Zusammenfassung
Störungen der somatosexuellen Differenzierung mit ambivalentem, intersexuellem äußerem Genitale treten bei einem von ca. 3000 bis 5000 Neugeborenen auf und konfrontieren Eltern und Behandler mit einer Fülle weitreichender Fragen: In welchem Geschlecht soll das Kind aufgezogen werden? Welche Geschlechtszuweisung hat die größte Aussicht auf Erfolg in dem Sinne, dass im weiteren Verlauf Kongruenz zwischen Geschlechtszuweisung und individueller Geschlechtsidentität besteht? Sind dafür hormonelle und/oder chirurgische Behandlungsmaßnahmen erforderlich? Wie sind diese technisch machbar? Wann sind sie optimalerweise durchzuführen? Wie werden sie vom Kind toleriert und wie wirken sie sich auf die spätere psychosexuelle Erlebnisfähigkeit und auf die Fertilität aus? Der Artikel gibt eine Übersicht über Befunde und Probleme bei der Geschlechtsidentitätsentwicklung und der psychosexuellen Erlebnisfähigkeit beim Adrenogenitalen Syndrom (AGS), beim kompletten und partiellen Androgeninsensitivitätssyndrom (cAIS und pAIS) und bei der reinen und gemischten Gonadendysgenesie (pGD und mGD). Die aktuellen Diskussionen zu Umfang und Timing genitalkorrigierender Operationen werden referiert und eigene Überlegungen zum Umgang mit den für Kind, Eltern und Behandler oft problematischen Störungsbildern vorgestellt.
Abstract
Disorders of somatosexual development that lead to ambiguous genitalia occur in one from 3,000–5,000 newborns. Parents and health care professionals are confronted with a number of crucial questions: to what sex should the child be assigned, what is the appropriate treatment in terms of hormonal and surgical interventions, when and how should these take place, and what impact do they have on the development of gender identity (GI), psychosexual well-being and fertility? This paper reviews the etiology, treatment and outcome in terms of GI and sexual health for the following syndromes: congenital adrenal hyperplasia (CAH), complete and partial androgen insensitivity (cAIS, pAIS), and pure and mixed gonadal dysgenesis (pGD, mGD). Emphasis is focussed on the current discussion involving the timing and extent of genital surgery. Finally, a procedure is introduced that covers the sexual-medical needs of patients, parents and health care professionals.
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Bosinski, H.A.G. Sexualmedizinische Aspekte bei Intersexsyndromen. Urologe 45, 981–991 (2006). https://doi.org/10.1007/s00120-006-1097-4
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DOI: https://doi.org/10.1007/s00120-006-1097-4
Schlüsselwörter
- Adrenogenitales Syndrom
- Androgeninsensitivitätssyndrom
- Gonadendysgenesie
- Geschlechtsidentität
- Genitaloperationen