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0702 Biopsychosocialerkek Yalanc.Indd Türk Psikiyatri Dergisi 2007; 18(2) Turkish Journal of Psychiatry Biopsychosocial Variables Associated With Gender of Rearing in Children With Male Pseudohermaphrodi sm Runa USLU, Didem ÖZTOP, Özlem ÖZCAN, Savaş YILMAZ, Merih BERBEROĞLU, Pelin ADIYAMAN, Murat ÇAKMAK, Efser KERİMOĞLU, Gönül ÖCAL Abstract Objective: The effect of parental rearing on gender identity development in children with ambiguous genitalia remains controversial. The present study aimed to address this issue by investigating the factors that may be associated with sex of rearing in children with male pseudohermaphroditism. Method: The study included 56 children with male pseudohermaphroditism that were consecutively referred to a child psychiatry outpatient clinic. At the time of referral the age range of the sample was 6 months-14 years; 28 children had been raised as boys and 28 as girls. Demographic and biological information was obtained from patient charts. An intersex history interview was administered to the children and parents, whereas The Gender Identity Interview and the Draw-A-Person Test were administered only to the children. The children were observed during free play. Comparisons of biological, psychological and social variables were made with respect to gender of rearing. Results: More children reared as boys were younger at time of referral, belonged to extended families, and had higher Prader scores. Although children’s gender roles were appropriate for their gender of rearing, findings of the Gender Identity Interview and the Draw-A-Person Test suggested that some of the girls presented with a male or neutral gender self-perception. Conclusion: The relationships between age at the time of problem identification, age at the time of diagnosis, and gender of rearing indicate the importance of taking measures to ensure that the intersex condition is identified at birth and children are referred for early diagnosis, gender assignment, and treatment. Key Words: Children, intersex, ambiguous genitalia, rearing, gender identity, gender roles INTRODUCTION ducts of Wolf and Muller. Genital development has a female foundation, and special factors are needed to in- From the moment of birth, the child’s sex is one of hibit the Mullerian ducts and virilize the internal and the most important factors determining parental child- rearing attitudes. Child-rearing attitudes can differ across external genitalia to achieve male development. For genders, in parallel to the values of a culture and society. the development of the testes, the SRY gene on the Y Parents learn the sex of their children from the people chromosome is needed. The ovarian-determining genes who help them with birth such as doctors or midwives. in humans are unclear at present; however, it has been The sex announced at time of a healthy child’s birth is found that DAX1 and WNT4 are 2 genes that antago- the first step in determining how the individual’s life long nize testes development. Estrogens are not required for gender identity will be perceived (Ahmed et al., 2004). the development of the female phenotype, whereas high In children with ambiguous genitalia the ambiguity may levels of androgens are needed for male differentiation. be first noticed at birth or at a later period in their lives. Testosterone and dihydrotestosterone can only function Ambiguous genitalia are conditions in which there by binding to their specific receptors at the target organ. are abnormalities in the development of the external In light of the afore-mentioned processes, the conditions genital organs, causing uncertainty about the sex of the that cause ambiguous genitalia can be classified as fol- baby (Hughes, 2002). Genital organs stem from the lows: Runa Uslu MD., e-mail: [email protected] 1 I. Conditions that cause virilization in girls til the problem is recognized and diagnosed, and the sex is assigned. During this time, which is filled with uncer- 1. The effect of fetal androgens: Congenital adrenal tainties, our impression is that families have difficulties hyperplasia and placental aromatase deficiency; in embracing the sex of the child and in reflecting and 2. The effect of maternal androgens: Adrenal tumors, reinforcing the gender through their attitudes. and ovarian tumors; This study, based on the observations mentioned II. Conditions that cause undervirilization in boys above, was planned to find answers to 2 questions within (male pseudohermaphroditism [MPH]) a sample of male pseudohermaphroditism (MPH) cases from Turkey: 1. Abnormality in testicular development: Gonadal dysgenesis and XO/XY mosaicism; 1. Is sex of rearing mainly dependent on the sex an- nounced at birth, or are there other related biopsychoso- 2. Deficiencies of androgen biosynthesis and me- cial variables? tabolism: 17 β-OH-dehydrogenase deficiency and 5α -reductase deficiency. 2. What are the timings of the recognition of the problem, diagnosis, and sex assignment? 3. Resistance to androgens: Partial androgen resist- ance; complete androgen resistance. METHODS III. True Hermaphroditism Sample: The study sample (n= 56) consisted of all MPH cases referred by the Ankara University Depart- 1. Presence of both testicular and ovarian tissue: XX, ment of Pediatric Endocrinology to the same Universi- XY, and XX/XY. ty’s Child and Adolescent Psychiatry Outpatient Clinic These etiological factors cause virilization in the gen- for evaluation of gender identity development between ital organs of both girls and boys, though to different 1990 and 2005. The purpose of the consultation was to degrees. Many classification systems used to describe the present an opinion to the Board of Ethics for Sex As- virilization of the external genital organs in children with signment about both the child’s and the family’s psy- ambiguous genitalia have been developed. For example, chosocial characteristics, and which sex was the closest according to the widely recognized Prader classification position held by the child in terms of gender identity, system, the degree of virilization ranges from phenotypic gender roles, and sexual orientation. The age range of the female with mild clitoromegaly (stage 1) to phenotypic children was 6 months-14 years (mean: 6.17 years; SD male with glandular hypospadias (Stage 5) (Ogilvy-Stu- = 4.50 years). At the time of referral, 28 of the children art and Brain, 2004). had been reared as girls and 28 had been reared as boys. Development of gender identity in children with am- Materials: A standard evaluation procedure was car- biguous genitalia is an important dimension of the prob- ried out for each ambiguous genitalia case that was re- lem. The optimal gender policy approach, dominant in ferred to our clinic for consultation. The children were the literature and pioneered by John Money (1957), administered assessments that were appropriate for their claims that parental attitude is the determining factor in age and level of development. The data obtained from the child’s gender development. In recent years, longitu- these evaluations conducted at time of referral to our dinal studies have shown that parental attitudes are not clinic were used in the present study. Demographic and enough to maintain the gender identity determined in biological data and sex assignment decisions of the board infancy (Creighton and Liao, 2004). Although limited of ethics were obtained from patient charts. in number, the studies that enable this discussion stem 1. History Interview: A semi-structured interview from the databases built in western countries where the form was used for all cases to record the patient’s his- problem is generally recognized at birth and the etiology tory of ambiguous genitalia. The form gathered demo- is determined in early infancy. graphic data, such as the child’s age, place of residence For example, in a longitudinal study by Slijper et al., (urban/rural, residing within Ankara/outside of Ankara), (1998) it was observed that the process of sex assignment age of parents, education level of the parents (illiterate, was completed no later than the end of the first year. primary education, high school, or university), occupa- Based on information gathered from families of children tion of parents (unemployed, worked on monthly wages, assessed at our clinic, a considerable time may elapse un- or worked as free lance); family structure (nuclear family, 2 Table I. Medical History Characteristics of Children With MPH Reared Male of Female. Male Female X2 P Date of referral 0.18 nonsignificant Up to1997 9 14 1998 and later 19 14 Age at referral 0.32 < 0.02 0-4 years 17 8 ≥ 5 years 11 20 Time of recognition 0.47 < 0.001 At birth 22 9 After birth 6 19 Sex announced at birth 0.82 < 0.001 Male 26 3 Female 2 25 Age at diagnosis 0.49 < 0.002 Birth 14 2 0-4 years 8 19 ≥ 5 years 6 7 Reason of delay 0.16 nonsignificant No delay 10 7 Due to family 7 11 Due to physician 11 10 extended family) and disease history, including date of ness of his/her condition were investigated along with referral, sex of rearing that was practiced until the time other mental and functional characteristics of the child. of the referral, age of the child when the problem was For statistical purposes, aiming to differentiate between recognized and diagnosed, if there was a delay in either the earlier and recent referrals, 2 time periods, 1990- referral or diagnosis, the reasons behind this delay (no 1997 and 1998-2005, each 8 years in duration, were delay, delay caused by health care professionals or delay described. caused by the family), and the age of the child at time of Even though gender identity develops early, the per- sex assignment. Information regarding the child’s gen- ception of gender constancy and maintenance is possible der roles (choice of toys and play, choice of friends in around the fifth year of life (Hetherington and Parke, terms of sex, and preferences for household chores) was 1987).
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