Dr Schwarz questions one of the statements in our article about sex reassignment.1 He wonders if we would not raise those individuals with CAIS as females. Indeed he is correct. We would raise as girls those phenotypically female individuals with CAIS. This is in keeping with our original statement because these individuals, because of their lack of adequate or functional androgen receptors, are born without the ability to respond to androgens as typical males and thus do not have a typical XY body or nervous system. The usual prenatal androgenization of an XY nervous system is missing in these individuals and is thereby biased female. Pubertal androgen will not change this picture.2

Our recommendations for individuals with androgen insensitivity syndrome are set forth with more detail in our! Commentary in this issue of the Archives.3 We recommend that individuals with CAIS grades 1 through 3 be raised male. Those with CAIS grades 4 through 7 should be raised female. In such cases we are using the appearance of the external genitalia as a bioassay of the degree of masculinisation or femininization of the nervous system. It is not always easy to grade individuals as 3 or 4, so that becomes a difficult yet crucial distinction to make; one does the best possible.

We repeat, we have not seen reported, nor do we know of, a successfully imposed sex reassignment of a typical XY individual to live as a happy and content androphilic female. We do not recommend such an imposed sex reassignment regardless of penile condition.

 

REFERENCES

1. Diamond M, Sigmundson HK. Sex reassignment at birth: long-term review and clinical implications. Arch Pediatr Adolesc Med. 1997;151:298-304.

2. Quigley C, De Bellis A, Merschke KB, El-Awady MK, Wilson EM, French FS. Androgen receptor defects: historical, clinical and molecular perspectives. Endocr Rev. 1995;16:271-321.

3. Diamond M, Sigmundson HK. Management of intersexuality: guidelines for dealing with individuals with ambiguous genitalia. Arch Pediatr Adolesc Med. 1997;151:1046-1050.


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