Home LiteratureArticle Details
PMID: 6895206 Published · ppublish English Journal Article

The gonads of human true hermaphrodites.

Human genetics ·Vol. 58 ·No. 1 ·1981-00-00 ·Pages 117-22

van Niekerk WA, Retief AE

Abstract

Gonadal distribution in 409 cases of human true hermaphroditism is reviewed. An ovary was found on the left side of the body in 62.8% of the cases and the testis on the right side in 59.5%. The ovotestis is the most common gonad of the true hermaphrodite; amongst 806 gonads in 406 cases it was found in 44.3%. In this paper we give a detailed description of the morphology of ovotestis, testis and ovary in the true hermaphrodite. In addition we discuss the effects of fetal androgens and Müllerian inhibiting factor on the Wolffian and Müllerian ducts. Correlations between chromosomal complement and gonadal distribution are presented. True hermaphrodites with a 46,XX karyotype most commonly have an ovary on one side and an ovotestis on the other side; those with a Y-chromosome have a testis in 61% of cases. An analysis of the ratio of ovarian and testicular tissue within ovotestes showed a continuum from very little ovarian tissue to a small portion of testicular tissue. Each type of tissue was clearly demarcated. Hypotheses for gonadal induction in the true hermaphrodite should take cognizance of these facts. True hermaphrodites with a 46,XX chromosomal complement were characterized by a male phenotype in 54% of cases. This group may suggest a greater testicular induction ability in the genome as compared to the 46% with a female phenotype.

MeSH Terms
Androgens/physiology Anti-Mullerian Hormone Disorders of Sex Development/embryology,pathology Dysgerminoma/pathology Embryonic Induction Female Genes Glycoproteins Gonads/pathology Growth Inhibitors H-Y Antigen/genetics Humans Karyotyping Male Mullerian Ducts Ovary/embryology Phenotype Risk Sex Chromosomes Testicular Hormones/physiology Testis/embryology Wolffian Ducts
Chemicals
Androgens Glycoproteins Growth Inhibitors H-Y Antigen Testicular Hormones Anti-Mullerian Hormone
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
van Niekerk W A
Retief A E
References (31)
31 references, click to expand
  1. True hermaphroditism: a rational appraoch to diagnosis and treatment.
    Urology. 1980 Jun;15(6):569-74 PMID: 7394982
  2. A cytogenetic investigation of inherited true hermaphroditism in BALB/cWt mice.
    Cytogenet Cell Genet. 1980;28(1-2):104-15 PMID: 7470243
  3. Dysgerminoma of the ovary associated with true hermaphroditism.
    Obstet Gynecol. 1980 Jul;56(1):102-6 PMID: 7383471
  4. Pregnancy and childbirth in a true hermaphrodite.
    Obstet Gynecol. 1975 May;45(5):593-5 PMID: 1124181
  5. Origin of chi46,XX/46,XY chimerism in a human true hermaphrodite.
    Science. 1980 Jan 18;207(4428):321-3 PMID: 7350665
  6. The antimüllerian hormone.
    Recent Prog Horm Res. 1976;33:117-67 PMID: 801190
  7. Endocrine studies and successful treatment in a patient with true hermaphroditism.
    Acta Endocrinol (Copenh). 1979 May;91(1):184-92 PMID: 377882
  8. H-Y antigen mosaicism in the gonad of a 46,XX true hermaphrodite.
    N Engl J Med. 1979 Apr 5;300(14):745-9 PMID: 570639
  9. X-Y chromosomal interchange in the aetiology of true hermaphroditism and of XX Klinefelter's syndrome.
    Lancet. 1966 Aug 27;2(7461):475-6 PMID: 4161595
  10. A case of true lateral hermaphroditism with seminiferous intratubular bodies.
    J Urol. 1962 Dec;88:822-5 PMID: 13948576
  11. True hermaphroditism: an analytic review with a report of 3 new cases.
    Am J Obstet Gynecol. 1976 Dec 1;126(7):890-907 PMID: 1033669
  12. Production and origin of estrogen in two true hermaphrodites.
    Am J Obstet Gynecol. 1978 Oct 15;132(4):401-9 PMID: 568390
  13. True hermaphrodite with multiple gonadal neoplasms: report of a case with cytogenetic study.
    Cancer. 1978 Dec;42(6):2726-32 PMID: 728870
  14. True hermaphroditism with peripheral blood and gonadal karyotyping.
    Obstet Gynecol. 1979 Nov;54(5):549-53 PMID: 503378
  15. H-Y antigen and the genetics of sex determination.
    Science. 1977 Nov 25;198(4319):797-9 PMID: 335511
  16. Familial true hermaphroditism.
    Turk J Pediatr. 1978 Jan-Apr;20(1-2):29-34 PMID: 757014
  17. The human Y chrosome and the etiology of true hermaphroditism with the report of a case with XX-XY sex chromosome mosaicism.
    Hereditas. 1965;53(1):231-46 PMID: 5889237
  18. Gonadoblastoma in a true hermaphrodite with a 46,XX karyotype.
    Obstet Gynecol. 1976 Mar;47(3):355-8 PMID: 1250564
  19. Report of a case of true hermaphroditism; a discussion of the 37 cases previously reported.
    J Urol. 1946 Dec;56(6):720-4 PMID: 20279554
  20. [On a new case of true hermaphroditism (Bilateral diversity with double ovotestis in the inguinal canal)].
    An R Acad Nac Med (Madr). 1960;77:261-87 PMID: 13803120
  21. Asymmetrical gonadal enlargement in adolescent true hermaphrodite with bilateral ovotestes.
    Urology. 1979 Jan;13(1):63-6 PMID: 442325
  22. Genetic basis of XX male syndrome and XX true hermaphroditism: evidence in the dog.
    Science. 1978 Aug 18;201(4356):644-6 PMID: 675252
  23. Pregnancy without benefit of reconstructive surgery in a bisexually active true hermaphrodite.
    Am J Obstet Gynecol. 1979 Oct 1;135(3):427-8 PMID: 484636
  24. Familial XX true hermaphroditism and the H-Y antigen.
    Hum Genet. 1979 Apr 17;48(1):45-52 PMID: 110671
  25. A gene controlling H-Y antigen on the X chromosome. Tentative assignment by deletion mapping to Xp223.
    Hum Genet. 1980;54(2):149-54 PMID: 7390489
  26. Hermaphroditism and pseudohermaphroditism: case reports.
    Del Med J. 1960 Apr;32:161-4 PMID: 14412963
  27. Evaluation of hypothalamo-pituitary-gonadal axis in true hermaphroditism: a case report.
    Int J Fertil. 1979;24(2):120-4 PMID: 40905
  28. Gonadoblastoma in a true hermaphrodite with 46, XY genotype.
    Obstet Gynecol. 1972 Oct;40(4):466-72 PMID: 4672494
  29. Pregnancy in a true hermaphrodite.
    Obstet Gynecol. 1979 Mar;53(3 Suppl):40S-42S PMID: 570686
  30. A true hermaphrodite.
    J Urol. 1955 Feb;73(2):424-9 PMID: 13234136
  31. Pregnancy and childbirth in a true hermaphrodite following reconstructive surgery.
    Br J Obstet Gynaecol. 1978 Apr;85(4):314-6 PMID: 638099
Article Info
Journal
Human genetics
Abbr.
Hum Genet
ISSN
0340-6717
Published
1981-00-00
Pages
117-22
Language
English
Region
Germany
NLM ID
7613873
Subset
IM
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: [email protected]