The ratio of male to female sex assignment increased from 1.4 in the pre-1990 cohort, to 2.3 in the 1990–1999 cohort, to 6.3 in the post-1999 cohort, respectively ( = .021) (Table 1).Sufficient data were available to calculate the EMS in 72 (83%) male and 27 (87%) female cases.Of these cases, there were 580 (50%) who were suspected to have a diagnosis of PAIS ( = 172).All cases of complete androgen insensitivity in the registry had been raised as female and PAIS was therefore selected as the only diagnostic category representing a disorder of androgen action.The I-DSD registry is approved by the National Research Ethics Service of the United Kingdom, and the terminology used within the registry is based on the nomenclature initially developed at the Chicago consensus meeting and which has continued to evolve subsequently.At the time of the study in September 2013, 1167 cases had been submitted from 24 centers in 15 different countries.
The temporal trends in sex assignment of affected cases entered in the International Disorder of Sex Development (I-DSD) Registry were studied.
Affected newborns often present with atypical genitalia, and these clinical situations can often be difficult to manage, particularly when the sex of rearing is uncertain.
In the face of genital ambiguity, the issue of sex assignment has been one of the most controversial aspects in the field of DSD management.
Of the 148 cases in the 1990–1999 cohort, 60 (41%) were raised as boys; and of the 188 cases in the post-1999 cohort, 128 (68%) were raised as boys (Fig 1).
In the subgroup of 382 cases in whom the EMS could be calculated, the median (5th–95th percentile) EMSs for those raised as boys and girls were 6 (2–11) and 1 (0–6) (Of 118 cases reported as PAIS, 87 (74%) were raised as boys and 31 (26%) were raised as girls.