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Abstract:
Purpose: To expose a pathophysiological
and clinical classification of the anomalies in the sex development more
appropriate for the moment and current knowledge after analyzing the processes
of sex determination and sex differentiation in the human sex development.
Methods: Revision of the topic
“Anomalies in the Sex Development” as presented in books on Obstetrics and
Gynecology and search in Medline, Google Scholar and references in articles of
all the papers related to Desorders of Sex Development published in recent
years.
Synthesis of data: Human sex development
can be divided into two different processes:1) sex determination; and 2) sex
differentiation, which takes place through hormones produced by gonads information
or once formed. In this review, the elements included in both sex determination
(chromosomal sex, genetic sex and gonadal sex) and posterior sex
differentiation (hormonal sex, gonophoric sex, phenotypic sex and others) are
analyzed, also considering all embryological aspects, proposing a new
nomenclature and exposing later in detail a pathophysiological and clinical
classification of the anomalies in the human sex development (ASDs).
Conclusions: New terms and a
pathophysiological and clinical classification for the ASDs are proposed.
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