TY - JOUR
T1 - Morbidity, Mortality, and Socioeconomics in Females With 46,XY Disorders of Sex Development
T2 - A Nationwide Study
AU - Berglund, Agnethe
AU - Johannsen, Trine H
AU - Stochholm, Kirstine
AU - Viuff, Mette H
AU - Fedder, Jens
AU - Main, Katharina M
AU - Gravholt, Claus H
PY - 2018/4/1
Y1 - 2018/4/1
N2 - Context: Little is known about long-term health outcomes in phenotypic females with 46,XY disorders of sex development (XY females), and the socioeconomic profile has not been described in detail.Objective: To describe morbidity, mortality, and socioeconomic status in XY females in a comparison to the general population.Design: Nationwide registry study with complete follow-up.Setting: Uniform public health care system.Participants: A total of 123 XY females karyotyped in Denmark during 1960 to 2012 and a randomly selected age-matched control cohort of 12,300 females and 12,300 males from the general population.Main Outcome Measures: Overall mortality and morbidity as well as cause-specific morbidity; medicine use and socioeconomics (education, income, cohabitation, motherhood, and retirement).Results: Compared with female controls, overall morbidity was increased in XY females [hazard ratio (HR), 1.72; 95% confidence interval (CI), 1.43 to 2.08] but not when excluding diagnoses associated with the specific disorder of sex development (DSD) diagnosis or pregnancy and birth (HR, 1.13; CI, 0.93 to 1.37). Mortality was similar to controls (HR, 0.79; CI, 0.35 to 1.77). Cohabitation (HR, 0.44; CI, 0.33 to 0.58) and motherhood (HR, 0.10; CI, 0.05 to 0.18) were reduced in XY females but education (HR, 0.92; CI, 0.61 to 1.37) was similar to controls. Income was higher than among controls in the older years.Conclusions: Morbidity was not increased in XY females when excluding diagnoses associated to the DSD condition per se. Judged on education and income, XY females perform well in the labor market. However, DSD seems to impact on the prospects of family life.
AB - Context: Little is known about long-term health outcomes in phenotypic females with 46,XY disorders of sex development (XY females), and the socioeconomic profile has not been described in detail.Objective: To describe morbidity, mortality, and socioeconomic status in XY females in a comparison to the general population.Design: Nationwide registry study with complete follow-up.Setting: Uniform public health care system.Participants: A total of 123 XY females karyotyped in Denmark during 1960 to 2012 and a randomly selected age-matched control cohort of 12,300 females and 12,300 males from the general population.Main Outcome Measures: Overall mortality and morbidity as well as cause-specific morbidity; medicine use and socioeconomics (education, income, cohabitation, motherhood, and retirement).Results: Compared with female controls, overall morbidity was increased in XY females [hazard ratio (HR), 1.72; 95% confidence interval (CI), 1.43 to 2.08] but not when excluding diagnoses associated with the specific disorder of sex development (DSD) diagnosis or pregnancy and birth (HR, 1.13; CI, 0.93 to 1.37). Mortality was similar to controls (HR, 0.79; CI, 0.35 to 1.77). Cohabitation (HR, 0.44; CI, 0.33 to 0.58) and motherhood (HR, 0.10; CI, 0.05 to 0.18) were reduced in XY females but education (HR, 0.92; CI, 0.61 to 1.37) was similar to controls. Income was higher than among controls in the older years.Conclusions: Morbidity was not increased in XY females when excluding diagnoses associated to the DSD condition per se. Judged on education and income, XY females perform well in the labor market. However, DSD seems to impact on the prospects of family life.
KW - Adolescent
KW - Adult
KW - Case-Control Studies
KW - Castration/methods
KW - Child
KW - Child, Preschool
KW - Denmark/epidemiology
KW - Female
KW - Gonadal Dysgenesis, 46,XY/drug therapy
KW - Humans
KW - Infant
KW - Infant, Newborn
KW - Male
KW - Morbidity
KW - Neoplasms/epidemiology
KW - Retirement
KW - Socioeconomic Factors
KW - Young Adult
U2 - 10.1210/jc.2017-01888
DO - 10.1210/jc.2017-01888
M3 - Journal article
C2 - 29165629
SN - 0021-972X
VL - 103
SP - 1418
EP - 1428
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 4
ER -
