Gonadectomy In Conditions Affecting Sex Development – A Registry-Based Cohort Study

in European Journal of Endocrinology
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  • 1 A Lucas-Herald, Developmental Endocrinology Research Group, University of Glasgow, Glasgow, G51 4TF, United Kingdom of Great Britain and Northern Ireland
  • 2 J Bryce, Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom of Great Britain and Northern Ireland
  • 3 A Kyriakou, Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom of Great Britain and Northern Ireland
  • 4 M Ljubicic, Department of Growth and Reproduction, Rigshospitalet, Kobenhavn, Denmark
  • 5 W Arlt, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom of Great Britain and Northern Ireland
  • 6 L Audí, Department of Pediatrics and Pediatric Endocrinology Unit, Hospital Vall d´Hebron, Barcelona, Spain
  • 7 A Balsamo, Pediatrics, University of Bologna Hospital of Bologna Sant'Orsola-Malpighi Polyclinic, Bologna, Italy
  • 8 F Baronio, Department of Medical and Surgical Sciences, SOrsola-Malpighi University Hospital, Bologna, Italy
  • 9 S Bertelloni, Department of Pediatrics, University Hospital Pisa, Pisa, Italy
  • 10 M Bettendorf, Division of Paediatric Endocrinology and Diabetes, Department of Paediatrics, University Hospital Heidelberg, Heidelberg, Germany
  • 11 A Brooke, Macleod Diabetes and Endocrine Centre, Exeter,, Royal Devon and Exeter Hospital, Exeter, United Kingdom of Great Britain and Northern Ireland
  • 12 H Claahsen-van der Grinten, Pediatrics, Radboud University Medical Center, Nijmegen, Netherlands
  • 13 J Davies, Faculty of Medicine, Paediatric Endocrinology, University of Southampton, Southampton, United Kingdom of Great Britain and Northern Ireland
  • 14 G Hermann, Department of Pediatrics and Adolescent Medicine, University Medical Center, Ljubljana, Slovenia
  • 15 L de Vries, The Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes, Schneider Children’s Medical Center of Israel, Petah-Tikva, Israel
  • 16 I Hughes, Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom of Great Britain and Northern Ireland
  • 17 R Tadokoro-Cuccaro, Paediatrics, University of Cambridge, Cambridge, United Kingdom of Great Britain and Northern Ireland
  • 18 F Darendeliler, Pediatric Endocrinology, Istanbul University Istanbul Medicine School, Istanbul, Turkey
  • 19 S Poyrazoglu, Department of Pediatrics, Pediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey
  • 20 M Ellaithi, Faculty of Medical laboratory Sciences, Al-Neelain University, Khartoum, Sudan
  • 21 O Evliyaoglu, Department of Pediatric Endocrinology, Istanbul University- Cerrahpasa, Istanbul, Turkey
  • 22 S Fica, Endocrinologie, Universitatea de Medicina si Farmacie Carol Davila Facultatea de Medicina, Bucuresti, Romania
  • 23 L Stejereanu, Endocrinologie, Universitatea de Medicina si Farmacie Carol Davila Facultatea de Medicina, Bucuresti, Romania
  • 24 A Gawlik, Department of Pediatrics and Pediatric Endocrinology, Medical University of Silesia, Katowice, Poland
  • 25 E Globa, Department of Pediatric Endocrinology, MoH of Ukraine, Kiev, Ukraine
  • 26 N Zelinska, Department of Pediatric Endocrinology, MoH of Ukraine, Kiev, Ukraine
  • 27 T Guran, Department of Pediatric Endocrinology, Marmara University, Istanbul, Turkey
  • 28 A Güven, Pediatric Endocrinology, Goztepe Educational Hospital, Istanbul, Turkey
  • 29 S Hannema, Pediatric Endocrinology, Erasmus Medical Center, Rotterdam, Netherlands
  • 30 O Hiort, Department of Paediatrics, University of Lübeck, Lübeck, Germany
  • 31 P Holterhus, Division of Pediatric Endocrinology and Diabetes, Department of Pediatrics, University Hospital of Schleswig-Holstein, Campus Kiel/Christian-Albrechts University of Kiel, Kiel, Germany
  • 32 V Iotova, UMHAT Sveta Marina, Medical University of Varna, Varna, Bulgaria
  • 33 V Mladenov, UMHAT Sveta Marina, Medical University of Varna, Varna, Bulgaria
  • 34 V Jain, Division of Paediatric Endocrinology, All India Institute of Medical Sciences, New Delhi, India
  • 35 R Sharma, Department of Paediatrics, All India Institute of Medical Sciences, New Delhi, India
  • 36 F Jennane, Unite de Endocrinologie/Diabetologie et Gynecologie des Enfants et des Adolescents, Hôpital d’Enfants, Casablanca, Morocco
  • 37 C Johnston, Department of Endocrinology, St Albans City Hospital Gynaecology Department, St Albans, United Kingdom of Great Britain and Northern Ireland
  • 38 G Guerra-Junior, Departamento de Pediatria e Departamento de Clínica Médica, Disciplina de Endocrinologia e Metabologia, Faculdade de Ciências Médicas da Universidade de Campinas, Campinas, Brazil
  • 39 D Konrad, Division of Pediatric Endocrinology and Diabetology and Children’s Research Center, University Children’s Hospital, Zurich, Switzerland
  • 40 O Gaisl, Department of Pediatric Endocrinology and Diabetology,, University Children's Hospital Zürich, Zurich, Switzerland
  • 41 N Krone, Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield, United Kingdom of Great Britain and Northern Ireland
  • 42 R Krone, Endocrinology, Birmingham Children's Hospital, Birmingham, United Kingdom of Great Britain and Northern Ireland
  • 43 K Lachlan, Wessex Clinical Genetics Service, Princess Anne Hospital, Southampton, United Kingdom of Great Britain and Northern Ireland
  • 44 D Li, Centre for Prenatal Diagnosis, Jilin University First Hospital, Changchun, China
  • 45 C Lichiardopol, Endocrinology, University of Medicine and Pharmacy Craiova, Craiova, Romania
  • 46 L Lisá, Endocrinology, Institute of Endocrinology, Prague 1, Czech Republic
  • 47 R Markosyan, Endocrinology, YSMU, Yerevan, Armenia
  • 48 I Mazen, Department of Clinical Genetics, National Research Center, Cairo, Egypt
  • 49 K Mohnike, Department of Pediatrics, Otto von Guericke Universitat Magdeburg, Magdeburg, Germany
  • 50 M Niedziela, Department of Pediatric Endocrinology, Poznan University of Medical Sciences, Poznan, Poland
  • 51 A Nordenstrom, Dept of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
  • 52 R Rey, Centro de Investigaciones Endocrinológicas "Dr. César Bergadá" (CEDIE), CONICET-FEI-División Endocrinología, Hospital de Niños R Gutiérrez, Buenos Aires, Argentina
  • 53 M Skae, Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, United Kingdom of Great Britain and Northern Ireland
  • 54 L Tack, Pediatric Endocrinology Service, University Hospital Ghent, Gent, Belgium
  • 55 J Tomlinson, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, United Kingdom of Great Britain and Northern Ireland
  • 56 N Weintrob, pediatric endocrinology, Dana-Dwek Children's Hospital, Tel Aviv, Israel
  • 57 M Cools, Pediatrics, University Hospital Ghent, Ghent, Belgium
  • 58 S Ahmed, Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom of Great Britain and Northern Ireland

Correspondence: S Ahmed, Email: faisal.ahmed@glasgow.ac.uk
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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 center; and location of center 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 centers. 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 centers 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 centers, 17 (5%) at 9 centers had undergone gonadectomy before their first presentation to the specialist center. Median age at gonadectomy of cases from high income countries and low/middle income countries (LMIC) was 13.0 yrs (0.1, 68) years and 16.5 yrs (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.

 

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