The XY female child.

نویسنده

  • C J Dewhurst
چکیده

It has been known for some years that apparently female patients are sometimes found to have an XY sex chromosome complement. Morris (1953) focused attention upon the syndrome of testicular feminization in which, despite the presence of XY sex chromosomes and testes, the phenotype and secondary sexual development are female. Harnden and Stewart (1959) applied the term pure gonadal dysgenesis to an XY patient of female phenotype, without secondary sexual development; many others have since been described; a condition with some similarities has been described in association with an XY karyotype and gonadal absence (agonadism (Overzier, 1963)). A further category constitutes those patients who have predominantly female genitalia but with some degree of masculinization and who show male manifestations at puberty (partial testicular failure). If the patient is seen for the first time about 16 or 17 years of age or more, the distinction between these conditions will not be difficult to draw from the presence or absence, nature and extent of the secondary sexual changes. If, however, the patient shown to be XY is a child with female, or predominantly female, external genitalia it becomes a matter of considerable importance to decide if secondary sexual development will occur and if it will be female or male in type. If a male type puberty seems likely, it must be prevented, since it can hardly fail to have important psychological effects upon a child who has been brought up in the female sex. This article deals with the management of such a problem.

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عنوان ژورنال:
  • Archives of disease in childhood

دوره 45 242  شماره 

صفحات  -

تاریخ انتشار 1970