Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report
Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder commonly caused by mutation of the CYP21A2 gene, resulting in deficiency of an enzyme required for cortisol synthesis in the adrenal cortex. In 90-95% of cases, the deficient enzyme is 21-hydroxylase (21-OH), with an incidence r...
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Indonesian Pediatric Society Publishing House
2021
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oai:doaj.org-article:9dac2396569c420187fcad545c89886f2021-11-29T02:29:22ZEthical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report0030-93112338-476X10.14238/pi61.6.2021.356-8https://doaj.org/article/9dac2396569c420187fcad545c89886f2021-11-01T00:00:00Zhttps://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/2376https://doaj.org/toc/0030-9311https://doaj.org/toc/2338-476XCongenital adrenal hyperplasia (CAH) is an autosomal recessive disorder commonly caused by mutation of the CYP21A2 gene, resulting in deficiency of an enzyme required for cortisol synthesis in the adrenal cortex. In 90-95% of cases, the deficient enzyme is 21-hydroxylase (21-OH), with an incidence ranging from 1 in 5,000 to 15,000 live births across various ethnic and racial backgrounds. In classical 21-OH deficiency (21-OHD) CAH, excessive androgen exposure in the fetus results in virilization at birth.1 The management of ambiguous genitalia in children with CAH presents a unique and ethically challenging decision-making dilemma for the medical team. Insensitive and poorly informed statements made in the delivery room may cause long-term psychological problems for the families. It is important to refrain from assigning gender until sufficient diagnostic information can be gathered. Parents, as guardians, and the supporting medical team must make decisions on behalf of the child, with the goal of enabling the child to grow into a healthy and happy adult with his or her assigned gender.2,3 We report a case of a child with CAH, focusing on the ethical challenges in management of ambiguous genitalia.Nur RochmahMuhammad FaiziAdwina Nurlita Kusuma WardhaniIndonesian Pediatric Society Publishing Housearticleethical approach; congenital adrenal hyperplasia; child; ambiguous genitalia; gender identityMedicineRPediatricsRJ1-570ENPaediatrica Indonesiana, Vol 61, Iss 6, Pp 356-8 (2021) |
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DOAJ |
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EN |
topic |
ethical approach; congenital adrenal hyperplasia; child; ambiguous genitalia; gender identity Medicine R Pediatrics RJ1-570 |
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ethical approach; congenital adrenal hyperplasia; child; ambiguous genitalia; gender identity Medicine R Pediatrics RJ1-570 Nur Rochmah Muhammad Faizi Adwina Nurlita Kusuma Wardhani Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
description |
Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder commonly caused by mutation of the CYP21A2 gene, resulting in deficiency of an enzyme required for cortisol synthesis in the adrenal cortex. In 90-95% of cases, the deficient enzyme is 21-hydroxylase (21-OH), with an incidence ranging from 1 in 5,000 to 15,000 live births across various ethnic and racial backgrounds. In classical 21-OH deficiency (21-OHD) CAH, excessive androgen exposure in the fetus results in virilization at birth.1
The management of ambiguous genitalia in children with CAH presents a unique and ethically challenging decision-making dilemma for the medical team. Insensitive and poorly informed statements made in the delivery room may cause long-term psychological problems for the families. It is important to refrain from assigning gender until sufficient diagnostic information can be gathered. Parents, as guardians, and the supporting medical team must make decisions on behalf of the child, with the goal of enabling the child to grow into a healthy and happy adult with his or her assigned gender.2,3 We report a case of a child with CAH, focusing on the ethical challenges in management of ambiguous genitalia. |
format |
article |
author |
Nur Rochmah Muhammad Faizi Adwina Nurlita Kusuma Wardhani |
author_facet |
Nur Rochmah Muhammad Faizi Adwina Nurlita Kusuma Wardhani |
author_sort |
Nur Rochmah |
title |
Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
title_short |
Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
title_full |
Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
title_fullStr |
Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
title_full_unstemmed |
Ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
title_sort |
ethical aspects of gender assignment in ambiguous genitalia - congenital adrenal hyperplasia: a case report |
publisher |
Indonesian Pediatric Society Publishing House |
publishDate |
2021 |
url |
https://doaj.org/article/9dac2396569c420187fcad545c89886f |
work_keys_str_mv |
AT nurrochmah ethicalaspectsofgenderassignmentinambiguousgenitaliacongenitaladrenalhyperplasiaacasereport AT muhammadfaizi ethicalaspectsofgenderassignmentinambiguousgenitaliacongenitaladrenalhyperplasiaacasereport AT adwinanurlitakusumawardhani ethicalaspectsofgenderassignmentinambiguousgenitaliacongenitaladrenalhyperplasiaacasereport |
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1718407662544093184 |