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Dreger and co-authors raise the interesting question of whether the current clinical
classification of infants and children with disorders of sexual differentiation
is appropriate (male or female and pseudohermaphroditism or true hermaphroditism
- a division initially based on gonadal histology.) They indicate that this terminology
is misleading, does not take into account current scientific information concerning
genetic and hormonal regulation of sexual differentiation, and is potentially
harmful to patient, family, and physician. The authors urge abandonment of the
diagnostic terms male and female and hermaphroditism and pseudohermaphroditism
because of the potential adverse and confusing implications such terms may have
for the patient and family (and physician). They support the development of a
taxonomy that is more scientifically based, does not imply gender assignment
or identity, and “..does not simply dictate therapy.” In reply to
these criticisms, Houk and colleagues present a classification of intersex disorders
based on chromosomal karyotype and specific cause of a disorder. They recognize
that perhaps any classification of intersex disorders may become “autocratic” and
support the primacy of individual assessment and management.
Dreger
AD, Chase C, Sousa A, Gruppuso PA, Frader J.. Changing the nomenclature/taxonomy
for intersex: a scientific and clinical rationale. J Pediatr Endocrinol Metab.
2005;18729 - 18733.
Houk CP, Lee PA, Rapaport R. Intersex classification scheme: A response to
the call for a change. J Pediatr Endocrinol Metab. 2005;18:735 - 738.
Editor’s Comment: The questions posed
by Dreger et al concerning classification of intersex disorders are important.
It would have been of interest had the authors offered an alternative classification.
However, that the classification has survived 125 years of use indicates
its usefulness. As Houk et al pointed out, the taxonomic system was developed
as a clinical guide to the establishment of an etiological diagnosis not
for selection of therapy or gender assignment. While the current taxonomy
is imperfect and incomplete, it nevertheless offers a relatively simple starting
point at which to begin to identify the cause of the problem and to guide
but not dictate management of an individual intersex patient. What Houk et
al propose is likely to be a useful addition to our thinking about these
disorders. In addition, they have listed guidelines for the management of
intersex patients after the diagnosis has been established. For tables of
the etiology and classification of intersex and guidelines for post-diagnostic
management of patients with intersex refer to the original paper by Houk
and colleagues.
Allen W. Root, MD
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Current GGH - Vol. 24 No. 1
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