Gender change from female to male in classical congenital adrenal hyperplasia

Hormones and Behavior

Volumn 30, Issue 4, 1996, Pages 319-332

  Meyer Bahlburg, Heino F L ^a,b   Gruen, Rhoda S ^a,b   New, Maria I ^c   Bell, Jennifer J ^b   Morishima, Akira ^b   Shimshi, Mona ^d   Bueno, Yvette ^a   Vargas, Ileana ^c   Baker, Susan W ^c  

^a NEW YORK STATE PSYCHIATRIC INSTITUTE   (United States)

^b Coulumbia University ^*  (United States)

^c WEILL CORNELL MEDICAL CENTER   (United States)

^d ELMHURST HOSPITAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADULT; ARTICLE; CASE REPORT; CONGENITAL ADRENAL HYPERPLASIA; FEMALE; GENDER IDENTITY; HUMAN; PRIORITY JOURNAL; PSYCHOSEXUAL DEVELOPMENT; SEXUAL BEHAVIOR; SEXUAL ORIENTATION;

EID: 0030423655     PISSN: 0018506X     EISSN: None     Source Type: Journal    
DOI: 10.1006/hbeh.1996.0039     Document Type: Article

References (67)

1. American Psychiatric Association (1994). DSM-IV. Diagnostic and Statistical Manual of Mental Disorders, Fourth ed. American Psychiatric Association, Washington, DC.
2. Bakker, A., van Kesteren, P. J. M., Gooren, L. J. G., and Bezemer, P. D. (1993). The prevalence of transsexualism in the Netherlands. Acta Psychiatr. Scand. 87, 237-238.
3. Berenbaum, S. A., and Hines, M. (1992). Early androgens are related to childhood sex-typed toy preferences. Psychol. Science 3, 203-206.
4. Blanchard, R., and Freund, K. (1983). Measuring masculine gender identity in females. J. Consult. Clin. Psychol. 51, 205-214.
5. Burris, A. S., Banks, S. M., Carter, C. S., Davidson, J. M., and Sherins, R. J. (1992). A long-term, prospective study of the physiologic and behavioral effects of hormone replacement in untreated hypogonadal men. J. Androl. 13, 297-304.
6. Chan-Cua, S., Freidenberg, G., and Jones, K. L. (1989). Occurrence of male phenotype in genotypic females with congenital virilizing adrenal hyperplasia. Am. J. Med. Genet. 34, 406-412.
7. Dittmann, R. W., Kappes, M. H., Kappes, M. E., Börger, D., Stegner, H., Willig, R. H., and Wallis, H. (1990a). Congenital adrenal hyperplasia I: Gender-related behavior and attitudes in female patients and sisters. Psychoneuroendocrinology 15, 401-420.
8. Dittmann, R. W., Kappes, M. H., Kappes, M. E., Börger, D., Meyer-Bahlburg, H. F. L., Stegner, H., Willig, R. H., and Wallis, H. (1990b). Congenital adrenal hyperplasia II: Gender-related behavior and attitudes in female salt-wasting and simple-virilizing patients. Psychoneuroendocrinology 15, 421-434.
9. Dittmann, R. W., Kappes, M. E., and Kappes, M. H. (1992). Sexual behavior in adolescent and adult females with congenital adrenal hyperplasia. Psychoneuroendocrinology 17, 153-170.
10. Dott, S., Walling, D., Avery, E., Cole, C., and Meyer, W. (1995). Schizophrenia and transsexualism: Defining the boundaries. In XIV Harry Benjamin International Gender Symposium. Gender Dysphoria: Transcultural Perspectives. Transsexualism: State of the Art Treatment, p. 20. Kloster Irsee, Bavaria, Germany, September 7-10, 1995 [Program & Abstracts].
11. Ehrhardt, A. A., and Baker, S. W. (1974). Fetal androgens, human central nervous system differentiation, and behavior sex differences. In R. C. Friedman, R. M. Richart, and R. L. Vande Wiele (Eds.), Sex Differences in Behavior, pp. 33-51. Wiley, New York.
12. Ehrhardt, A. A., Epstein, R., and Money, J. (1968a). Fetal androgens and female gender identity in the early-treated adrenogenital syndrome. Johns Hopkins Med. J. 122, 160-167.
13. Ehrhardt, A. A., Evers, K., and Money, J. (1968b). Influence of androgen and some aspects of sexually dimorphic behavior in women with the late-treated adrenogenital syndrome. Johns Hopkins Med. J. 123, 115-122.
14. Ehrhardt, A. A., and Meyer-Bahlburg, H. F. L. (1981). Effects of prenatal sex hormones on gender-related behavior. Science 211, 1312-1318.
15. Ehrhardt, A. A., and Meyer-Bahlburg, H. F. L. (1984). Gender-Role Assessment Schedule - Adult (GRAS-A), 1984 ed. Copyrighted 1984.
16. Federman, D. D., and Donahoe, P. K. (1995). Ambiguous genitalia - Etiology, diagnosis, and therapy. Adv. Endocrinol. Metab. 6, 91-116.
17. Goy, R. W., and McEwen, B. S. (1980). Sexual Differentiation of the Brain. MIT Press, Cambridge.
18. Grellert, E. A., Newcomb, M. D., and Bentler, P. M. (1982). Childhood play activities of male and female homosexuals and heterosexuals. Arch. Sex. Behav. 11, 451-478.
19. Harry Benjamin International Gender Dysphoria Association (1990). Standards of Care. In The Hormonal and Surgical Sex Reassignment of Gender Dysphoric Persons, 1/90 Revision. (Available from The Harry Benjamin International Gender Dysphoria Association, Inc., 1515 El Camino Real, Palo Alto, CA 94306).
20. Herdt, G. (1994). Mistaken sex: Culture, biology and the third sex in New Guinea. In G. Herdt (Ed.), Third Sex, Third Gender. Beyond Sexual Dimorphism in Culture and History, pp. 419-445. Zone Books, New York.
21. Hines, M., and Collaer, M. L. (1993). Gonadal hormones and sexual differentiation of human behavior: Developments from research on endocrine syndromes and studies of brain structure. Ann. Rev. Sex Res. 4, 1-48.
22. Hines, M., and Kaufman, F. R. (1994). Androgen and the development of human sex-typical behavior: Rough-and-tumble play and sex of preferred playmates in children with congenital adrenal hyperplasia (CAH). Child Dev. 65, 1042-1053.
23. Hinman, F., Jr. (1951). Sexual trends in female pseudohermaphrodism. J. Clin. Endocrinol. Metab. 11, 477-486.
24. Hochberg, Z., Gardos, M., and Benderly, A. (1987). Psychosexual outcome of assigned females and males with 46,XX virilizing congenital adrenal hyperplasia. Eur. J. Pediatr. 146, 497-499.
25. Imperato-McGinley, J., Peterson, R. E., Gautier, T., and Sturla, E. (1979). Androgens and the evolution of male-gender identity among male pseudohermaphrodites with 5α-reductase deficiency. N. Engl. J. Med. 300, 1233-1237.
26. Intersex Society of North America (1995). Recommendations for Treatment: Intersex Infants and Children. Pamphlet, available from ISNA, P.O. Box 31791, San Francisco, CA 94131.
27. Kemper, T. D. (1990). Social Structure and Testosterone. Rutgers University Press, New Brunswick, NJ.
28. Legros, J. J., van Cauwenberge, H., Lambotte, R., Bauduin, A., Franchmont, P., and Legros, J. (1974). Problèmes psychoendocriniens posés par un cas d'hyperplasie, surrenalienne congenitale reconnu tardivement. Rev. Med. Liège 29, 73-80.
29. Meyer-Bahlburg, H. F. L. (1982). Hormones and psychosexual differentiation: Implications for the management of intersexuality, homosexuality, and transsexuality. Clinics Endocrinol. Metab. 11(3), 681-701.
30. Meyer-Bahlburg, H. F. L. (1984). Psychoendocrine research on sexual orientation. Current status and future options. Prog. Brain Res. 61, 375-398.
31. Meyer-Bahlburg, H. F. L. (1985). Gender Identity Disorder of Childhood. Introduction. J. Am. Acad. Child Adolesc. Psychiatry 24, 681-683.
32. Meyer-Bahlburg, H. F. L. (1993). Gender identity development in intersex patients. Child Adolesc. Psychiatr. Clinics N. Am. 2, 501-512.
33. Meyer-Bahlburg, H. F. L. (1994). Intersexuality and the diagnosis of gender identity disorder. Arch. Sex. Behav. 23, 21-40.
34. Meyer-Bahlburg, H. F. L. (1995). What causes low rates of child-bearing in CAH? Symposium "Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency," Baltimore, MD, June 11-13, 1995.
35. Meyer-Bahlburg, H. F. L., and Ehrhardt, A. A. (1983). Sexual Behavior Assessment Schedule - Adult (SEBAS-A), 1983 ed. Copyrighted 1983.
36. Meyer-Bahlburg, H. F. L., Sandberg, D. E., Dolezal, C. L., and Yager, T. J. (1994a). Gender-related assessment of childhood play. J. Abnorm. Child Psychol. 22, 643-660.
37. Meyer-Bahlburg, H. F. L., Sandberg, D. E., Yager, T. J., Dolezal, C. L., and Ehrhardt, A. A. (1994b). Questionnaire scales for the assessment of atypical gender development in girls and boys. J. Psychol. Hum. Sexuality 6, 19-39.
38. Money, J. (1968). Psychologic approach to psychosexual misidentity with elective mutism: Sex reassignment in two cases of hyperadrenocortical hermaphroditism. Clin. Pediatr. 7, 331-339.
39. Money, J. (1991). Biographies of Gender and Hermaphroditism in Paired Comparisons. Elsevier Science, Amsterdam, The Netherlands.
40. Money, J. (1994). Sex Errors of the Body and Related Syndromes. A Guide to Counseling Children, Adolescents, and Their Families, 2nd ed. Paul H. Brookes Publishing, Baltimore, MD.
41. Money, J., and Ambinder, R. (1978). Two-year, real-life diagnostic test: Rehabilitation versus cure. In J. P. Brady and H. K. H. Brodie (Eds.), Controversy in Psychiatry, pp. 833-845. Saunders, Philadelphia.
42. Money, J., and Daléry, J. (1976). Iatrogenic homosexuality: Gender identity in seven 46,XX chromosomal females with hyperadrenocortical hermaphroditism born with a penis, three reared as boys, four as girls. J. Homosex. 1, 357-371.
43. Money, J., Devore, H., and Norman, B. F. (1986). Gender identity and gender transposition: Longitudinal outcome study of 32 male hermaphrodites assigned as girls. J. Sex Marital Ther. 12, 165-181.
44. Money, J., and Ehrhardt, A. A. (1972). Man and Woman, Boy and Girl: Differentiation and Dimorphism of Gender Identity from Conception to Maturity. The Johns Hopkins Univ. Press, Baltimore, MD.
45. Money, J., Hampson, J. G., and Hampson, J. L. (1955). Hermaphroditism: Recommendations concerning assignment of sex, change of sex, and psychologic management. Bull. Johns Hopkins Hosp. 97, 284-300.
46. Money, J., Lehne, G. K., and Pierre-Jerome, F. (1985). Micropenis: Gender, erotosexual coping strategy, and behavioral health in nine pediatric cases followed to adulthood. Comp. Psychiatry 26, 29-42.
47. Money, J., and Schwartz, M. (1977). Dating, romantic and nonromantic friendships, and sexuality in 17 early-treated adrenogenital females, aged 16-25. In P. A. Lee, L. P. Plotnick, A. A. Kowarski, and C. J. Migeon (Eds.), Congenital Adrenal Hyperplasia, pp. 419-431. University Park Press, Baltimore.
48. Mulaikal, R. M., Migeon, C. J., and Rock, J. A. (1987). Fertility rates in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. N. Engl. J. Med. 316, 178-182.
49. New, M. I. (1995). Steroid 21-hydroxylase deficiency (congenital adrenal hyperplasia). Am. J. Med. 98(1A), 2S-8S.
50. Newman, K., Randolph, J., and Parson, S. (1992). Functional results in young women having clitoral reconstruction as infants. J. Pediatr. Surg. 27, 180-184.
51. Pilgrim, Ch., and Reisert, I. (1992). Differences between male and female brains - Developmental mechanisms and implications. Horm. Metab. Res. 24, 353-359.
52. Rally, J. M., and Woodhouse, C. R. J. (1989). Small penis and the male sexual role. J. Urol., Part 2, 142, 569-571.
53. Rosenwald, A. K., Handlon, J. H., Rosenthal, I. M., Hyde, J. S., and Bronstein, I. P. (1958). Psychologic studies before and after clitoridectomy in female pseudohermaphroditism caused by congenital virilizing adrenal hyperplasia. Pediatrics 21, 832-839.
54. Sato, T., and Koizumi, S. (1991). Effects of fetal androgen on childhood behavior. Acta Paediatr. Jpn. 33, 639-644.
55. Silverstein, A. B. (1982). Two- and four-subtest short forms of the Wechsler Adult Intelligence Scale-Revised. J. Consult. Clin. Psychol. 50, 415-418.
56. Slijper, F. M. E. (1984). Androgens and gender role behaviour in girls with congenital adrenal hyperplasia (CAH). Prog. Brain Res. 61, 417-422.
57. Slijper, F. M. E., van der Kamp, H. J., Brandenburg, H., de Muinck Keizer-Schrama, S. M. P. F., Drop, S. L. S., and Molenaar, J. C. (1992). Evaluation of psychosexual development of young women with CAH: A pilot study. In W. Bezemer, P. Cohen-Kettenis, K. Slob, and N. van Son-Schoones (Eds.), Sex Matters, pp. 47-50. Elsevier, Amsterdam.
58. Spitzer, R. L., and Endicott, J. (1978). Schedule for Affective Disorder and Schizophrenia - Life-time Version, 3rd ed. (For use in clinical studies of the NIMH - Clinical Research Branch Collaborative Program on the Psychobiology of Depression.)
59. Stoller, R. J. (1964). The hermaphroditic identity of hermaphrodites. J. Nerv. Ment. Dis. 139, 453-457.
60. Udry, J. R., Morris, N. M., and Kovenock, J. (1995). Androgen effects on women's gendered behaviour. J. Biosoc. Sci. 27, 359-368.
61. van der Kamp, H. J., Slijper, F. M. E., Brandenburg, H., de Muinck Keizer-Schrama, S. M. P. F., Drop, S. L. S., and Molenaar, J. C. (1992). Evaluation of young women with congenital adrenal hyperplasia: A pilot study. Horm. Res. 2(Suppl. 3), 45-49.
62. van Goozen, S. H. M., Cohen-Kettenis, P. T., Gooren, L. J. G., Frijda, N. H., and van de Poll, N. E. (1995). Gender differences in behaviour: Activating effects of cross-sex hormones. Psychoneuroendocrinology 20, 343-363.
63. van Seters, A. P., and Slob, A. K. (1988). Mutually gratifying heterosexual relationship with micropenis of husband. J. Sex Marital Ther. 14, 98-107.
64. Wechsler, D. (1981). WAIS-R Manual: Wechsler Adult Intelligence Scale-Revised. Psychological Corp., New York.
65. White, P. C., Curnow, K. M., and Pascoe, L. (1994). Disorders of steroid 11 beta-hydroxylase isozymes. Endocr. Rev. 15, 421-438.
66. Wilson, R. C., Mercado, A. B., Cheng, K. C., and New, M. I. (1995). Steroid 21-hydroxylase deficiency: Genotype may not predict phenotype. J. Clin. Endocrinol. Metab. 80, 2322-2329.
67. Zucker, K. J., Bradley, S. J., Oliver, G., Blake, J., Fleming, S., and Hood, J. Psychosexual assessment of women with congenital adrenal hyperplasia. Hormones Behav., 30, 300-318.