Disorders of sex differentiation: A pediatric urologist's perspective of new terminology and recommendations

Journal of Urology

Volumn 185, Issue 2, 2011, Pages 393-400

  Barthold, Julia Spencer ^a  

^a NEMOURS ALFRED I DUPONT HOSPITAL FOR CHILDREN   (United States)

Author keywords

abnormalities; evidence based practice; genitalia; sex differentiation disorders; terminology as topic

Indexed keywords

AMBIGUOUS GENITALIA; ARTICLE; CONSENSUS; EVIDENCE BASED MEDICINE; FAMILY COUNSELING; FUTUROLOGY; HUMAN; INTERPERSONAL COMMUNICATION; MEDICAL DECISION MAKING; MEDLINE; NOMENCLATURE; PEDIATRICS; PRIORITY JOURNAL; PSYCHOSOCIAL CARE; QUALITY OF LIFE; SEX DIFFERENTIATION DISORDER; UNITED STATES; UROLOGIC SURGERY; UROLOGY;

EID: 78651319914     PISSN: 00225347     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.juro.2010.09.083     Document Type: Review

References (50)

1. H.F. Meyer-Bahlburg Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation Arch Sex Behav 34 2005 423
2. P.A. Lee, C.P. Houk, and S.F. Ahmed Consensus statement on management of intersex disorders International Consensus Conference on Intersex Pediatrics 118 2006 e488
3. C. Chase Rethinking treatment for ambiguous genitalia Pediatr Nurs 25 1999 451
4. J. Daaboul, and J. Frader Ethics and the management of the patient with intersex: a middle way J Pediatr Endocrinol Metab 14 2001 1575
5. S.M. Creighton, and L.M. Liao Changing attitudes to sex assignment in intersex BJU Int 93 2004 659
6. A.D. Dreger, C. Chase, and A. Sousa Changing the nomenclature/taxonomy for intersex: a scientific and clinical rationale J Pediatr Endocrinol Metab 18 2005 729
7. S.M. Creighton, C.L. Minto, and S.J. Steele Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood Lancet 358 2001 124
8. C.P. Houk, and P.A. Lee Consensus statement on terminology and management: disorders of sex development Sex Dev 2 2008 172
9. I.A. Hughes Disorders of sex development: a new definition and classification Best Pract Res Clin Endocrinol Metab 22 2008 119
10. M. Cools, L.H. Looijenga, and K.P. Wolffenbuttel Disorders of sex development: update on the genetic background, terminology and risk for the development of germ cell tumors World J Pediatr 5 2009 93
11. D. Wilhelm, S. Palmer, and P. Koopman Sex determination and gonadal development in mammals Physiol Rev 87 2007 1
12. E. Reis Divergence or disorder? The politics of naming intersex Perspect Biol Med 50 2007 535
13. C. Wiesemann, S. Ude-Koeller, and G.H. Sinnecker Ethical principles and recommendations for the medical management of differences of sex development (DSD)/intersex in children and adolescents Eur J Pediatr 169 2010 671
14. I.A. Hughes, C. Nihoul-Fekete, and B. Thomas Consequences of the ESPE/LWPES guidelines for diagnosis and treatment of disorders of sex development Best Pract Res Clin Endocrinol Metab 21 2007 351
15. V. Pasterski, P. Prentice, and I. Hughes Consequences of the Chicago consensus on disorders of sex development (DSD): current practices in Europe Arch Dis Child 95 2010 618
16. Yang JH, Baskin LS and DiSandro M: Gender identity in disorders of sex development: review article. Urology 75: 153.
17. J. Mieszczak, C.P. Houk, and P.A. Lee Assignment of the sex of rearing in the neonate with a disorder of sex development Curr Opin Pediatr 21 2009 541
18. H.F. Meyer-Bahlburg, C.J. Migeon, and G.D. Berkovitz Attitudes of adult 46, XY intersex persons to clinical management policies J Urol 171 2004 1615
19. C. Nihoul-Fekete How to deal with congenital disorders of sex development in 2008 (DSD) Eur J Pediatr Surg 18 2008 364
20. A.B. Dessens, F.M. Slijper, and S.L. Drop Gender dysphoria and gender change in chromosomal females with congenital adrenal hyperplasia Arch Sex Behav 34 2005 389
21. F. Gastaud, C. Bouvattier, and L. Duranteau Impaired sexual and reproductive outcomes in women with classical forms of congenital adrenal hyperplasia J Clin Endocrinol Metab 92 2007 1391
22. W.G. Reiner Gender identity and sex-of-rearing in children with disorders of sexual differentiation J Pediatr Endocrinol Metab 18 2005 549
23. J. Woelfle, W. Hoepffner, and W.G. Sippell Complete virilization in congenital adrenal hyperplasia: clinical course, medical management and disease-related complications Clin Endocrinol (Oxf) 56 2002 231
24. L. Frisen, A. Nordenstrom, and H. Falhammar Gender role behavior, sexuality, and psychosocial adaptation in women with congenital adrenal hyperplasia due to CYP21A2 deficiency J Clin Endocrinol Metab 94 2009 3432
25. H.F. Meyer-Bahlburg, C. Dolezal, and S.W. Baker Sexual orientation in women with classical or non-classical congenital adrenal hyperplasia as a function of degree of prenatal androgen excess Arch Sex Behav 37 2008 85
26. N.M. Stikkelbroeck, C.C. Beerendonk, and W.N. Willemsen The long term outcome of feminizing genital surgery for congenital adrenal hyperplasia: anatomical, functional and cosmetic outcomes, psychosexual development, and satisfaction in adult female patients J Pediatr Adolesc Gynecol 16 2003 289
27. G. Verkauskas, F. Jaubert, and S. Lortat-Jacob The long-term followup of 33 cases of true hermaphroditism: a 40-year experience with conservative gonadal surgery J Urol 177 2007 726
28. P.T. Cohen-Kettenis Gender change in 46,XY persons with 5alpha-reductase-2 deficiency and 17beta-hydroxysteroid dehydrogenase-3 deficiency Arch Sex Behav 34 2005 399
29. V. Sobel, and J. Imperato-McGinley Gender identity in XY intersexuality Child Adolesc Psychiatr Clin N Am 13 2004 609
30. A.B. Wisniewski, and T. Mazur 46,XY DSD with female or ambiguous external genitalia at birth due to androgen insensitivity syndrome, 5alpha-reductase-2 deficiency, or 17beta-hydroxysteroid dehydrogenase deficiency: a review of quality of life outcomes Int J Pediatr Endocrinol 2009 2009 567430
31. T. Mazur Gender dysphoria and gender change in androgen insensitivity or micropenis Arch Sex Behav 34 2005 411
32. A.B. Wisniewski, C.J. Migeon, and J.P. Gearhart Congenital micropenis: long-term medical, surgical and psychosexual follow-up of individuals raised male or female Horm Res 56 2001 3
33. C.J. Migeon, A.B. Wisniewski, and J.P. Gearhart Ambiguous genitalia with perineoscrotal hypospadias in 46,XY individuals: long-term medical, surgical, and psychosexual outcome Pediatrics 110 2002 e31
34. C. Nihoul-Fekete, E. Thibaud, and S. Lortat-Jacob Long-term surgical results and patient satisfaction with male pseudohermaphroditism or true hermaphroditism: a cohort of 63 patients J Urol 175 2006 1878
35. B. Mukherjee, E. McCauley, and R.B. Hanford Psychopathology, psychosocial, gender and cognitive outcomes in patients with cloacal exstrophy J Urol 178 2007 630
36. W.G. Reiner, and J.P. Gearhart Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth N Engl J Med 350 2004 333
37. L. Brinkmann, K. Schuetzmann, and H. Richter-Appelt Gender assignment and medical history of individuals with different forms of intersexuality: evaluation of medical records and the patients' perspective J Sex Med 4 2007 964
38. C.J. Migeon, A.B. Wisniewski, and T.R. Brown 46,XY intersex individuals: phenotypic and etiologic classification, knowledge of condition, and satisfaction with knowledge in adulthood Pediatrics 110 2002 e32
39. Liao LM, Green H, Creighton SM, et al. Service users' experiences of obtaining and giving information about disorders of sex development. BJOG 117: 193.
40. A. Nordenskjold, G. Holmdahl, and L. Frisen Type of mutation and surgical procedure affect long-term quality of life for women with congenital adrenal hyperplasia J Clin Endocrinol Metab 93 2008 380
41. A.B. Wisniewski, C.J. Migeon, and M.A. Malouf Psychosexual outcome in women affected by congenital adrenal hyperplasia due to 21-hydroxylase deficiency J Urol 171 2004 2497
42. T.H. Johannsen, C.P. Ripa, and E.L. Mortensen Quality of life in 70 women with disorders of sex development Eur J Endocrinol 155 2006 877
43. J.E. Dayner, P.A. Lee, and C.P. Houk Medical treatment of intersex: parental perspectives J Urol 172 2004 1762
44. M. Diamond, and H.G. Beh Changes in the management of children with intersex conditions Nat Clin Pract Endocrinol Metab 4 2008 4
45. N.S. Crouch, L.M. Liao, and C.R. Woodhouse Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia J Urol 179 2008 634
46. J. Yang, D. Felsen, and D.P. Poppas Nerve sparing ventral clitoroplasty: analysis of clitoral sensitivity and viability J Urol 178 2007 1598
47. L.H. Braga, and J.L. Pippi Salle Congenital adrenal hyperplasia: a critical appraisal of the evolution of feminizing genitoplasty and the controversies surrounding gender reassignment Eur J Pediatr Surg 19 2009 203
48. L. Allen Opinion one: a case for delayed gonadectomy J Pediatr Adolesc Gynecol 22 2009 381
49. D.A. Kiddo Opinion two: a case for early gonadectomy J Pediatr Adolesc Gynecol 22 2009 384
50. R. Hersmus, B.H. de Leeuw, and K.P. Wolffenbuttel New insights into type II germ cell tumor pathogenesis based on studies of patients with various forms of disorders of sex development (DSD) Mol Cell Endocrinol 291 2008 1