Pseudohermaphroditism masculinusQ56.1
Synonym(s)
testicular hermaphroditism
DefinitionThis section has been translated automatically.
Incomplete masculinization in individuals with normal male genotype.
Occurrence/EpidemiologyThis section has been translated automatically.
Prevalence: 1/20.000 inhabitants.
EtiopathogenesisThis section has been translated automatically.
Partial or complete androgen resistance of the terminal organs (caused by heterogeneous genetic defects). Reduced testosterone production due to disturbance of biosynthesis. Disturbance in the conversion of testosterone into its active metabolite 5-DHT as a result of a defect in the 5alpha-reductase gene.
TherapyThis section has been translated automatically.
- Problem "sex assignment": refer the patient to the appropriate centre (e.g. Andrological Department of the University Dermatology Clinic Giessen).
- Pronounced enzyme defect: Education as a girl, early removal of the tests, estrogen substitution from puberty on are recommended.
- Patients with more male genitals: Education as a boy, surgical correction of hypospadias, in puberty examination of the need for substitution.
- There is an increased risk of degeneration of the intra-abdominal tests in case of testicular feminization, then surgery if necessary.