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DOI: 10.1055/s-2002-35375
Psychological Consequences
Publikationsverlauf
Publikationsdatum:
12. November 2002 (online)
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ABSTRACT
Sex assignment of children born with ambiguous genitalia is a difficult and responsible decision based on limited empirical evidence regarding future development of gender identity/role, sexual orientation, and sexual functioning. Sex of assignment and rearing has appeared to be a prognosticator of future gender identity, usually better than the other criteria of sex (chromosomes, gonadal tissue, prenatal and postnatal hormonal profiles). The decision to assign a sex is guided by the prognosis of the ``optimal'' sex for the newborn, of which the elements are an overall sex-appropriate appearance with a stable gender identity; good sexual function, preferably combined with reproductive function if attainable; minimal medical procedures; and a reasonably happy life given the limitations. The limited follow-up studies indicate that this is a reasonable policy, although a limited number of subjects will experience gender dysphoria later in life and will cross over to the other sex. This is more often the case in subjects assigned to the female sex with considerable prenatal or postnatal androgen exposure. Children in this predicament must receive guidance well into adulthood. It may be difficult to engage in sexual relations when the anatomy of the genitalia (often operated upon) is not fully normal.
KEYWORDS
Sexual differentiation disorders - sex assignment - gender identity - sexual orientation - androgens
REFERENCES
- 1 Money J, Ehrhardt A A. Man and Woman, Boy and Girl: The Differentiation and Dimorphism of Gender Identity from Conception to Maturity. Baltimore: Johns Hopkins University Press 1972
- 2 Money J. Love & Lovesickness. Baltimore: The Johns Hopkins University Press 1980
- 3 Money J. Biographies of Gender and Hermaphroditism in Paired Comparisons. Amsterdam: Elsevier Science 1991
- 4 Money J, Hampson J G, Hampson J L. An examination of some basic sexual concepts: the evidence of human hermaphroditism. Bull Johns Hopkins Hosp . 1955; 97 301-319
- 5 Klebs E. Handbuch der pathologischen Anatomie. Berlin: August Hirschwald, Germany; 1876. I. Band, zweite Abteilung:718
- 6 Hines M. Prenatal gonadal hormones and sex differences in human behaviour. Psychol Bull . 1982; 92 56-80
- 7 McEwen B S. Gonadal steroid influence on brain development and sexual differentiation. Int Rev Physiol . 1983; 27 99-145
- 8 Feder H H. Hormones and sexual behaviour. Annu Rev Psychol . 1984; 35 165-200
- 9 Collaer M L, Hines M. Human behavioural sex differences: a role for gonadal hormones during early development. Psychol Bull . 1995; 118 55-107
- 10 Beach F A. Animal models for human sexuality. Ciba Found Symp . 1979; 62 113-146
- 11 Diamond M, Sigmundson H K. Sex assignment at birth: long-term review and clinical implications. Arch Pediatr Adolesc Med . 1997; 151 298-304
- 12 Diamond M. Sexual identity and sexual orientation in children with traumatized or ambiguous genitalia. J Sex Res . 1997; 34 199-211
- 13 Meyer-Bahlburg H FL. Psychoendocrine research on sexual orientation: current status and future options. Prog Brain Res . 1984; 61 367-390
- 14 Swaab D F, Hofman M A. Sexual differentiation of the human hypothalamus: ontogeny of the sexually dimorphic nucleus. Dev Brain Res . 1988; 44 314-318
- 15 Arnold A P, Gorski R A. Gonadal steroid induction of structural sex differences in the central nervous system. Annu Rev Neurosci . 1984; 7 413-442
- 16 Swaab D F, Gooren L JG, Hofman M A. Brain research, gender and sexual orientation. J Homosex . 1995; 28 383-302
- 17 Swaab D F, Gooren L JG, Hofman M A. The human hypothalamus in relation to gender and sexual orientation. Prog Brain Res . 1992; 93 205-219
- 18 Swaab D F, Fliers E. A sexually dimorphic nucleus in the human brain. Science . 1985; 228 1112-1114
- 19 Zhou J N, Hofman M A, Gooren L JG. A sex difference in the human brain and its relation to transsexuality. Nature . 1995; 378 68-70
- 20 LeVay S. A difference in hypothalamic structure between heterosexual and homosexual men. Science . 1991; 253 1034-1037
- 21 Kruijver F PM, Fernandez-Guasti M, Fodor M, Kraan E M, Swaab D F. Sex differences in androgen receptors of the human mammillary bodies are related to endocrine status rather than to sexual orientation or transsexuality. J Clin Endocrinol Metab . 2001; 86 818-827
- 22 Zucker K J. Intersexuality and gender identity differentiation. Annu Rev Sex Res . 1999; 10 1-69
- 23 Hines M. Abnormal sexual development and psychosexual issues. Baillieres Clin Endocrinol Metab . 1998; 12 173-189
- 24 Migeon C J, Wisniewski A B. Human sexual differentiation: from transcription factors to gender. Horm Res . 2000; 53 111-119
- 25 Money J. Hormones, hormonal anomalies and psychological health care. In: Kappy MS, Blizzard RM, Migeon CJ, eds. Wilkins Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence 4th ed. Springfield, IL: Charles C Thomas 1994: 1141-1178
- 26 Alvarez-Nava F, Gonzalez S, Soto M. Complete androgen insensitivity syndrome: clinical and anatomopathological findings in 23 patients. Genet Couns . 1997; 8 7-12
- 27 Money J, Schwartz M, Lewis V G. Adult erotosexual status and fetal hormonal masculinization and demasculinization: 46,XX congenital virilizing adrenal hyperplasia and 46,XY androgen insensitivity syndrome compared. Psychoneuroendocrinology . 1984; 9 405-414
- 28 Wisniewski A B, Meyer-Bahlburg H F, Gearhardt J P. Complete androgen insensitivity syndrome: long-term medical, surgical and psychosexual outcome. J Clin Endocrinol Metab . 2000; 85 2664-2669
- 29 Boehmer A L, Bruggenwirth H, van Assendelft C. Genotype versus phenotype in families with androgen insensitivity syndrome. J Clin Endocrinol Metab . 2001; 86 4151-4160
- 30 Price P, Wass J AH, Griffin J E. High dose androgen therapy in male pseudohermaphroditism due to 5α-reductase deficiency and disorders of the androgen receptor. J Clin Invest . 1984; 74 1496-1508
- 31 Slijper F M, Frets P G, Boehmer A L, Drop S L, Niermeijer M F. Androgen insensitivity syndrome (AIS): emotional reactions of parents and adult patients to the clinical diagnosis of AIS and its confirmation by androgen receptor gene mutation analysis. Horm Res . 2000; 53 9-15
- 32 Gooren L JG, Cohen-Kettenis P T. Development of a male gender identity/role and a sexual orientation to women in a 46,XY subject with an incomplete form the androgen insensitivity syndrome. Arch Sex Behav . 1991; 20 459-470
- 33 Imperato-McGinley J, Peterson R E, Gautier T. Androgens and the evolution of male-gender identity among male pseudohermaphrodites with 5α-reductase deficiency. N Engl J Med . 1979; 300 1233-1237
- 34 Herdt G, Davidson J: The Sambia ``turnim-man'': sociocultural and clinical aspects of gender formation in male pseudohermaphrodites with 5α-reductase deficiency in Papua New Guinea. Arch Sex Behav . 1988; 17 33-56
- 35 Wilson J D. The role of androgens in male gender role behavior. Endocr Rev . 1999; 20 726-737
- 36 Andersson S, Geisler W M, Wu L. Molecular genetics and pathophysiology of 17β-Hydroxysteroid dehydrogenase 3 deficiency. J Clin Endocrinol Metab . 1996; 81 130-136
- 37 Boehmer A L, Brinkmann A O, Sandkuijl L A. 17beta-Hydroxysteroid dehydrogenase: diagnosis, phenotypic variability, population genetics, worldwide distribution of ancient and de novo mutations. J Clin Endocrinol Metab . 1999; 84 4713-4721
- 38 Andersson S, Russell D W, Wilson J D. 17β-Hydroxysteroid dehydrogenase 3 deficiency. Trends Endocrinol Metab . 1996; 7 121-126
- 39 Dittman R W, Kappes M H, Kappes M E. Congenital adrenal hyperplasia: I. Gender-related behaviour and attitudes in female patients and sisters. Psychoneuroendocrinology . 1990; 15 401-420
- 40 Dittman R W, Kappes M E, Kappes M H. Sexual behavior in adolescent and adult females with congenital adrenal hyperplasia. Psychoneuroendocrinology . 1992; 17 153-170
- 41 Kuhnle U, Bullinger M, Schwarz H P. Partnership and sexuality in adult female patients with congenital adrenal hyperplasia: first results of a cross-sectional quality of life evaluation. J Steroid Biochem Mol Biol . 1993; 45 123-126
- 42 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 malformation and chronic disease on female patients' life. Eur J Pediatr . 1995; 154 708-715
- 43 Meyer-Bahlburg H F. Gender and sexuality in classic congenital adrenal hyperplasia. Endocrinol Metab Clin North Am . 2001; 30 155-171
- 44 Milaikal R M, Migeon C J, Rock J A. Fertility rates in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. N Engl J Med . 1987; 316 178-182
- 45 Meyer-Bahlburg H F, Gruen R S, New M I. Gender change from female to male in classical congenital adrenal hyperplasia. Horm Behav . 1996; 30 319-332
- 46 Money J. Gay, Straight and In-between. New York: Oxford University Press 1988
- 47 Hines M, Alsum P, Roy M. Oestrogenic contributions to sexual differentiation in the female guinea pig: influences of diethylstilbestrol and tamoxifen on neural, behavioural and ovarian development. Horm Behav . 1987; 21 402-417
- 48 Olsen K L. Androgen-insensitive rats are defeminized by their testes. Nature . 1979; 279 238-239
- 49 Money J, Matthews D. Prenatal exposure to virilizing progestins: an adult follow-up study of twelve women. Arch Sex Behav . 1982; 11 73-83
- 50 Ehrhardt A A, Meyer-Bahlburg H FL, Rosen L R. Sexual orientation after prenatal exposure to oestrogen. Arch Sex Behav . 1985; 14 57-77
- 51 Gooren L JG, Fliers E, Courtney K. Biological determinants of sexual orientation. Annu Rev Sex Res . 1990; 1 175-196
- 52 Gooren L JG. The endocrinology of transsexualism: a review and a commentary. Psychoneuroendocrinology . 1990; 15 3-14
- 53 Faustini-Fustini M, Rochira V, Carani C. Oestrogen deficiency in men: where are we today?. Eur J Endocrinol . 1999; 140 111-129
- 54 MacDonald P C, Madden J D, Brennes P F. Origin of oestrogens in normal men and women with testicular feminization. J Clin Endocrinol Metab . 1979; 49 905-916
- 55 Meyer-Bahlburg H FL. Gender assignment in intersexuality. J Psychol Hum Sex . 1998; 10 1-21
- 56 Meyer-Bahlburg H FL. Gender assignment and reassignment in 46,XY pseudohermaphroditism and related conditions. J Clin Endocrinol Metab . 1999; 84 3455-3458
- 57 Money J, Devore H, Norman B F. Gender identity and gender transposition: Longitudinal outcome study of 32 male hermaphrodites assigned as girls. J Sex Marital Ther . 1986; 12 165-181
- 58 Slijper F M, Drop S L, Molenaar J C. Long-term psychological evaluation of intersex children. Arch Sex Behav . 1998; 27 125-144
- 59 Bin-Abbas B, Conte F A, Grumbach M M, Kaplan S L. Congenital hypogonadotropic hypogonadism and micropenis: effect of testosterone treatment on adult penis size-why sex reversal is not indicated. J Pediatr . 1999; 134 579-584
- 60 Intersex Society of North America. Recommendations for treatment: intersex infants and children. Pamphlet, available from ISNA, P.O. Box 31791, San Francisco, CA 94131, 1995. Available at: htttp://www.isna.org .
- 61 Schober J M. Sexual behaviors, sexual orientation and gender identity in adult intersexuals: a pilot study. J Urol . 2001; 165 2350-2353
- 62 Melton L. New perspectives on the management of intersex. Lancet . 2001; 357 2210
- 63 Ochoa B. Trauma of the external genitalia in children: amputation of the penis and emasculation. J Urol . 1998; 160 1116-1119
- 64 Van Wijk J J, Calikoglu A S. Should boys with a micropenis be reared as girls?. J Pediatr . 1999; 134 537-538