Gonadal Dysgenesis, 46,XX [gonadální dysgeneze karyotypu 46,XX]

topical
2
Terms

čistá forma gonadální dysgeneze typu 46,XX
dysgeneze gonád typu 46,XX
gonády - dysgeneze, 46,XX

 

Gonadal Dysgenesis, 46, XX
Gonadal Dysgenesis, XX Type
Pure Gonadal Dysgenesis, 46, XX
Pure Gonadal Dysgenesis, 46,XX

Persistent link   https://www.medvik.cz/link/D023961
Definition

The 46,XX gonadal dysgenesis may be sporadic or familial. Familial XX gonadal dysgenesis is transmitted as an autosomal recessive trait and its locus was mapped to chromosome 2. Mutation in the gene for the FSH receptor (RECEPTORS, FSH) was detected. Sporadic XX gonadal dysgenesis is heterogeneous and has been associated with trisomy-13 and trisomy-18. These phenotypic females are characterized by a normal stature, sexual infantilism, bilateral streak gonads, amenorrhea, elevated plasma LUTEINIZING HORMONE and FSH concentration.

DUI
D023961 MeSH Browser
CUI
M0368823
Previous indexing
Sex Differentiation Disorders (1966-1979); Gonadal Dysgenesis (1980-2001)
History note
2002
Public note
2002

C Diseases
C12.050.351.875 Urogenital Abnormalities 145
C12.050.351.875.253 Disorders of Sex Development 231
C12.050.351.875.253.064 46, XX Disorders of Sex Development 20
C12.050.351.875.253.064.249 Gonadal Dysgenesis, 46,XX 2
C12.050.351.875.253.064.500 Hyperandrogenism 107
C12.050.351.875.253.309 Gonadal Dysgenesis 59
C12.050.351.875.253.309.193 Gonadal Dysgenesis, 46,XX 2
C12.050.351.875.253.309.388 Gonadal Dysgenesis, 46,XY 22
C12.050.351.875.253.309.391 Gonadal Dysgenesis, Mixed 9
C12.050.351.875.253.309.631 Sexual Infantilism 3
C12.050.351.875.253.309.872 Turner Syndrome 272
C12.200.706.316 Disorders of Sex Development 231
C12.200.706.316.064.249 Gonadal Dysgenesis, 46,XX 2
C12.200.706.316.064.500 Hyperandrogenism 107
C12.200.706.316.309 Gonadal Dysgenesis 59
C12.200.706.316.309.193 Gonadal Dysgenesis, 46,XX 2
C12.200.706.316.309.388 Gonadal Dysgenesis, 46,XY 22
C12.200.706.316.309.391 Gonadal Dysgenesis, Mixed 9
C12.200.706.316.309.631 Sexual Infantilism 3
C12.200.706.316.309.872 Turner Syndrome 272
C12.800.316.064.249 Gonadal Dysgenesis, 46,XX 2
C12.800.316.064.500 Hyperandrogenism 107
C12.800.316.309 Gonadal Dysgenesis 59
C12.800.316.309.193 Gonadal Dysgenesis, 46,XX 2
C12.800.316.309.388 Gonadal Dysgenesis, 46,XY 22
C12.800.316.309.391 Gonadal Dysgenesis, Mixed 9
C12.800.316.309.631 Sexual Infantilism 3
C12.800.316.309.872 Turner Syndrome 272
C16.131.939.316 Disorders of Sex Development 231
C16.131.939.316.064.249 Gonadal Dysgenesis, 46,XX 2
C16.131.939.316.064.500 Hyperandrogenism 107
C16.131.939.316.309 Gonadal Dysgenesis 59
C16.131.939.316.309.193 Gonadal Dysgenesis, 46,XX 2
C16.131.939.316.309.388 Gonadal Dysgenesis, 46,XY 22
C16.131.939.316.309.391 Gonadal Dysgenesis, Mixed 9
C16.131.939.316.309.631 Sexual Infantilism 3
C16.131.939.316.309.872 Turner Syndrome 272
C19.391.119.064.249 Gonadal Dysgenesis, 46,XX 2
C19.391.119.064.500 Hyperandrogenism 107
C19.391.119.309 Gonadal Dysgenesis 59
C19.391.119.309.193 Gonadal Dysgenesis, 46,XX 2
C19.391.119.309.388 Gonadal Dysgenesis, 46,XY 22
C19.391.119.309.391 Gonadal Dysgenesis, Mixed 9
C19.391.119.309.631 Sexual Infantilism 3
C19.391.119.309.872 Turner Syndrome 272

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