Pure gonadal dysgenesis will be diagnosed in the presence of::
1. Bilateral streak gonads.
2. Bilateral dysgenetic gonads.
3. One side streak and other dygenetic gonads.
4. One side streak and other normal looking gonad.
1. Bilateral streak gonads.
Reference
Harrison 15th Edition Page 2176
Quality
Spotter
Status
Repeat
QTDF
Harrison
Discussion
Few conditions that are favourite for examiners:
- Turners Syndrome
- Gonadal dysgenesis is characterized by
- primary amenorrhea,
- sexual infantilism,
- short stature,
- multiple congenital anomalies,
- and bilateral streak gonads in phenotypic women with any of several defects of the X chromosome.
- Mixed gonadal dysgenesis
- in which a unilateral testis and a contralateral streak gonad may be present;
- Pure gonadal dysgenesis
- in which bilateral streak gonads are associated with a
- normal 46,XX or 46,XY karyotype,
- normal stature, and
- primary amenorrhea; and
- Noonan syndrome,
- an autosomal dominant disorder in both sexes characterized by
- webbed neck,
- short stature,
- congenital heart disease,
- cubitus valgus, and
- other congenital defects despite
- normal karyotypes and
- normal gonads
Explanation
Self Explanatory
Comments
In Pure Gonadal Dysgenesis, the gonads are not developed and the Female Phenotype is due to the failure of Gonadal development
Tips
Chromosomal Aberrations and Variations in Sexual Development are hot topics
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Source of this Question: RxPG AIPG 2004
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