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Zusammenfassung

Die normale somatische Geschlechtsentwicklung besteht aus chromosomal bedingter Geschlechtsdetermination und Differenzierung des gonadalen Geschlechts (Testis oder Ovar), mit nachfolgender Entwicklung des jeweiligen phänotypischen Geschlechts, männlich oder weiblich. Die Geschlechtsdifferenzierung wird, etwas schematisch gesehen, auf mehreren Ebenen determiniert: 1) genetisches Geschlecht (chromosomal determiniert), 2) gonadales Geschlecht (hormoneh determiniert), 3) phänotypisches Geschlecht (anatomisch determiniert), 4) psychologisches Geschlecht. Eine weitere Ebene wäre die juristische, standesamtlich festgelegte Geschlechtszuordnung. Bei normalem Verlauf der Geschlechtsdifferenzierung stimmen alle Ebenen überein.

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Literatur

  1. Aiman J, Griffin JE, Gazak JM, Wilson JE, MacDonald PC (1979) Androgen insensidvity as a cause of infertility in otherwise normal men. Engl J Med 300:223–227

    Article  CAS  Google Scholar 

  2. Amrhein J, Meyer W, Jones H, Migeon C (1976) Androgen insensitivity in man: Evidence for genetic heterogeneity. Proc Nad Acad Sci USA 73:891

    Article  CAS  Google Scholar 

  3. Armendares S, Buentello L, Frenk S (1973) Two smaU sibs with uterus and fallopian tubes, a rare, probably inherited disorder. Chn Genet 4:291

    Article  CAS  Google Scholar 

  4. Axelrod L, Neer R, Kilman B (1979) Hypogonadism in a male with immunologically acdve, biologically inactive luteinizing hormone: An exception to a venerable rule. J Clin Endocrinol Metab 48:279

    Article  PubMed  CAS  Google Scholar 

  5. Bardin CW, Catterall JF (1981) Testosterone: A major determinant of extragenital sexual dimorphism. Science 211:1285–1294

    Article  PubMed  CAS  Google Scholar 

  6. Bergsma D (ed) (1971) The chnical delineation of birth defects, part X. Nadonal Foundation, New York (The endocrine system. Birth defects: Original article series vol VII/6)

    Google Scholar 

  7. Berthezene F, Forest MG, Grimauld JA, Claustrat B, Momex R (1976) Leydig ceU agenesis. A cause of male pseudohermaphrodidsm. N Engl J Med 295:969–972

    Article  PubMed  CAS  Google Scholar 

  8. Brown D, Markland C, Dehner L (1978) Leydig cell hypoplasia: A cause of male pseudohermaphroditism. J Chn Endocrinol Metab 46:1

    Article  CAS  Google Scholar 

  9. Bühler EM (1980) A synopsis of the human Y chromosome. Hum Genet 55:145–175

    Article  PubMed  Google Scholar 

  10. Crigler JF, Scully RE (1977) Severe hypospadias and cryptorchidism in a five-year-old boy. N Engl J Med 296:803–809

    Article  Google Scholar 

  11. Davis RM (1981) Locahsation of male determin factors in man: A thorough review of structural anomahes of the Y chromosome. J Med Genet 18:161–195

    Article  PubMed  CAS  Google Scholar 

  12. De la Chapelle A (1981) The etiology of maleness in XX men. Hum Genet 58:105–116

    Article  PubMed  Google Scholar 

  13. De la Chapelle A, Koo GS, Wachtel SS (1978) Recessive sex-determining genes in human XX male syndrome. Cell 15:837–842

    Article  PubMed  Google Scholar 

  14. DiGeorge A (1979) Genetics of endocrine diseases. In: Jackson LG, Schimke RN (eds) Clinical genetics. A source book for physicians. Wiley & Sons, New York, Chichester, pp 285

    Google Scholar 

  15. Evans HJ, Buckton KE, Spowart G, Carothers AD (1979) Heteromorphic X chromosomes in 46, XX males: Evidence for the involvement of X-Y interchange. Hum Genet 49:11–31

    PubMed  CAS  Google Scholar 

  16. Fichman KR, Migeon BR, Migeon CJ (1980) Genetic disorders of male sexual differentiation. Adv Hum Genet 10:333–377

    PubMed  CAS  Google Scholar 

  17. Fraccaro M, Zuffardi O, Baggio P, Console V, Valagussa E (1980) Campomelic dysplasia and sex reversal. In: Spranger J, Tolksdorf M (Hrsg) Khnische Genetik in der Pädiatrie. Thieme, Stuttgart, pp 62–68

    Google Scholar 

  18. German J, Simpson JL, Chaganti RSK (1978) Genetically determined sex-reversal in 46, XY humans. Science 202:53–56

    Article  PubMed  CAS  Google Scholar 

  19. Gordon JW, Ruddle FH (1981) Mammahan gonadal determination and gametogenesis. Science 211:1265–1271

    Article  PubMed  CAS  Google Scholar 

  20. Griffm JE, Wilson JD (1980) The syndromes of androgen resistance. Engl J Med 302:198–209

    Article  Google Scholar 

  21. Hah BD, Spranger JW (1980) Campomelic dysplasia. Further elucidation of a distinct entity. Am J Dis Child 134:285–289

    Google Scholar 

  22. Haseltine FP, Ohno S (1981) Mechanisms of gonadal differentiation. Science 211:1272–1278

    Article  PubMed  CAS  Google Scholar 

  23. Hovmüller ML, Osuna A, Eklöf O etal. (1977) Camptomehc dwarfism. A genetically determined mesenchymal disorder combined with sex reversal. Hereditas 86:51–62

    Article  Google Scholar 

  24. Imperato-McGinley J, Peterson RE (1976) Male pseudohermaphroditism: The complexities of male phenotypic development. Am J Med 61:251–272

    Article  PubMed  CAS  Google Scholar 

  25. Imperato-McGinley J, Peterson RE, Gautier T, Sturia E (1979) Androgenes and the evolution of male-gender identity among male pseudohermaphrodites with 5a-reductase deficiency. N Engl Med 300:1233–1237

    Article  CAS  Google Scholar 

  26. Isurugi K, Aso Y, Ishida H etal. (1977) Prepubertal XY gonadal dysgenesis. Pediatrics 59:569–573

    PubMed  CAS  Google Scholar 

  27. Josso N (ed) (1981) The intersex child. Karger, Basel (Pediatric and adolescent endocrinology, vol 8)

    Google Scholar 

  28. Josso N, Picard J, Tran D (1977) The anti-Muherian hormone. Birth Defects 13/2:59–84

    PubMed  CAS  Google Scholar 

  29. Kai H, Nose O, lida Y, Ono J, Harada T, Yabuuchi H (1979) Female pseudohermaphroditism caused by maternal congenital adrenal hyperplasia. J Pediatr 95:418–419

    Article  PubMed  CAS  Google Scholar 

  30. Kauffman M, Straisfeld C, Pinsky L (1976) Male pseudohermaphroditism presumably due to target organ unresponsiveness to androgens. J Chn Invest 58:345

    Article  Google Scholar 

  31. Lenz W (1964) Pseudohermaphroditismus masculinus externus mit Sekundärbehaarung und ohne Brustentwicklung (pseudovaginale, perineoskrotale Hypospadie). In: Becker PE (Hrsg) Handbuch der Humangenetik, Bd III/l. Thieme, Stuttgart, S 385–387

    Google Scholar 

  32. Müher CR, Migl B, Traupe H, Ropers HH (1980) X-hnked steroid sulfatase: Evidence for different gene-dosage in males and females. Hum Genet 54:197–199

    Article  Google Scholar 

  33. Müher U (1981) Immunological and functional aspects of H-Y antigen. Hum Genet 58:29–33

    Google Scholar 

  34. Müller U, Aschmoneit I, Zenzes MT, Wolf U (1978) Binding studies of H-Y antigen in rat tissues. Indications for a gonad specific receptor.Hum Genet 43:151–157

    Google Scholar 

  35. Ohno S (1976) Major regulatory genes for mamilian sexual development. Ceh 7:315–321

    CAS  Google Scholar 

  36. Ohno S (1979) Major sex-determining genes. Springer, Berlin Heidelberg New York (Monographs on endocrinology, vol 11)

    Book  Google Scholar 

  37. Opitz JM (1980) Comments on some genetic abnormahdes of sex determinadon and sex differendation in homo sapiens. Eur J Pediat 133:77–91

    Article  CAS  Google Scholar 

  38. Passarge E (1979) Elemente der Khnischen Genetik. Grundlagen und Anwendung der Humangenetik in Studium und Praxis. Fischer, Stuttgart

    Google Scholar 

  39. Passarge E, Wolf U (1981) Genetic heterogeneity of XY gonadal dysgenesis (Swyer syndrome): H-Y andgen-negative XY gonadal dysgenesis associated with inflammatory bowel disease. Am J Med Genet 8:437–441

    Article  PubMed  CAS  Google Scholar 

  40. Rimoin DL, Schimke RN (1971) Genedc disorders of the endocrine glands. Mosby, St Louis

    Google Scholar 

  41. Shapiro LJ, Mohandas T, Weiss R, Romeo G (1979) Non-inactivadon of an X-chromosome locus in man. Science 204:1224–1226

    Article  PubMed  CAS  Google Scholar 

  42. Silvers WK, Wachtel SS (1977) H-Y antigen: Behavior and function. Science 195:956–960

    Article  PubMed  CAS  Google Scholar 

  43. Simpson JL (1976) Disorders of sexual differendadon. Edology and chnical dehneadon. Academic Press, New York

    Google Scholar 

  44. Simpson JL (1978) True hermaphrodidsm: Edology and phenotypic consideradon. Birth Defects 14/6C:9–35

    PubMed  CAS  Google Scholar 

  45. Simpson JL, Blagowidow N, Mardn AO (1981) XY gonadal dysgenesis: Genedc heterogeneity based upon chnical observadons, H-Y antigen status and segregation analysis. Hum Genet 58:91–97

    Article  PubMed  CAS  Google Scholar 

  46. Summitt RL (1979) Genetic forms of hypogonadism in the male. Prog Med Genet 3:1–72

    Article  PubMed  CAS  Google Scholar 

  47. Wachtel SS (ed) (1981) Errors of sex determination. Hum Genet 58:1–127

    Google Scholar 

  48. Wachtel SS, Koo GC, Breg WR etal. (1976) Serologic detecdon of a Y-linked gene in XX males and XX true hermaphrodites. N Engl J Med 295:750–754

    Article  PubMed  CAS  Google Scholar 

  49. Wachtel SS, Koo GC, Breg WR etal. (1976) Serologic detecdon of a Y-hnked gene in XX males and XX true hermaphrodites. N Engl J Med 295:750–754

    Article  PubMed  CAS  Google Scholar 

  50. Wachtel SS, Koo GC, de la Chapelle A, Kallio H, Heyman JM, Miher OJ (1980) H-Y an- dgen in 46,XY gonadal dysgenesis. Hum Genet 54:25–30

    Article  PubMed  CAS  Google Scholar 

  51. Wilson JD, George FW, Griffm JE (1981) The hormonal control of sexual development. Science 211:1278–1284

    Article  PubMed  CAS  Google Scholar 

  52. Winters SJ, Wachtel SS, White BJ, Koo GC, Javadpur N, Loriaux DL, Sherins RJ (1979) H-Y andgen mosaicism in the gonad of 46,XX true hermaphrodite. N Engl J Med 300:745–749

    Article  PubMed  CAS  Google Scholar 

  53. Wolf U (1978) Zum Mechanismus der Gonadendifferenzierung. Bull Schweiz Akad Med Wiss 34:357–368

    PubMed  CAS  Google Scholar 

  54. Wolf U (1979) XY gonadal dysgenesis and the H-Y andgen. Report on 12 cases. Hum Genet 47:269–277

    Article  PubMed  CAS  Google Scholar 

  55. Wolf U (1980) Geschlechtsumkehr beim Menschen infolge genetischer Defekte im H-Y- Antigen-System. In: Spranger J, Tolksdorf M (Hrsg) Klinische Genetik in der Pädiatrie. Thieme, Stuttgart, S 20–33

    Google Scholar 

  56. Wolf U (1981) Genetic aspects of H-Y antigen. Hum Genet 58:25–28

    Article  PubMed  CAS  Google Scholar 

  57. Wolf U, Fraccaro M, Mayerova A, Hecht T, Maraschio P, Hameister H (1980) A gene controlling H-Y andgen on the X chromosome. Tentative assignment by deledon mapping to Xp223. Hum Genet 54:149–154

    Article  PubMed  CAS  Google Scholar 

  58. World Health Organizadon (1981) The effect of female sex hormones on fetal development and infant health. WHO Tech Rep Ser 657

    Google Scholar 

  59. Zenzes MT, Wolf U, Engel W (1978) Organization in vitro of ovarian ceUs into testicular structures. Hum Genet 44:333–338

    Article  PubMed  CAS  Google Scholar 

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© 1982 Springer-Verlag Berlin Heidelberg

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Passarge, E. (1982). Intersexualität. In: Stolecke, H. (eds) Endokrinologie des Kindes- und Jugendalters. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-96694-1_22

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  • DOI: https://doi.org/10.1007/978-3-642-96694-1_22

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