Cargando…

ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD

INTRODUCTION: Over recent decades, controversy regarding standards of care has contributed to questioning past management recommendations for infants and children with a 46,XY DSD. Surveys conducted in 3 waves (2003, 2010, 2020) provide evidence of changes in recommendations for care have changed ov...

Descripción completa

Detalles Bibliográficos
Autores principales: Lee, Peter, Gardner, Melissa, Kogan, Barry, Khorashed, Behzad Sorouri, Suorsa-Johnson, Kristina, Sandberg, David
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9625405/
http://dx.doi.org/10.1210/jendso/bvac150.1246
_version_ 1784822487890001920
author Lee, Peter
Gardner, Melissa
Kogan, Barry
Khorashed, Behzad Sorouri
Suorsa-Johnson, Kristina
Sandberg, David
author_facet Lee, Peter
Gardner, Melissa
Kogan, Barry
Khorashed, Behzad Sorouri
Suorsa-Johnson, Kristina
Sandberg, David
author_sort Lee, Peter
collection PubMed
description INTRODUCTION: Over recent decades, controversy regarding standards of care has contributed to questioning past management recommendations for infants and children with a 46,XY DSD. Surveys conducted in 3 waves (2003, 2010, 2020) provide evidence of changes in recommendations for care have changed over this period. METHODS: Online surveys of the members of the Pediatric Endocrine Society (PE) and Societies for Pediatric Urology (PU) included case vignettes representing partial androgen insensitivity syndrome (PAIS), micropenis resulting from primary testicular failure, and penile ablation (non-DSD). Survey items included questions regarding recommended gender of rearing (GoR), genital surgery (and timing), and disclosure of diagnostic details to patient. RESULTS: The newborn PAIS vignette included elevated LH and FSH and a 1.2 cm. phallus that increased to 1.7 after exogenous testosterone. Male GoR was chosen by 47, 55 & 55% PE and 81, 83 & 69% of PU in waves 1 to 3, respectively; 27 PE and 28% PU chose an added "non-binary" option in 2020. The recommendation that parents, rather than patients, should decide about perineal hypospadias repair was chosen by 70, 60 & 50% of PE and 93, 90 & 79% of PU. Recommending against surgery were 25, 22 & 10% PE and 23, 13 & 8% PU. Recommending surgery be completed at <1 year of age: 56, 59, 66% PE and 69, 78, 84% PU. Recommendation against disclosure of the underlying condition to the patient 12, 17 & 28% PE and 17, 19 & 22% PU, while 35, 28 to 32% PE and 34, 43 & 30% PU recommended that disclosure occur by 11 years of age. For the penile ablation vignette, 78, 92 & 92% PE and 98, 95 & 97% PU recommended male GoR. The 2020 non-binary option was chosen by 4 and 2% PE and PU. The majority of both groups recommended male GoR for the micropenis vignette with testicular failure; 94, 94 & 89% PE and 96, 97 & 88% PU. The decline in 2020 resulted from the recommendation of the non-binary option by 9% PE and 8% PU. CONCLUSIONS: It seems clear that a female GoR recommendation is currently rare across 46,XY vignettes with evidence of testicular function during at least some of fetal life. It is also apparent that the current emphasis/controversy regarding GoR has resulted in confusion as to how to apply this poorly understood concept. The resulting shift of delaying decisions regarding surgery until the patient can decide is inconsistent with the recommendation that hypospadias repair occur before age 1 year. Finally, lack of or delayed disclosure is inconsistent with patient centered care recommendations. Presentation: No date and time listed
format Online
Article
Text
id pubmed-9625405
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Oxford University Press
record_format MEDLINE/PubMed
spelling pubmed-96254052022-11-14 ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD Lee, Peter Gardner, Melissa Kogan, Barry Khorashed, Behzad Sorouri Suorsa-Johnson, Kristina Sandberg, David J Endocr Soc Pediatric Endocrinology INTRODUCTION: Over recent decades, controversy regarding standards of care has contributed to questioning past management recommendations for infants and children with a 46,XY DSD. Surveys conducted in 3 waves (2003, 2010, 2020) provide evidence of changes in recommendations for care have changed over this period. METHODS: Online surveys of the members of the Pediatric Endocrine Society (PE) and Societies for Pediatric Urology (PU) included case vignettes representing partial androgen insensitivity syndrome (PAIS), micropenis resulting from primary testicular failure, and penile ablation (non-DSD). Survey items included questions regarding recommended gender of rearing (GoR), genital surgery (and timing), and disclosure of diagnostic details to patient. RESULTS: The newborn PAIS vignette included elevated LH and FSH and a 1.2 cm. phallus that increased to 1.7 after exogenous testosterone. Male GoR was chosen by 47, 55 & 55% PE and 81, 83 & 69% of PU in waves 1 to 3, respectively; 27 PE and 28% PU chose an added "non-binary" option in 2020. The recommendation that parents, rather than patients, should decide about perineal hypospadias repair was chosen by 70, 60 & 50% of PE and 93, 90 & 79% of PU. Recommending against surgery were 25, 22 & 10% PE and 23, 13 & 8% PU. Recommending surgery be completed at <1 year of age: 56, 59, 66% PE and 69, 78, 84% PU. Recommendation against disclosure of the underlying condition to the patient 12, 17 & 28% PE and 17, 19 & 22% PU, while 35, 28 to 32% PE and 34, 43 & 30% PU recommended that disclosure occur by 11 years of age. For the penile ablation vignette, 78, 92 & 92% PE and 98, 95 & 97% PU recommended male GoR. The 2020 non-binary option was chosen by 4 and 2% PE and PU. The majority of both groups recommended male GoR for the micropenis vignette with testicular failure; 94, 94 & 89% PE and 96, 97 & 88% PU. The decline in 2020 resulted from the recommendation of the non-binary option by 9% PE and 8% PU. CONCLUSIONS: It seems clear that a female GoR recommendation is currently rare across 46,XY vignettes with evidence of testicular function during at least some of fetal life. It is also apparent that the current emphasis/controversy regarding GoR has resulted in confusion as to how to apply this poorly understood concept. The resulting shift of delaying decisions regarding surgery until the patient can decide is inconsistent with the recommendation that hypospadias repair occur before age 1 year. Finally, lack of or delayed disclosure is inconsistent with patient centered care recommendations. Presentation: No date and time listed Oxford University Press 2022-11-01 /pmc/articles/PMC9625405/ http://dx.doi.org/10.1210/jendso/bvac150.1246 Text en © The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Pediatric Endocrinology
Lee, Peter
Gardner, Melissa
Kogan, Barry
Khorashed, Behzad Sorouri
Suorsa-Johnson, Kristina
Sandberg, David
ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD
title ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD
title_full ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD
title_fullStr ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD
title_full_unstemmed ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD
title_short ODP381 Changes in Perceptions of Pediatric Endocrinologists (PE) and Urologists (PU) to Infants and Children regarding care of individuals with 46,XY DSD
title_sort odp381 changes in perceptions of pediatric endocrinologists (pe) and urologists (pu) to infants and children regarding care of individuals with 46,xy dsd
topic Pediatric Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9625405/
http://dx.doi.org/10.1210/jendso/bvac150.1246
work_keys_str_mv AT leepeter odp381changesinperceptionsofpediatricendocrinologistspeandurologistsputoinfantsandchildrenregardingcareofindividualswith46xydsd
AT gardnermelissa odp381changesinperceptionsofpediatricendocrinologistspeandurologistsputoinfantsandchildrenregardingcareofindividualswith46xydsd
AT koganbarry odp381changesinperceptionsofpediatricendocrinologistspeandurologistsputoinfantsandchildrenregardingcareofindividualswith46xydsd
AT khorashedbehzadsorouri odp381changesinperceptionsofpediatricendocrinologistspeandurologistsputoinfantsandchildrenregardingcareofindividualswith46xydsd
AT suorsajohnsonkristina odp381changesinperceptionsofpediatricendocrinologistspeandurologistsputoinfantsandchildrenregardingcareofindividualswith46xydsd
AT sandbergdavid odp381changesinperceptionsofpediatricendocrinologistspeandurologistsputoinfantsandchildrenregardingcareofindividualswith46xydsd