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21 Cards in this Set
- Front
- Back
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what are the major groups of female genital tract malformations?
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transsexuality
gonadal dysgenesia intersexuality Mullerian duct anomalities pubertas tarda/ pubertas precox |
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what is the embryologic pathogenesis of gonadal dysgenesis?
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hypoplastic and dysfunctional so called
streak gonads |
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what form of hypogonadism is gonadal dysgenesis?
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primary --> defect lies in gonads
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what are the most common forms of gonadal dysgenesis?
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Turner syndrome 45X0
Swyer syndrome 46XY |
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what are the principal classes of gonadal dysgenesis syndromes?
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with gonosomal abberations
without gonosomal abberations = pure gonadal dysgenesis syndromes |
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what is the frequency of turner syndrome?
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1:3000 new born females
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what are the characteristics of a true hermaphrodite?
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gonads with regular testicular and ovarian parts (ovotestes)
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what is the morphology of the external genitalia in true hermaphrodites?
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often ambiguous
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what are the characteristics of male hermaphroditismus?
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testes but with female phenotype
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what are the characteristics in female hermaphroditism?
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ovary but with male phenotype
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what are the formal essentials of pseudohermaphroditism?
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phenotype of genital is inconsistent with gonadal and chromosomal sex
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what are disorders causing male pseudohermaphroditism?
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varioius enzyme defects leading to decreased hormone, ie testosterone production
missing or dysfunctional testosterone receptors --> testicular feminisation = hairless woman Swyer syndrome |
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what are disorders causing female pseudohermaphroditism?
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enzyme defects leading to increased hormone, ie androgen production --> adrenogenital syndrome
endogenous or exogenous androgen exposition |
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what are the principal groups of malformation of the Mullerian ducts?
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agenesia
aplasia atresia |
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what is the definition of agenesia?
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organ has not been created
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what is the definition of aplasia?
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organ is created but not developed
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what is the definition of atresia?
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body orifice is abnormally closed
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what is a malformation with aplasia?
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Mayer-Rokitansky-Küster-Hauser syndrome with vaginal aplasia and uterus
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how is early puberty defined?
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development of secondary sex characteristics before age 8
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how is delayed puberty defined?
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abscence of signs of pubertal developement like thelarche and pubarche until 14th and menarche until 16th year of life
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what is the main cause for delayed puberty?
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hypergonadotrop hypogonadism, ie primary ovarian insufficiency, eg Turner syndrome 45X0
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