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Gonadectomy in conditions affecting sex development: a registry-based cohort study
AK. Lucas-Herald, J. Bryce, A. Kyriakou, ML. Ljubicic, W. Arlt, L. Audi, A. Balsamo, F. Baronio, S. Bertelloni, M. Bettendorf, A. Brooke, HL. Claahsen van der Grinten, JH. Davies, G. Hermann, L. de Vries, IA. Hughes, R. Tadokoro-Cuccaro, F....
Language English Country Great Britain
Document type Journal Article
NLK
Open Access Digital Library
from 1948-06-01
Open Access Digital Library
from 1997-07-01
PubMed
33780351
DOI
10.1530/eje-20-1058
Knihovny.cz E-resources
- MeSH
- Adult MeSH
- Castration statistics & numerical data MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Disorders of Sex Development diagnosis epidemiology surgery MeSH
- Registries MeSH
- Retrospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
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.
Centre for Prenatal Diagnosis Jilin University 1st Hospital Jilin China
Department of Clinical Genetics National Research Centre Cairo Egypt
Department of Endocrinology St Albans City Hospital West Hertfordshire Hospitals Trust St Albans UK
Department of Growth and Reproduction Rigshospitalet University of Copenhagen Copenhagen Denmark
Department of Paediatric Endocrinology Faculty of Medicine University of Southampton Southampton UK
Department of Paediatric Endocrinology Royal Manchester Children's Hospital Manchester UK
Department of Paediatrics University of Cambridge Cambridge UK
Department of Pediatric Endocrinology Istanbul University Istanbul Medical School Istanbul Turkey
Department of Pediatric Endocrinology Marmara University Istanbul Turkey
Department of Pediatrics and Adolescent Medicine University Medical Centre Ulm Germany
Department of Pediatrics and Pediatric Endocrinology Medical University of Silesia Katowice Poland
Department of Pediatrics Leiden University Medical Center Leiden the Netherlands
Department of Pediatrics Otto von Guericke University Magdeburg Germany
Department of Pediatrics Radboud University Medical Center Nijmegen the Netherlands
Department of Pediatrics University Hospital Pisa Pisa Italy
Dept of Women's and Children's Health Karolinska Institutet Stockholm Sweden
Developmental Endocrinology Research Group University of Glasgow Glasgow UK
Division of Paediatrics University of Lübeck Lübeck Germany
Endocrinology Birmingham Children's Hospital Birmingham UK
Endocrinology Institute of Prague Czech Republic
Endocrinology University of Medicine and Pharmacy Craiova Craiova Romania
Endocrinology Yerevan State Medical University Endocrinology Clinic Yerevan Armenia
Faculty of Medical Laboratory Sciences Al Neelain University Khartoum Sudan
Institute of Metabolism and Systems Research University of Birmingham Birmingham UK
Macleod Diabetes and Endocrine Centre Royal Devon and Exeter Hospital Exeter UK
Paediatrics Tel Aviv Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel
Pediatric Endocrinology Clinic Zeynep Kamil Women and Children Hospital Istanbul Turkey
Pediatric Endocrinology Dana Dwek Children's Hospital Tel Aviv Medical Centre Tel Aviv Israel
Pediatric Endocrinology Service University Hospital Ghent Ghent Belgium
UMHAT 'Sveta Marina' Medical University of Varna Varna Bulgaria
References provided by Crossref.org
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- $a 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.
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