True hermaphroditism: Presentation, management, outcome

Endocrinologist

Volumn 13, Issue 3, 2003, Pages 214-218

  Kuhnle, Ursula ^a   Krob, Gabriele ^a   Maier, Esther ^a,b  

^a UNIVERSITY OF MUNICH   (Germany)

^b Center for Child/Adolescent Health   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ESTROGEN; TESTOSTERONE;

ADULT; CASE REPORT; CHROMOSOME; CLINICAL FEATURE; CONFERENCE PAPER; FEMALE; FERTILITY; GENDER IDENTITY; GONAD; HORMONE SUBSTITUTION; HUMAN; HUMAN TISSUE; INCIDENCE; INHERITANCE; MALE; MALIGNANT NEOPLASTIC DISEASE; PATHOGENESIS; PHENOTYPE; PREGNANCY; PSYCHOLOGICAL ASPECT; TRUE HERMAPHRODITISM;

EID: 0037591717     PISSN: 10512144     EISSN: None     Source Type: Journal    
DOI: 10.1097/01.ten.0000081681.43801.52     Document Type: Conference Paper

References (25)

1. van Niekerk WA: True hermaphroditism. In Josso N, Laron Z, eds: The Intersex Child. Pediatric and Adolescent Endocrinology, vol. 8. Basel: Karger; 1981:80-99.
2. Salen: Ein Fall von Hermaphroditismus verus beim Menschen. Verh Dt Ges Path 1899;2:241-3.
3. Krob G, Braun A, Kuhnle U: True hermaphroditism: Worldwide incidence, chromosomal distribution, clinical findings, and gonadal histology. Eur J Pediatr 1994;153:2-10.
4. Sarafoglou K, Ostrer H: Familial sex reversal: A Review. J Clin Endocrin Metab 2000;85:483-93.
5. Yordam N, Alikasifoglu A, Kandemir N, et al.: True hermaphroditism: clinical features, genetic variants and gonadal histology. J Pediatr Endocrinol Metab 2001;14:421-7.
6. Domenice S, Nishi MY, Billerbeck AE, et al.: Molecular analysis of SRY gene in Brazilian 46,XX sex reversed patients: absence of SRY sequence in gonadal tissue. Med Sci Monit 2001;7:238-41.
7. Wiersma R: Management of the African child with true hermaphroditism. J Pediatr Surg 2001;36:397-9.
8. Guo L, Liu T, Tian Q: Pathological characteristics of gonads in nine patients with true hermaphroditism. Zhonghua Bing Li Xue Za Zhi 1998;27:209-12.
9. Damiani D, Fellous M, McElvarey K, et al.: True hermaphroditism: clinical aspects and molecular studies in 16 cases. Eur J Endocrinol 1997;136:201-4.
10. Mittwoch U: Sex differentiation in mammals and tempo of growth: probabilities. J Theor Biol 1989;137:445-55.
11. Kuhnle U, Krahl W: The impact of culture on sex assignment and gender development in intersex patients. Persp Biol Med 2002;45:85-103.
12. Lim HN, Berkovitz GD, Hughes IA, Hawkins JR: Mutation analysis of subjects with 46,XX sex reversal and 46,XY gonadal dysgenesis does not support the involvement of SOX3 in testis determination. Hum Genet 2000;107:650-2.
13. Kuhnle U, Schwarz HP, Löhrs U, et al.: Familial true hermaphroditism: paternal and maternal transmission of true hermaphroditism (46,XX) and XX-maleness in the absence of Y-chromosomal sequences Hum Genetics 1993;92:571-6.
14. Maier EM, Leitner C, Loehrs U, et al.: True hermaphroditism due to a familial point mutation of the SRY gene in an extended family. Hormone Res 2000;53(suppl 2):87.
15. Hawkins JR: Genetics of XY sex reversal. J Endocrinol 1995;147:183-7.
16. Braun A, Kammerer S, Cleve H, et al.: True hermaphroditism in a 46,XY individual caused by a post-cygotic somatic point mutation in the male gonadal sex-determining locus (SRY). Molecular genetics and histological findings in a sporadic case. Am J Hum Gen 1993;52:578-85.
17. Brown S, Yu C, Lanzano P, et al.: A de novo mutation (Gln2Stop) at the 5′ end of the SRY gene leads to sex reversal with partial ovarian function Am J Hum Gen 1998;62:189-92.
18. Palter SF, Tavares AB, Hourvitz A, et al.: Are estrogens of import to primate/human ovarian folliculogenesis? Endocrine Rev 2001;22:389-424.
19. Parvin SD: Ovulation in a cytogenetically proved phenotypically male fertile hermaphrodite. Br J Surg 1982;69:279-80.
20. Mayou BJ, Armon P, Lindenbaum RH: Pregnancy and childbirth in a true hermaphrodite following reconstrutive surgery. Br J Obstet Gynaecol 1978;85:314-6.
21. Tiltman AJ, Sweerts M: Multiparity in a covert true hermaphrodite. Obstet Gynecol 1982;60:752-4.
22. Toublanc JE, Boucekkine C, Abbas N, et al: Hormonal and molecular genetic findings in 46,XX subjects with sexual ambiguity and testicular differentiation Eur J Pediatr 1993;152(suppl 2):570-575.
23. Boucekkine C, Toublanc JE, Abbas N, et al.: Clinical and anatomical spectrum in XX sex reversed patients. Relationship to the presence of Y specific DNA-sequences. Clin Endocrin 1994;40:733-42.
24. Aaronson IA: True hermaphroditism. A review of 41 cases with observations on testicular histology and function. Br J Urol 1985;57:775-9.
25. Fausto-Sterling A: Sexing the Body. Gender Politics and the Construction of Sexuality. New York: Basic Books; 2000.