1. Gonadectomy in conditions affecting sex development: a registry-based cohort study
- Author
-
Lucas-Herald, A.K., Bryce, J., Kyriakou, A., Ljubicic, M.L., Arlt, W., Audi, L., Balsamo, A., Baronio, F., Bertelloni, S., Bettendorf, M., Brooke, A., Claahsen-van der Grinten, H.L., Davies, J.H., Hermann, G., Vries, L. de, Hughes, I.A., Tadokoro-Cuccaro, R., Darendeliler, F., Poyrazoglu, S., Ellaithi, M., Evliyaoglu, O., Fica, S., Nedelea, L., Gawlik, A., Globa, E., Zelinska, N., Guran, T., Güven, A., Hannema, S.E., Hiort, O., Holterhus, P.M., Iotova, V., Mladenov, V., Jain, V., Sharma, R., Jennane, F., Johnston, C., Junior, G. Guerra, Konrad, D., Gaisl, O., Krone, N., Krone, R., Lachlan, K., Li, D., Lichiardopol, C., Lisa, L., Markosyan, R., Mazen, I., Mohnike, K., Niedziela, M., Nordenstrom, A., Rey, R., Skaeil, M., Tack, L.J.W., Tomlinson, J., Weintrob, N., Cools, M., Ahmed, S.F., Lucas-Herald, A.K., Bryce, J., Kyriakou, A., Ljubicic, M.L., Arlt, W., Audi, L., Balsamo, A., Baronio, F., Bertelloni, S., Bettendorf, M., Brooke, A., Claahsen-van der Grinten, H.L., Davies, J.H., Hermann, G., Vries, L. de, Hughes, I.A., Tadokoro-Cuccaro, R., Darendeliler, F., Poyrazoglu, S., Ellaithi, M., Evliyaoglu, O., Fica, S., Nedelea, L., Gawlik, A., Globa, E., Zelinska, N., Guran, T., Güven, A., Hannema, S.E., Hiort, O., Holterhus, P.M., Iotova, V., Mladenov, V., Jain, V., Sharma, R., Jennane, F., Johnston, C., Junior, G. Guerra, Konrad, D., Gaisl, O., Krone, N., Krone, R., Lachlan, K., Li, D., Lichiardopol, C., Lisa, L., Markosyan, R., Mazen, I., Mohnike, K., Niedziela, M., Nordenstrom, A., Rey, R., Skaeil, M., Tack, L.J.W., Tomlinson, J., Weintrob, N., Cools, M., and Ahmed, S.F.
- Abstract
Contains fulltext : 234031.pdf (Publisher’s version ) (Open Access), OBJECTIVES: To determine trends in clinical practice for individuals with DSD 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 to the International DSD Registry and who were over 16 years old in January 2019. RESULTS: Data regarding gonadectomy were available in 668 (88%) individuals 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 cases (53%). Females were more likely to undergo gonadectomy (n = 311, P < 0.0001). The indication for gonadectomy was reported in 268 (76%). The most common indication was mitigation of tumour risk in 172 (64%). Variations in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergone 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), respectively (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 diagnosis, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all forms of DSD will allow a better understanding of the variation in the practice of gonadectomy.
- Published
- 2021