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The External Genitalia Score (EGS): A European multicenter validation study

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  • Saskia van der Straaten
  • Alexander Springer
  • Aleksandra Zecic
  • Doris Hebenstreit
  • Ursula Tonnhofer
  • Aneta Gawlik
  • Malgorzata Baumert
  • Kamila Szeliga
  • Sara Debulpaep
  • An Desloovere
  • Lloyd Tack
  • Koen Smets
  • Malgorzata Wasniewska
  • Domenico Corica
  • Mariarosa Calafiore
  • Marie Lindhardt Ljubicic
  • Alexander Siegfried Busch
  • Anders Juul
  • Anna Nordenström
  • Jon Sigurdsson
  • Christa E Flück
  • Tanja Haamberg
  • Stefanie Graf
  • Sabine E Hannema
  • Katja P Wolffenbuttel
  • Olaf Hiort
  • S Faisal Ahmed
  • Martine Cools
Vis graf over relationer

CONTEXT: Standardized description of external genitalia is needed in the assessment of children with atypical genitalia.

OBJECTIVES: To validate the External Genitalia Score (EGS), to present reference values for preterm and term babies up to 24 months and correlate obtained scores with anogenital distances (AGDs).

DESIGN, SETTING: A European multicenter (n = 8) validation study was conducted from July 2016 to July 2018.

PATIENTS AND METHODS: EGS is based on the external masculinization score but uses a gradual scale from female to male (range, 0-12) and terminology appropriate for both sexes. The reliability of EGS and AGDs was determined by the interclass correlation coefficient (ICC). Cross-sectional data were obtained in 686 term babies (0-24 months) and 181 preterm babies, and 111 babies with atypical genitalia.

RESULTS: The ICC of EGS in typical and atypical genitalia is excellent and good, respectively. Median EGS (10th to 90th centile) in males < 28 weeks gestation is 10 (8.6-11.5); in males 28-32 weeks 11.5 (9.2-12); in males 33-36 weeks 11.5 (10.5-12) and in full-term males 12 (10.5-12). In all female babies, EGS is 0 (0-0). The mean (SD) lower/upper AGD ratio (AGDl/u) is 0.45 (0.1), with significant difference between AGDl/u in males 0.49 (0.1) and females 0.39 (0.1) and intermediate values in differences of sex development (DSDs) 0.43 (0.1). The AGDl/u correlates with EGS in males with typical genitalia and in atypical genitalia.

CONCLUSIONS: EGS is a reliable and valid tool to describe external genitalia in premature and term babies up to 24 months. EGS correlates with AGDl/u in males. It facilitates standardized assessment, clinical decision-making and multicenter research.

OriginalsprogEngelsk
TidsskriftThe Journal of clinical endocrinology and metabolism
Vol/bind105
Udgave nummer3
ISSN0021-972X
DOI
StatusUdgivet - 1 mar. 2020

Bibliografisk note

© Endocrine Society 2019. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

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