Surgical Practice in Girls with Congenital Adrenal Hyperplasia: An International Registry Study.
| Title: | Surgical Practice in Girls with Congenital Adrenal Hyperplasia: An International Registry Study. |
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| Authors: | Hebenstreit D; Department of Urology, Hanusch Krankenhaus, Vienna, Austria.; Ahmed SF; Developmental Endocrinology Research Group, Royal Hospital for Children, University of Glasgow, Glasgow, United Kingdom.; Krone N; University of Sheffield, Sheffield, United Kingdom.; Krall C; Center for Medical Statistics, Informatics, and Intelligent Systems, Section for Medical Statistics, Medical University of Vienna, Vienna, Austria.; Bryce J; Developmental Endocrinology Research Group, Royal Hospital for Children, University of Glasgow, Glasgow, United Kingdom.; Alvi S; Paediatric Endocrinology, Leeds Children's Hospital, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom.; Ortolano R; Department of Medical and Surgical Sciences, Pediatric Unit, S.Orsola-Malpighi University Hospital, Bologna, Italy.; Lima M; Department of Medical and Surgical Sciences, Pediatric Surgery Unit, S.Orsola-Malpighi University Hospital, Bologna, Italy.; Birkebaek N; Department of Paediatrics, Aarhus University Hospital, Aarhus, Denmark.; Bonfig W; Department of Pediatrics TU Munich, Munich, Germany.; Klinikum Wels-Grieskirchen, Wels-Grieskirchen, Austria.; Claahsen van der Grinten H; Department of Paediatric Endocrinology, Radboud University Amalia Childers Hospital Medical Centre, Nijmegen, The Netherlands.; Costa EC; Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, UFRGS, Porto Alegre, Brazil.; Poyrazoglu S; Istanbul Faculty of Medicine, Department of Paediatrics, Paediatric Endocrinology Unit, Istanbul University, Istanbul, Turkey.; de Vries L; The Jesse and Sara Lea Shafer Institute of Endocrinology and Diabetes, Schneider Tel Aviv Children's Medical Center of Israel, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Flück CE; Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics and Department of BioMedical Research, Bern University Hospital Inselspital, University of Bern, Bern, Switzerland.; Guran T; Marmara University, Department of Pediatric Endocrinology and Diabetes, Pendik, Istanbul, Turkey.; Bugrul F; Marmara University, Department of Pediatric Endocrinology and Diabetes, Pendik, Istanbul, Turkey.; Güven A; Saglik Bilimleri University Medical Faculty, Zeynep Kamil Women and Children Hospital, Pediatric Endocrinology Clinic, Istanbul, Turkey.; Iotova V; Department of Paediatrics, Medical University-Varna, UMHAT 'Sv. Marina', Varna, Bulgaria.; Koehler B; Klinik für Pädiatrische Endokrinologie und Diabetologie, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.; Schröder JT; Klinik für Pädiatrische Endokrinologie und Diabetologie, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.; Konrad D; Division of Paediatric Endocrinology and Diabetology, University Children's Hospital, Zurich, Switzerland.; Gevers E; Department of Paediatric Endocrinology, Barts Health NHS Trust, Royal London Hospital, London, United Kingdom.; Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, United Kingdom.; Krone R; Birmingham Women's & Children's Hospital, Department for Endocrinology & Diabetes, Birmingham, United Kingdom.; Milenkovic T; Department of Endocrinology, Mother and Child Health Care Institute of Serbia 'Dr Vukan Čupić', Belgrade, Serbia.; Vieites A; Centro de Investigaciones Endocrinológicas, División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina.; Ross R; University of Sheffield, Sheffield, United Kingdom.; Tadokoro Cuccaro R; Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom.; Hughes I; Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom.; Acerini C; Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom.; Springer A; Department of Paediatric Surgery, Medical University Vienna, Vienna, Austria. |
| Source: | Sexual development : genetics, molecular biology, evolution, endocrinology, embryology, and pathology of sex determination and differentiation [Sex Dev] 2021; Vol. 15 (4), pp. 229-235. Date of Electronic Publication: 2021 Jul 21. |
| Publication Type: | Journal Article; Research Support, Non-U.S. Gov't |
| Language: | English |
| Journal Info: | Publisher: S. Karger Country of Publication: Switzerland NLM ID: 101316472 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1661-5433 (Electronic) Linking ISSN: 16615425 NLM ISO Abbreviation: Sex Dev Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: Basel ; New York : S. Karger, 2007- |
| MeSH Terms: | Adrenal Hyperplasia, Congenital*/surgery; Female ; Humans ; Registries ; Urogenital Surgical Procedures |
| Abstract: | In this article international trends in surgical practice in girls with congenital adrenal hyperplasia (CAH) are evaluated. All cases that had been classified in the I-CAH/I-DSD registry as 46,XX CAH and who were born prior to 2017 were identified. Centers were approached to obtain information on surgical decision making. Of the 330 included participants, 208 (63.0%) presented within the first month of life, and 326 (98.8%) cases were assigned female. Genital surgery had been performed in 250 (75.8%). A total of 64.3, 89.2, and 96.8% of cases residing in Europe, South America and Asia, respectively, had at least one surgery. In a logistic regression model for the probability of surgery before the second birthday (early surgery) over time an increase of probability for early vaginal surgery could be identified, but not for clitoral surgery or both surgeries combined. Genitoplasty in girls with CAH remains controversial. This large international study provides a snapshot of current practice and reveals geographical and temporal differences. Fewer surgeries were reported for Europe, and there seems to be a significant trend towards aiming for vaginal surgery within the first 2 years of life.; (© 2021 The Author(s). Published by S. Karger AG, Basel.) |
| Grant Information: | G1100236 United Kingdom MRC_ Medical Research Council |
| Contributed Indexing: | Keywords: Congenital adrenal hyperplasia; Disorders of sexual development; Intersex; Surgery |
| Entry Date(s): | Date Created: 20210805 Date Completed: 20220314 Latest Revision: 20250530 |
| Update Code: | 20260130 |
| DOI: | 10.1159/000517055 |
| PMID: | 34350892 |
| Database: | MEDLINE |
Journal Article; Research Support, Non-U.S. Gov't