Disorders of sexual development in a cultural context

Abstract

BackgroundDisorders of sexual development (DSD) are congenital conditions in which the development of the chromosomal, gonadal or anatomical sex can be deemed atypical. The external genitalia should appear ‘normal’ in size and shape from birth, with no question of abnormality, and the individual must receive appropriate social-environmental feedback in the course of the sexual maturation process.MethodsWe review regional differences in the variables considered important for gender assignment in individuals with DSD. Various approaches to certain forms of DSD are analysed within their cultural context.ResultsThe decision to leave the sex of rearing undisturbed or to change it is difficult. It depends on the patient’s age and the extent to which the gender identity has been established with parental gender preference, social, cultural and religious factors. Severe forms of genetically female congenital adrenal hyperplasia, androgen insensitivity syndrome, 17β-hydroxysteroid dehydrogenase-3, 5α-reductase and cytochrome P450 oxidoreductase deficiencies are found to be the most difficult cases to diagnose and/or manage.ConclusionGender assignment in children with DSD is a subject of intense debate. Each case of DSD must be evaluated individually and on its merits and potentials. Although early admission and appropriate diagnostic facilities could provide the correct diagnosis, this is not the case in some cultures. It is seen that ‘gender panic’, social and religious concepts affect the decision-making process in gender assignment, especially in delayed cases.

Psychological Aspects of Androgen Insensitivity Syndrome: Two Cases Illustrating Therapeutical Challenges

“…They conceptualize their psychosexual development as a female's one and, up to now, there is no observed dissatisfaction with their assigned gender. This might be explained by the effect of androgen unresponsiveness of the brain, in addition to unambiguous female sex of rearing [13]. On the other hand, many patients with PAIS are raised as males, as in case 2; the female gender is preferred only in selected cases with severe PAIS, as in the hereby presented case 1.…”

“…In the past, the standard physicians' approach was not to reveal the whole situation either to the patient or to his relatives. Nowadays, according to relevant interview studies [7, 8, 11, 13, 14], the full or partial disclosure constitutes the first-line choice, abandoning the paternalistic approach of the past. Social and cultural factors may be important modifiers in this process, since different societies do not accept this patient-based approach.…”

Multidisciplinary Management of Disorders of Sex Development in Indonesia, A Prototype in Developing Country

“…10 The wide spectrum of the clinical presentation of DSD is another aspect, and the interval of detection from the antenatal period into childhood or adolescence is also a factor to consider. 11,12 Financial resources, health insurance issues, social, and cultural variables can also influence the attitudes of the parents for gender preference of the newborn or child with DSD. Because of National Health Insurance (JKN) cannot cover for diagnostic testing and treatment of DSD, the funds sometimes came from fund rising.…”

Disorders of sexual development: Small patients, big decisions

“…Çocukların ve ailelerinin tıbbi, cerrahi, psikolojik destek ve tedavilerinin standart bir yönte-mi yoktur, uygulanacak tedavi yöntemleri etik ve kül-türel sorgulamaya her zaman açıktır (18) . Tıbbi, cerrahi ve psikolojik desteğin sonuçları baskın olan sosyal, kültürel yapı ve dini özellikler ile yakın ilişkilidir ve gecikmiş olgularda bu özellikler ailenin kararını daha fazla etkilemektedir (19) . Bu tür hastalar için en tipik örnek, konjenital adrenal hiperplazi (KAH) nedeniyle tedavi edilen hastalardır.…”