; GENDER IDENTITY; HUMANS; MALE; PATIENT EDUCATION AS TOPIC; PRENATAL DIAGNOSIS; SEX REASSIGNMENT PROCEDURES; SEXUALITY;

EID: 84867001695     PISSN: 15268004     EISSN: 15264564     Source Type: Journal    
DOI: 10.1055/s-0032-1324729     Document Type: Article

References (80)

1. Crouter A. C., Manke B. A., McHale S. M. The family context of gender intensification in early adolescence. Child Dev: 1995; 66 2 317 329
2. Pickles A., Hill J. Developmental psychopathology. In: Cicchetti D., Cohen D., eds. Developmental psychopathology Theory and method. 2nd ed. Hoboken, New Jersey John Wiley & Sons, Inc: 2006; 211 43
3. Lee P. A., Houk C. P., Ahmed S. F., Hughes I. A.; International Consensus Conference on Intersex organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. Consensus statement on management of intersex disorders. Pediatrics: 2006; 118 2 e488 e500
4. Pasterski V., Prentice P., Hughes I. A. Consequences of the Chicago consensus on disorders of sex development (DSD): current practices in Europe. Arch Dis Child: 2010; 95 8 618 623
5. Ahmed S. F., Achermann J. C., Arlt W., et al. UK guidance on the initial evaluation of an infant or an adolescent with a suspected disorder of sex development. Clinical Endocrinology (Oxf.): 2011; 75 12 26
6. Chitayat D., Glanc P. Diagnostic approach in prenatally detected genital abnormalities. Ultrasound Obstet Gynecol: 2010; 35 6 637 646
7. Devaney S. A., Palomaki G. E., Scott J. A., Bianchi D. W. Noninvasive fetal sex determination using cell-free fetal DNA: a systematic review and meta-analysis. JAMA: 2011; 306 6 627 636
8. Marteau T. M., Nippert I., Hall S., et al. DADA Study Group. Decision-making after diagnosis of fetal abnormality. Outcomes of pregnancies diagnosed with Klinefelter syndrome: the possible influence of health professionals. Prenat Diagn: 2002; 22 7 562 566
9. Abramsky L., Hall S., Levitan J., Marteau T. M. What parents are told after prenatal diagnosis of a sex chromosome abnormality: interview and questionnaire study. BMJ: 2001; 322 7284 463 466
10. Girardin C. M., Van Vliet G. Counselling of a couple faced with a prenatal diagnosis of Klinefelter syndrome. Acta Paediatr: 2011; 100 6 917 922
11. Speiser P. W., White P. C. Congenital adrenal hyperplasia. N Engl J Med: 2003; 349 8 776 788
12. Evans M. I., Chrousos G. P., Mann D. W., et al. Pharmacologic suppression of the fetal adrenal gland in utero. Attempted prevention of abnormal external genital masculinization in suspected congenital adrenal hyperplasia. JAMA: 1985; 253 7 1015 1020
13. Dreger A., Feder E., Tamar-Mattis A. Preventing Homosexuality (and Uppity Women) in the Womb? Bioethics Forum. 2010. http://www.thehastingscenter.org/ Bioethicsforum/Post.aspx?id=4754&blogid=140&terms= dreger+and+%23filename+.html. Accessed July 15, 2010
14. Speiser P. W., Azziz R., Baskin L. S., et al. Endocrine Society. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab: 2010; 95 9 4133 4160
15. Matthews S. G. Antenatal glucocorticoids and the developing brain: mechanisms of action. Semin Neonatol: 2001; 6 4 309 317
16. Murphy K. E., Hannah M. E., Willan A. R., et al. MACS Collaborative Group. Multiple courses of antenatal corticosteroids for preterm birth (MACS): a randomised controlled trial. Lancet: 2008; 372 9656 2143 2151
17. Lajic S., Nordenström A., Hirvikoski T. Long-Term Outcome of Prenatal Treatment of Congenital Adrenal Hyperplasia. Endocrine Development. In: Flück C., Miller W., eds. Disorders of the Human Adrenal Cortex. Basel Karger: 2008; 82 98
18. Huang W. L., Beazley L. D., Quinlivan J. A., Evans S. F., Newnham J. P., Dunlop S. A. Effect of corticosteroids on brain growth in fetal sheep. Obstet Gynecol: 1999; 94 2 213 218
19. Hirvikoski T., Lindholm T., Lajic S., Nordenström A. Gender role behaviour in prenatally dexamethasone-treated children at risk for congenital adrenal hyperplasiaa pilot study. Acta Paediatr: 2011; 100 9 e112 e119
20. Hirvikoski T., Nordenström A., Lindholm T., et al. Cognitive functions in children at risk for congenital adrenal hyperplasia treated prenatally with dexamethasone. J Clin Endocrinol Metab: 2007; 92 2 542 548
21. Trautman P. D., Meyer-Bahlburg H. F., Postelnek J., New M. I. Effects of early prenatal dexamethasone on the cognitive and behavioral development of young children: results of a pilot study. Psychoneuroendocrinology: 1995; 20 4 439 449
22. Kazak A. E., Rourke M. T., Crump T. A., Roberts M. C. Families and other systems in pediatric psychology. In: Handbook of pediatric psychology. New York, NY Guildford Press: 2003; 159 75
23. Warne G. L., Raza J. Disorders of sex development (DSDs), their presentation and management in different cultures. Rev Endocr Metab Disord: 2008; 9 3 227 236
24. Dessens A. B., Slijper F. ME, Drop S. LS. Gender dysphoria and gender change in chromosomal females with congenital adrenal hyperplasia. Arch Sex Behav: 2005; 34 4 389 397
25. Mazur T. Gender dysphoria and gender change in androgen insensitivity or micropenis. Arch Sex Behav: 2005; 34 4 411 421
26. Cohen-Kettenis P. T. Gender change in 46,XY persons with 5α-reductase-2 deficiency and 17β-hydroxysteroid dehydrogenase-3 deficiency. Arch Sex Behav: 2005; 34 4 399 410
27. Meyer-Bahlburg H. FL. Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation. Arch Sex Behav: 2005; 34 4 423 438
28. Ruble D., Martin C., Berenbaum S. Gender development. In: Damon W., Lerner R., Eisenberg N., eds. Handbook of child psychology: Social, emotional, and personality 6ed. New York Wiley: 2006; 858 932
29. Berenbaum S. A. Psychological outcome in children with disorders of sex development: implications for treatment and understanding typical development. Annu Rev Sex Res: 2006; 17 1 38
30. Cohen-Kettenis P. Psychological long-term outcome in intersex conditions. Horm Res: 2005; 64 02 27 30
31. Richter-Appelt H., Discher C., Gedrose B. Gender identity and recalled gender related childhood play-behaviour in adult individuals with different forms of intersexuality. Anthropol Anz: 2005; 63 3 241 256
32. de Vries A. L., Doreleijers T. A., Cohen-Kettenis P. T. Disorders of sex development and gender identity outcome in adolescence and adulthood: understanding gender identity development and its clinical implications. Pediatr Endocrinol Rev: 2007; 4 4 343 351
33. Stout S. A., Litvak M., Robbins N. M., Sandberg D. E. Congenital Adrenal Hyperplasia: Classification of studies employing psychological endpoints. International Journal of Pediatric Endocrinology. 2010;;2010(doi:10.1155/2010/ 191520):11 pages. http://www.hindawi.com/journals/ijpe/2010/191520/
34. Meyer-Bahlburg H. F., Dolezal C., Baker S. W., Ehrhardt A. A., New M. I. Gender development in women with congenital adrenal hyperplasia as a function of disorder severity. Arch Sex Behav: 2006; 35 6 667 684
35. Frisén L., Nordenström A., Falhammar H., et al. Gender role behavior, sexuality, and psychosocial adaptation in women with congenital adrenal hyperplasia due to CYP21A2 deficiency. J Clin Endocrinol Metab: 2009; 94 9 3432 3439
36. Nordenström A., Servin A., Bohlin G., Larsson A., Wedell A. Sex-typed toy play behavior correlates with the degree of prenatal androgen exposure assessed by CYP21 genotype in girls with congenital adrenal hyperplasia. J Clin Endocrinol Metab: 2002; 87 11 5119 5124
37. Imperato-McGinley J., Guerrero L., Gautier T., Peterson R. E. Steroid 5alpha-reductase deficiency in man: an inherited form of male pseudohermaphroditism. Science: 1974; 186 4170 1213 1215
38. Richter-Appelt H., Discher C., Gedrose B. Gender identity and recalled gender related childhood play-behaviour in adult individuals with different forms of intersexuality. Anthropol Anz: 2005; 63 241 256
39. Maimoun L., Philibert P., Cammas B., et al. Phenotypical, biological, and molecular heterogeneity of 5α-reductase deficiency: an extensive international experience of 55 patients. J Clin Endocrinol Metab: 2011; 96 2 296 307
40. T'Sjoen G., De Cuypere G., Monstrey S., et al. Male gender identity in complete androgen insensitivity syndrome. Archives of Sexual Behavior: 2011; 40 3 635 638
41. Hines M., Ahmed S. F., Hughes I. A. Psychological outcomes and gender-related development in complete androgen insensitivity syndrome. Arch Sex Behav: 2003; 32 2 93 101
42. Matsubara K., Iwamoto H., Yoshida A., Ogata T. Semen analysis and successful paternity by intracytoplasmic sperm injection in a man with steroid 5α-reductase-2 deficiency. Fertil Steril: 2010; 94 7 2770 10, e7e10
43. Thyen U., Krege S. [DSD/intersexuality network. A project in the BMBF program for rare diseases]. Urologe A: 2007; 46 9 1304 1306
44. Auyeung B., Baron-Cohen S., Ashwin E., et al. Fetal testosterone predicts sexually differentiated childhood behavior in girls and in boys. Psychol Sci: 2009; 20 2 144 148
45. Hines M., Golombok S., Rust J., Johnston K. J., Golding J.; Avon Longitudinal Study of Parents and Children Study Team. Testosterone during pregnancy and gender role behavior of preschool children: a longitudinal, population study. Child Dev: 2002; 73 6 1678 1687
46. Hines M. Prenatal testosterone and gender-related behaviour. Eur J Endocrinol: 2006; 155 01 S115 S121
47. Knickmeyer R. C., Wheelwright S., Taylor K., Raggatt P., Hackett G., Baron-Cohen S. Gender-typed play and amniotic testosterone. Dev Psychol: 2005; 41 3 517 528
48. Cohen-Bendahan C. C., van de Beek C., Berenbaum S. A. Prenatal sex hormone effects on child and adult sex-typed behavior: methods and findings. Neurosci Biobehav Rev: 2005; 29 2 353 384
49. Servin A., Nordenström A., Larsson A., Bohlin G. Prenatal androgens and gender-typed behavior: a study of girls with mild and severe forms of congenital adrenal hyperplasia. Dev Psychol: 2003; 39 3 440 450
50. Liao L. M., Doyle J., Crouch N. S., Creighton S. M. Dilation as treatment for vaginal agenesis and hypoplasia: a pilot exploration of benefits and barriers as perceived by patients. J Obstet Gynaecol: 2006; 26 2 144 148
51. Ismail-Pratt I. S., Bikoo M., Liao L-M, Conway G. S., Creighton S. M. Normalization of the vagina by dilator treatment alone in Complete Androgen Insensitivity Syndrome and Mayer-Rokitansky-Kuster-Hauser Syndrome. Hum Reprod: 2007; 22 7 2020 2024
52. Foundation for Informed Medical Decision Making. 2011; http://www.informedmedicaldecisions.org/. Accessed October 9, 2011.
53. Strauss R. P., Sharp M. C., Lorch S. C., Kachalia B. Physicians and the communication of "bad newso": parent experiences of being informed of their child's cleft lip and/or palate. Pediatrics: 1995; 96 1 Pt 1 82 89
54. Committee on Health Literacy. Institute of Medicine, Nielsen-Bohlman L, Panzer AM, Kindig DA. Health literacy: A prescription to end confusion. Washington, DC The National Academies Press: 2004
55. Money J. Sex errors of the body and related syndromes: a guide to counseling children, adolescents, and their families. Baltimore, MD Paul H. Brookes Publishing Co: 1994
56. Money J. Sex Errors of the Body: Dilemmas, Education, Counseling. Baltimore The Johns Hopkins Press: 1968
57. Meyer-Bahlburg H. FL, Martini L. Gender assignment and psychosocial management. In: Encyclopedia of Endocrine Diseases: Elsevier, Inc; 2004:12534.
58. Carmichael P., Ransley P. Telling children about a physical intersex condition. Dialogues in Pediatric Urology: 2002; 25 7 8
59. Harbeck-Weber C., Fisher J., Dittner C. Promoting coping and enhancing adaptation to illness. In: Roberts M., ed. Handbook of Pediatric Psychology. 3rd ed. New York, NY Guilford Press: 2003; 99 118
60. Brewer S., Gleditsch S. L., Syblik D., Tietjens M. E., Vacik H. W. Pediatric anxiety: child life intervention in day surgery. J Pediatr Nurs: 2006; 21 1 13 22
61. Money J., Lamacz M. Genital examination and exposure experienced as nosocomial sexual abuse in childhood. J Nerv Ment Dis: 1987; 175 12 713 721
62. Chase C. What is the agenda of the intersex patient advocacy movement? Endocrinologist: 2003; 13 240 242
63. Crissman H., Warner L., Gardner M., et al. Children with disorders of sex development: a qualitative study of early parental experience. International Journal of Pediatric Endocrinology 2011;2011:10 http://www.ijpeonline.com/ content/2011/1/10
64. Standards of Care for the Health of Transsexual, Transgender, and Gender Nonconforming People. 2011. (Accessed August 20, 2012, at http://www.wpath.org/ documents/SOC%20V7%2003-17-12.pdf)
65. Meyer-Bahlburg H. FL. Variants of gender differentiation in somatic disorders of sex development: recommendations for Version 7 of the World Professional Association for Transgender Health's Standards of Care. Int J Transgenderism: 2009; 11 226 237
66. Zucker K. J. Measurement of psychosexual differentiation. Arch Sex Behav: 2005; 34 4 375 388
67. Zucker K., Cohen-Kettinis P. Gender identity disorder in children and adolescents. In: Rowland D., Incrocci L., eds. Handbook of Sexual and Gender Identity Disorders. New York, NY Wiley & Sons: 2008; 376 422
68. Menvielle E. J., Tuerk C., Jellinek M. S. A support group for parents of gender-nonconforming boys. J Am Acad Child Adolesc Psychiatry: 2002; 41 8 1010 1013
69. Mazur T., Colsman M., Sandberg D. Intersex: definition, examples, gender stability, and the case against merging with transsexualism. In: Ettner R., Monstrey S., Eyler A. E., eds. Principles of Transgender Medicine and Surgery. Binghamton, NY Haworth Press: 2007; 235 259
70. Lang C., Kuhnle U. Intersexuality and alternative gender categories in non-Western cultures. Horm Res: 2008; 69 4 240 250
71. Ismail I., Creighton S. Surgery for intersex. Rev Gynaecol Pract: 2005; 5 57 64
72. Schönbucher V., Schweizer K., Rustige L., Schützmann K., Brunner F., Richter-Appelt H. Sexual quality of life of individuals with 46,XY disorders of sex development. Journal of Sexual Medicine Epub: 2010; Jan 6
73. Kuhnle U., Bullinger M., Schwarz H. P. The quality of life in adult female patients with congenital adrenal hyperplasia: a comprehensive study of the impact of genital malformations and chronic disease on female patients life. Eur J Pediatr: 1995; 154 9 708 716
74. Slijper F., van der Kamp H., Brandenbrug H., de Muinck Keizer-Schrama S., Drop S., Molenaar J. Evaluation of psychosexual development of young women with congenital adrenal hyperplasia: A pilot study. J Sex Educ Ther: 1992; 18 200 207
75. Zucker K. J., Bradley S. J., Oliver G., Blake J., Fleming S., Hood J. Psychosexual development of women with congenital adrenal hyperplasia. Horm Behav: 1996; 30 4 300 318
76. Hines M., Brook C., Conway G. S. Androgen and psychosexual development: core gender identity, sexual orientation and recalled childhood gender role behavior in women and men with congenital adrenal hyperplasia (CAH). J Sex Res: 2004; 41 1 75 81
77. Reader T. W., Flin R., Mearns K., Cuthbertson B. H. Developing a team performance framework for the intensive care unit. Crit Care Med: 2009; 37 5 1787 1793
78. Salas E., DiazGranados D., Weaver S. J., King H. Does team training work? Principles for health care. Acad Emerg Med: 2008; 15 11 1002 1009
79. Lemieux-Charles L., McGuire W. L. What do we know about health care team effectiveness? A review of the literature. Med Care Res Rev: 2006; 63 3 263 300
80. Bell S. T. Deep-level composition variables as predictors of team performance: a meta-analysis. J Appl Psychol: 2007; 92 3 595 615