Synonym(s)
Hermaphroditism ambiglandularis; hermaphroditism complexus; Hermaphroditism of genuine; Hermaphroditism real; true hermaphroditism
DefinitionThis section has been translated automatically.
Simultaneous presence of ovary and test tissue, independent of chromosomal sex.
ClassificationThis section has been translated automatically.
- Lateral form with testicles and contralateral ovaries (30% of patients); see below hermaphroditism verus lateralis.
- Bilateral form with bilateral testes and ovarian tissue (mostly as ovotestes) (20% of patients); see below Hermaphroditism verus bilateralis.
- Unilateral form with unilateral ovotestis and testicles or ovary contralateral (50% of patients); see below hermaphroditism verus lateralis.
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EtiopathogenesisThis section has been translated automatically.
Genetic defects, mostly chromosomal aberrations (47, XXY; 46, XX/46, XY; 46, XX/47, XXY) or mosaics of some cell lines in normal karyotype (mostly in 46, XX).
Clinical featuresThis section has been translated automatically.
The external genitals can vary from purely female to mixed to purely male, so that the sex assignment can be difficult.
TherapyThis section has been translated automatically.
Depending on the predominance of external gender characteristics, education to become a female or male individual (if necessary, psychological care to promote gender identification). Surgical correction of the external genitals. Depending on the location of the testes (groin or higher) surgical removal due to the risk of degeneration with lifelong substitution therapy of testosterone (initial 50 mg/month i.m. with slow increase over one year to 250 mg) in male patients from puberty. In female patients, remaining ovarian tissue should be biopsied to identify testicular parts and to rule out the development of malignancy.
LiteratureThis section has been translated automatically.
- Krob G et al (1996) Hermaphroditism: Clinic, diagnostics and treatment. Mschr paediatrics 144: 362-368
- Takano T et al (2003) True hermaphroditism with 46,X + 22p/46,XY and gonadal mosaicism detected by fluorescence in situ hybridization. Ann Genet 46: 57-60
- Walker AM et al (2000) True hermaphroditism. J Paediatr Child Health 36: 69-73
- Yordam N et al (2001) True hermaphroditism: clinical features, genetic variants and gonadal histology. J Pediatr Endocrinol Metab 14: 421-427
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Hermaphroditism; Hermaphroditism ambiglandularis; Hermaphroditism complexus; Hermaphroditism, female; Hermaphroditism, male; Hermaphroditism, real; Hermaphroditism, real; Hermaphroditism verus bilateralis; Hermaphroditism verus lateralis; Hermaphroditism verus unilateralis; ... Show allDisclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer