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Prophylactic gonadectomy is done in

A
Testicular feminising syndrome
B
Klinefelter's syndrome
C
Kalman's syndrome
D
Down's syndrome
High-Yield Explanation
Gonadectomy is often advised in CAIS due to the risk of gonadal malignancy. The malignancy risk is poorly defined (possibly 5-10% in childhood rising to 30% by the age of 50 years) and is thought to be due to intra abdominal position of the testes. Timing of gonadectomy is controversial. Some clinicians advocate early i.e. childhood gonadectomy due to the malignant potential with subsequent HRT to induce puberty. Others prefer to delay gonadectomy until after puberty to allow endogenous gonadal hormones to work throughout puberty. Hormone replacement therapy is essential after gonadectomy for many aspects of health and well being. One of these is the maintenance of bone mineral density. More on management of CAIS Majority of women with CAIS will have a shortened vagina. The hypoplastic vagina requires treatment. The two main options for vaginal enlargement are vaginoplasty surgery or manual vaginal dilatation. Vaginal dilatation is usually the first line treatment.

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