SFEBES2018 Poster Presentations Reproduction (23 abstracts)
Gonadectomy for adults with DSD conditions at risk of hypogonadism in the international disorders of sex development registry
Angela Lucas Herald 1 , Andreas Kyriakou 1 , Jillian Bryce 1 , Martina Rodie 1 , Carlo Acerini 2 , Wiebke Arlt 3 , Antonio Balsamo 4 , Federico Baronico 4 , Silvano Bertelloni 5 , Antonia Brooke 6 , Hedi Claahsen van der Grinten 7 , Martine Cools 8 , Feyza Darendeliler 9 , Justin H Davies 10, , An Desloovere 8 , Mona Ellaithi 12 , Simona Fica 13 , Aneta Gawlik 14 , Tulay Guran 15 , Sabine Hannema 16, , Olaf Hiort 18 , Paul-Martin Holterhus 19 , Violeta Iotova 20 , Farida Jennane 21 , Katherine Lachlan 22, , Dejun Li 23 , Lidka Lisa 24 , Inas Mazen 25 , Wilchelm Mladenov 20 , Klaus Mohnike 26 , Lavnia Nedelea 13 , Marek Niedziela 27 , Anna Nordenstrom 28 , Sukran Poyrazoglu 9 , Rodolfo Rey 29 , Rieko Tadokoro-Cuccaro 2 , Naomi Weintrob 30, & Syed Faisal Ahmed 1
1Developmental Endocrinology Research Group, School of Medicine, Dentistry and Nursing, University of Glasgow, Glasgow, UK; 2Department of Paediatrics, University of Cambridge, Cambridge, UK; 3Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK; 4Pediatric Unit, Center for Rare Endocrine Diseases (CARENDO BO), S. Orsola-Malpighi University Hospital, Bologna, Italy; 5Pediatric and Adolescent Endocrinology, University Hospital Pisa (S.B.), Pisa, Italy; 6Macleod Diabetes and Endocrine Centre, Royal Devon and Exeter Foundation Trust, Exeter, UK; 7Radboudumc Amalia Children’s Hospital, Nijmegen, Netherlands; 8Department of Pediatric Endocrinology, Ghent University Hospital, Ghent University, Ghent, Belgium; 9Istanbul Faculty of Medicine, Pediatric Endocrinology Unit, Istanbul, Turkey; 10Department of Paediatric Endocrinology, Southampton Children’s Hospital, Southampton, UK; 11Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UK; 12Faculty of Medical Laboratory Sciences, Al-Neelain University, Khartoum, Sudan; 13Department of Endocrinology, Carol Davila University of Medicine, Bucharest, Romania; 14Medical University of Silesia, School of Medicine, Katowice, Poland; 15Marmara University, Marmara, Turkey; 16Department of Pediatric Endocrinology, Erasmus Medical Centre, Sophia Children’s Hospital, Rotterdam, Netherlands; 17Department of Pediatrics, Leiden University Medical Centre, Leiden, Netherlands; 18Division of Paediatric Endocrinology and Diabetes, Department of Paediatrics, University of Luebeck, Luebeck, Germany; 19Christian-Albrechts-University of Kiel and University Hospital of Schleswig-Holstein, Kiel, Germany; 20Medical University of Varna, Varna, Bulgaria; 21Unité d’Endocrinologie/Diabétologie et Gynécologie de l’Enfant et de l’Adolescent, Hôpital d’Enfants, Casablanca, Morocco; 22Wessex Clinical Genetics Service, University Hospitals Southampton, Southampton, UK; 23Centre for Prenatal Diagnosis, The First Hospital of Jilin University, Jilin, China; 24University Hospital Motol, Prague, Czech Republic; 25Clinical Genetic Department, National Research Center, Cairo, Egypt; 26Otto-von-Guericke University, Magdeburg, Germany; 27Department of Pediatric Endocrinology and Rheumatology, Poznan University of Medical Sciences, Poznan, Poland; 28Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden; 29Hospital de Niños R. Gutiérrez, Buenos Aires, Argentina; 30Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel; 31Dana Dwek Children’s Hospital, Tel Aviv Medical Center, Tel Aviv, Israel.
Introduction: Disorders of Sex Development (DSD) can be associated with an increased risk of germ cell tumours depending on the underlying diagnosis. To date however knowledge regarding the indications and timing of gonadectomy is lacking.
Methods: The I-DSD Registry was interrogated for anonymised information regarding the diagnosis, karyotype, sex of rearing and timing of gonadectomy, if undertaken, of all individuals of any karyotype who were over the age of 16 years at the time of search and who had one of the following disorders that may lead to long-term hypogonadism: androgen action, androgen synthesis; gonadal dysgenesis; Leydig cell hypoplasia; persistent Müllerian duct syndrome or a non-specific disorder of undermasculinisation.
Results: At the time of search in January 2017, 2,141 cases were accessible on the I-DSD Registry. A total of 614 of these (57%) met the study inclusion criteria. Data regarding gonadectomy was available in 520 (85%). The cases were registered from 30 different centres in 21 different countries, over 4 continents. 158 (30%) (median age 24 years, range 17–72 years) of these individuals were currently registered male. 362 (70%) individuals were currently registered as females (median age 28 years, range 16–90 years). Gonadectomy was performed in 315 (61%) cases. There was no statistically significant difference in timing of gonadectomy (P=0.09) between males and females with a median age at time of gonadectomy of 14 (range 0.3–68) years in females and 5 (range 0.1–54) in males. The most common indication for gonadectomy was complete androgen insensitivity syndrome in females (n=194, 68%) and partial gonadal dysgenesis in males (n=15, 50%), with females more likely to have gonadectomy than males for all conditions.
Conclusions: The I-DSD Registry contains a large number of young adults who are at risk of germ cell tumours and provides an opportunity to investigate current trends in gonadectomy internationally.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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