Hermaphroditism verus Q56.01

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hermaphroditism ambiglandularis; hermaphroditism complexus; Hermaphroditism of genuine; Hermaphroditism real; true hermaphroditism

Definition
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Simultaneous presence of ovary and test tissue, independent of chromosomal sex.

Classification
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Etiopathogenesis
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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 features
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The external genitals can vary from purely female to mixed to purely male, so that the sex assignment can be difficult.

Therapy
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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.

Literature
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  1. Krob G et al (1996) Hermaphroditism: Clinic, diagnostics and treatment. Mschr paediatrics 144: 362-368
  2. 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
  3. Walker AM et al (2000) True hermaphroditism. J Paediatr Child Health 36: 69-73
  4. 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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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020