1. Gonadectomy in conditions affecting sex development:A registry-based cohort study
- Author
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Lucas-Herald, Angela K., Bryce, Jillian, Kyriakou, Andreas, Ljubicic, Marie Lindhardt, Arlt, Wiebke, Audi, Laura, Balsamo, Antonio, Baronio, Federico, Bertelloni, Silvano, Bettendorf, Markus, Brooke, Antonia, Van Der Grinten, Hedi L.Claahsen, Davies, Justin H., Hermann, Gloria, De Vries, Liat, Hughes, Ieuan A., Tadokoro-Cuccaro, Rieko, Darendeliler, Feyza, Poyrazoglu, Sukran, Ellaithi, Mona, Evliyaoglu, Olcay, Fica, Simone, Nedelea, Lavinia, Gawlik, Aneta, Globa, Evgenia, Zelinska, Nataliya, Guran, Tulay, Güven, Ayla, Hannema, Sabine E., Hiort, Olaf, Holterhus, Paul Martin, Iotova, Violeta, Mladenov, Vilhelm, Jain, Vandana, Sharma, Rajni, Jennane, Farida, Johnston, Colin, Guerra, Gil, Konrad, Daniel, Gaisl, Odile, Krone, Nils, Krone, Ruth, Lachlan, Katherine, Li, Dejun, Lichiardopol, Corina, Lisa, Lidka, Markosyan, Renata, Mazen, Inas, Mohnike, Klaus, Niedziela, Marek, Nordenstrom, Anna, Rey, Rodolfo, Skaeil, Mars, Tack, Lloyd J.W., Tomlinson, Jeremy, Weintrob, Naomi, Cools, Martine, Ahmed, S. Faisal, Lucas-Herald, Angela K., Bryce, Jillian, Kyriakou, Andreas, Ljubicic, Marie Lindhardt, Arlt, Wiebke, Audi, Laura, Balsamo, Antonio, Baronio, Federico, Bertelloni, Silvano, Bettendorf, Markus, Brooke, Antonia, Van Der Grinten, Hedi L.Claahsen, Davies, Justin H., Hermann, Gloria, De Vries, Liat, Hughes, Ieuan A., Tadokoro-Cuccaro, Rieko, Darendeliler, Feyza, Poyrazoglu, Sukran, Ellaithi, Mona, Evliyaoglu, Olcay, Fica, Simone, Nedelea, Lavinia, Gawlik, Aneta, Globa, Evgenia, Zelinska, Nataliya, Guran, Tulay, Güven, Ayla, Hannema, Sabine E., Hiort, Olaf, Holterhus, Paul Martin, Iotova, Violeta, Mladenov, Vilhelm, Jain, Vandana, Sharma, Rajni, Jennane, Farida, Johnston, Colin, Guerra, Gil, Konrad, Daniel, Gaisl, Odile, Krone, Nils, Krone, Ruth, Lachlan, Katherine, Li, Dejun, Lichiardopol, Corina, Lisa, Lidka, Markosyan, Renata, Mazen, Inas, Mohnike, Klaus, Niedziela, Marek, Nordenstrom, Anna, Rey, Rodolfo, Skaeil, Mars, Tack, Lloyd J.W., Tomlinson, Jeremy, Weintrob, Naomi, Cools, Martine, and Ahmed, S. Faisal
- Abstract
Objectives: To determine trends in clinical practice for individuals with D SD requiring gonadectomy. Design: Retrospective cohort study. Methods: Information regarding age at gonadectomy according to diagnosis ; reported sex; time of presentation to specialist centre; and location of centre from cases reported t o the International DSD Registry and who were over 16 years old in January 2019. Results: Data regarding gonadectomy were available in 668 (88%) individu als from 44 centres. Of these, 248 (37%) (median age (range) 24 (17, 75) years) were male and 420 (63%) (median age (range) 26 (16, 86) years) were female. Gonadectomy was reported from 36 centres in 351/668 cas es (53%). Females were more likely to undergo gonadectomy (n = 311, P < 0.0001). The indication for gonadectomy was reported in 268 (7 6%). The most common indication was mitigation of tumour risk in 172 (64%). Variatio ns in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergo ne gonadectomy before their first presentation to the specialist centre. Median age at gonadectomy of cases from high -income countries and low-/middle-income countries (LMIC) was 13.0 years (0.1, 68) years and 16.5 years (1, 28), r espectively (P < 0.0001) with the likelihood of long-term retention of gonads being higher in LMIC countries. Conclusions: The likelihood of gonadectomy depends on the underlying diagnos is, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all f orms of DSD will allow a better understanding of the variation in the practice of gonadectomy.
- Published
- 2021