Gonadal dysgenesis

Gonadal Dysgenesis

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Gonadal dysgenesis

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Description:
Gonadal dysgenesis is a term used to describe multiple reproductive system development disorders. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo.This loss leads to extremely hypoplastic (underdeveloped) and dysfunctioning gonads mainly composed of fibrous tissue, hence the name streak gonads.

It originally referred to Turner syndrome, but use of the term has expanded to cover other conditions.

Pathogenesis

During embryogenesis, without any external influences for or against, the human reproductive system is intrinsically conditioned to give rise to a female reproductive organisation.As a result, if a gonad cannot express its sexual identity via its hormones—as in gonadal dysgenesis—then the affected person, no matter whether genetically male or female, will develop both internal and external female genitalia.

In both sexes, the commencement and progression of puberty require functional gonads that will work in harmony with the hypothalamic and pituitary glands to produce adequate hormones.For this reason, in gonadal dysgenesis the accompanying hormonal failure also prevents the development of secondary sex characteristics in either sex, resulting in a sexually infantile female appearance and......
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