Gonadectomy in conditions affecting sex development: a registry-based cohort study

. 2021 May 04 ; 184 (6) : 791-801. [epub] 20210504

Jazyk angličtina Země Velká Británie, Anglie Médium electronic

Typ dokumentu časopisecké články

Perzistentní odkaz   https://www.medvik.cz/link/pmid33780351

Grantová podpora
MR/N003403/1 Medical Research Council - United Kingdom
MR/P011462/1 Medical Research Council - United Kingdom

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 Endocrinology Diabetes and Metabolism University Hospital Birmingham NHS Foundation Trust Birmingham UK

Centre for Prenatal Diagnosis Jilin University 1st Hospital Jilin China

CONICET FEI División de Endocrinología Centro de Investigaciones Endocrinológicas 'Dr César Bergadá' Buenos Aires Argentina

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 Human Development and Health Faculty of Medicine University of Southampton Southampton UK

Department of Oncology and Metabolism Academic Unit of Child Health University of Sheffield Sheffield UK

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 Cerrahpasa Cerrahpaşa Medical School Istanbul Turkey

Department of Pediatric Endocrinology Istanbul University Istanbul Medical School Istanbul Turkey

Department of Pediatric Endocrinology Marmara University Istanbul Turkey

Department of Pediatric Endocrinology Poznan University of Medical Sciences Poznan Wielkopolskie Poland

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 and Pediatric Endocrinology Unit Vall d'Hebron Research Institute Hospital Vall d'Hebron Barcelona Spain

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

Department of Pediatrics University of Bologna Hospital of Bologna Sant Orsola Malpighi Bologna Italy

Dept of Women's and Children's Health Karolinska Institutet Stockholm Sweden

Developmental Endocrinology Research Group University of Glasgow Glasgow UK

Disciplina de Endocrinologia e Metabologia Faculdade deo Ciencias Medicas da Universidade de Campinas Departamento de Pediatria e Departamento de Clinica Medica Sao Paolo Brazil

Division of Paediatric Endocrinology and Diabetes Department of Paediatrics University Hospital Heidelberg Heidelberg Germany

Division of Paediatrics University of Lübeck Lübeck Germany

Division of Pediatric Endocrinology and Diabetes Department of Pediatrics University Hospital of Schleswig Holstein Campus Kiel Christian Albrechts University of Kiel Kiel Germany

Division of Pediatric Endocrinology and Diabetology and Children's Research Center University Children's Hospital Zürich Switzerland

Division of Pediatric Endocrinology Department of Pediatrics All India Institute of Medical Sciences Ansari Nagar New Delhi India

Endocrinologie Universitatea de Medicina si Farmacie Carol Davila Facultatea de Medicine Bucharest Romania

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

Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes Schneider Children's Medical Centre of Israel Petah Tikvah Israel

Macleod Diabetes and Endocrine Centre Royal Devon and Exeter Hospital Exeter UK

Oxford Centre for Diabetes Endocrinology and Metabolism NIHR Oxford Biomedical Research Centre Churchill Hospital University of Oxford Oxford 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 Erasmus Medical Centre University Medical Centre Rotterdam Rotterdam the Netherlands

Pediatric Endocrinology Service University Hospital Ghent Ghent Belgium

Ukrainian Research Center of Endocrine Surgery Endocrine Organs and Tissue Transplantation MOH of Ukraine Kyiv Ukraine

UMHAT 'Sveta Marina' Medical University of Varna Varna Bulgaria

Unité d'Endocrinologie Diabétologie et Gynécologie de l'Enfant et de l'Adolescent Hôpital d'Enfants Casablanca Morocco

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