Changes Over Time in Sex Assignment for Disorders of Sex Development

被引：85

作者：

Kolesinska, Zofia ^{[1
]}

Ahmed, S. Faisal ^{[2
]}

Niedziela, Marek ^{[1
]}

Bryce, Jillian ^{[2
]}

Molinska-Glura, Marta ^{[1
]}

Rodie, Martina ^{[2
]}

Jiang, Jipu ^{[2
]}

Sinnott, Richard O. ^{[3
]}

Hughes, Ieuan A. ^{[4
]}

Darendeliler, Feyza ^{[5
]}

Hiort, Olaf ^{[6
]}

van der Zwan, Yvonne ^{[7
]}

Cools, Martine ^{[8
]}

Guran, Tulay ^{[9
]}

Holterhus, Paul-Martin ^{[10
]}

Bertelloni, Silvano ^{[11
]}

Lisa, Lidka ^{[12
]}

Arlt, Wiebke ^{[13
]}

Krone, Nils ^{[13
]}

Ellaithi, Mona ^{[14
,15
]}

Balsamo, Antonio ^{[16
]}

Mazen, Inas ^{[17
]}

Nordenstrom, Anna ^{[18
]}

Lachlan, Katherine ^{[19
]}

Alkhawari, Mona ^{[20
]}

Chatelain, Pierre ^{[21
]}

Weintrob, Naomi ^{[22
,23
,24
]}

机构：

[1] Poznan Univ Med Sci, Poznan, Poland

[2] Univ Glasgow, Glasgow G3 8SJ, Lanark, Scotland

[3] Univ Melbourne, Melbourne, Vic 3010, Australia

[4] Univ Cambridge, Cambridge, England

[5] Istanbul Univ, Istanbul, Turkey

[6] Univ Lubeck, Lubeck, Germany

[7] Sophia Childrens Univ Hosp, Erasmus MC, Rotterdam, Netherlands

[8] Univ Ghent, Univ Hosp Ghent, B-9000 Ghent, Belgium

[9] Marmara Univ, Istanbul, Turkey

[10] Univ Hosp Schleswig Holstein, Kiel, Germany

[11] Univ Hosp Pisa, Pisa, Italy

[12] Inst Endocrinol, Prague, Czech Republic

[13] Univ Birmingham, Ctr Endocrinol Diabet & Metab, Birmingham, W Midlands, England

[14] Ahfad Univ Women, Omdurman, Sudan

[15] Al Neelain Med Res Ctr, Khartoum, Sudan

[16] Univ Hosp Bologna, Bologna, Italy

[17] Natl Res Ctr, Cairo, Egypt

[18] Karolinska Inst, Stockholm, Sweden

[19] Univ Southampton, Southampton, Hants, England

[20] Amiri Hosp, Kuwait, Kuwait

[21] Univ Lyon 1, F-69365 Lyon, France

[22] Tel Aviv Med Ctr & Sch Med, Dana Childrens Hosp, Pediat Endocrinol & Diabet Unit, IL-64239 Tel Aviv, Israel

[23] Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, IL-69978 Tel Aviv, Israel

[24] Tel Aviv Univ, Sackler Fac Med, IL-69978 Tel Aviv, Israel

来源：

PEDIATRICS | 2014年 / 134卷 / 03期

基金：

英国医学研究理事会;

关键词：

atypical; differences; DSD; gender; genitalia; intersex; CLOACAL EXSTROPHY; GENDER ASSIGNMENT; IDENTITY; INTERSEX; MALES; 46; XY; CONSEQUENCES; CONSENSUS;

D O I：

10.1542/peds.2014-1088

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

BACKGROUND AND OBJECTIVE: It is unclear whether the proportion of infants with a disorder of sex development who are raised as male or female has changed over time. The temporal trends in sex assignment of affected cases entered in the International Disorder of Sex Development (I-DSD) Registry were studied. METHODS: Cases of disorders of sex development reported as partial androgen insensitivity syndrome (PAIS; n = 118), disorder of gonadal development (DGD; n = 232), and disorder of androgen synthesis (DAS; n = 104) were divided into those who were born before 1990, 1990-1999, and after 1999. External appearance of the genitalia was described by the external masculinization score. RESULTS: The median (5th-95th percentile) external masculinization scores of those infants with PAIS, DGD, and DAS who were raised as boys were 6 (2-9), 6 (3-9), and 6 (1-12), respectively, and were significantly higher than in those raised as girls (2 [0-6], 2 [0-7], and 0 [0-5], respectively); this difference was maintained in the 3 temporal birth cohorts (P < .01). Of the 118 cases in the pre-1990 cohort, 41 (35%) were raised as boys; 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. CONCLUSIONS: Although there is an association between the external appearance of the genitalia and the choice of sex assignment, there are clear temporal trends in this practice pointing toward an increased likelihood of affected infants being raised as boys. The impact of this change in practice on long-term health outcomes requires additional focus.

引用

页码：E710 / E715

页数：6

共 25 条

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