Male gender identity in children with 46,XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood

Chowdhury, Tanvir Kabir; Laila, Kamrun; Hutson, John M.; Banu, Tahmina

doi:10.1016/j.jpedsurg.2015.08.023

Cited by 22 publications

(11 citation statements)

References 15 publications

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“…Similar to our study, Khattab et al (13) report three 46,XX with 21-OHD CAH patients who reared as male gender. In another study, of the 50 DSD patients, the 4/11 cases diagnosed with 46,XX DSD due to CAH had assumed male gender role (15). This condition occurs due to prenatal and/or postnatal exposure to high levels of androgens that promote the masculinization of gender behaviors (16,50).…”

Section: Discussionmentioning

confidence: 99%

“…And this condition is the main structure of sexual identity, and it is influenced by genetic status, pre/postnatal exposure to androgens, sociocultural factors, and family dynamics (6,39,48,49). Gender assignment is an important problem in DSD patients who have virilized brain with undervirilized external genitalia (13)(14)(15)39). Eleven of 97 46,XX patients (11.3%) had male gender orientation in the psychological evaluation, and raised as male gender by parents (9 were 21-OHD, 1 was 11-OHD, and 1 was Sertoli cell only syndrome, mean age of cases was 9.92 ± 4.96 years).…”

Section: Discussionmentioning

confidence: 99%

“…The long-term physical, social and psychological outcomes of patients with DSD are still unclear. There are increasing concerns regarding early decisions about gender assignment in recent reports (13)(14)(15)(16)(17)(18). Studies were generally conducted on psychosexual and surgical outcomes of this group of patients (19)(20)(21)(22).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Gender Identity and Assignment Recommendations in Disorders of Sex Development Patients: 20 Years’ Experience and Challenges

Gürbüz

Alkan

Çelik

et al. 2020

Jcrpe

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What is already known on this topic? Gender assignment in Disorders of Sex Development (DSD) patients is always very difficult, complex and demanding experience in the management for both families and clinicians, particularly in cases where the gender appropriate for the clinical diagnosis is incompatible with the psychological gender of the patient. Gender assignment councils must have an experienced and multidisciplinary approach. What this study adds? Here, we present 20 years of experience and challenges in gender assignment, the causes and clinical characteristics of patients with DSD. This study is the longest timeframe, is the most comprehensive and has the largest number of cases in terms of gender assignment recommendation and assessing the factors affecting gender assignment from Turkey.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Gender Identity and Assignment Recommendations in Disorders of Sex Development Patients: 20 Years’ Experience and Challenges

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Çelik

et al. 2020

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“…It was forward translated by two independent translators to produce a single reconciled version and back translated and compared with the source instruments to make amendments. The questionnaire was then pre-tested, and modified to be culturally appropriate under supervision of context experts and administered to mothers of all participants [ 21 ].…”

Section: Methodsmentioning

confidence: 99%

Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

et al. 2021

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Introduction Girls with classical congenital adrenal hyperplasia (CAH) are exposed to excess fetal adrenal androgens in-utero, and often born with masculinised genitalia. They are conventionally reared as females, but show more “boyish” gender-role behaviour (GRB) and gender-identity (GI) issues in childhood and adolescence. Male-rearing is also reported mainly due to delayed treatment and/or socio-cultural factors. We compared GRB/GI in girls with CAH with healthy age matched children, and explored for associations with socio-demographic and diagnosis/treatment related factors. Methods GRB and GI were assessed using the Gender Identity Questionnaire for children (GIQC) in 27 girls with classical CAH at a specialised clinic, and compared with 50 age-matched healthy controls, with exploratory-analysis based on socio-demographic and diagnosis/treatment-related factors. Results Girls with CAH had lower total GIQC scores compared to healthy children (3.29 vs. 4.04, p = < 0.001) with lower GRB score (3.39 vs. 4.23, p < 0.001), and tendency for lower GI score (3.19 vs. 3.5, p = 0.08). Exploratory analysis showed no differences based on diagnosis/treatment factors including age, degree of virilisation at diagnosis and surgical procedures. and only subtle changes based on ethnicity and maternal education. Discussion/conclusion Girls with CAH managed at a specialised centre showed more masculinised GRB and tendency for ambiguous GI, which did not vary upon diagnosis/treatment related factors, suggesting that prenatal androgen exposure was the likely contributor. Clinicians should be vigilant about the increased risk of gender-related problems in girls with CAH, irrespective of sociocultural background and despite early treatment.

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“…These complicated problems were described thoroughly in Egyptian and Malaysian cultures (Dessouky, 2001;Kuhnle and Krahl, 2002;Zainuddin et al, 2013). Parents of children with DSD preferred a male gender assignment for their children (if that was possible) because of the social advantages of growing up male in a patriarchal society (Kulkarni et al, 2009;Gupta et al, 2010;Chowdhury et al, 2015;Ediati et al, 2015b;Joseph et al, 2016).…”

Section: Gender Assignment or Reassignmentmentioning

confidence: 99%

Sociocultural aspects of disorders of sex development

Ediati

Maharani

Utari

2016

Birth Defects Research Pt C

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Disorders of sex development (DSD) is a congenital condition in which the development of chromosomes, gonads, hormones, and reproductive structures are atypical. DSD brings with it a psychological impact on the affected individual and their families. The consensus statement on management of DSD strongly advised an integrated and multidisciplinary approach in providing care to the affected individuals. Studies have been conducted focusing on medical intervention, and more recently, there is increasing attention paid to psychological aspects of DSD. However, studies reporting cultural aspects of DSD are lacking. This review provides an overview on how culture impacts the affected individuals in coping with DSD and making decisions with regard to gender assignment or reassignment, help‐seeking behavior for medical treatments, attitudes toward medical treatment, religious beliefs, and values concerning marriage and fertility. The involvement of social scientists is needed to study sociocultural aspects of DSD from more diverse cultures, to help affected individuals and their families in gaining better social acceptance. Birth Defects Research (Part C) 108:380–383, 2016. © 2016 Wiley Periodicals, Inc.

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Male gender identity in children with 46,XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood

Cited by 22 publications

References 15 publications

Gender Identity and Assignment Recommendations in Disorders of Sex Development Patients: 20 Years’ Experience and Challenges

Gender Identity and Assignment Recommendations in Disorders of Sex Development Patients: 20 Years’ Experience and Challenges

Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

Sociocultural aspects of disorders of sex development

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