This article reviews the literature on studies and case reports on gender identity and gender identity problems, gender dysphoria, and gender change in chromosomal females with congenital adrenal hyperplasia, raised male or female. The large majority (94.8%) of the patients raised female (N= 250) later developed a gender identity as girls and women and did not feel gender dysphoric. But 13 (5.2%) patients had serious problems with their gender identity. This percentage is higher than the prevalence of female-to-male transsexuals in the general population of chromosomal females. Among patients raised male, serious gender identity problems were reported in 4 (12.1%) out of 33 patients. From these observations, we conclude that the assignment to the female gender as a general policy for 46,XX patients with CAH appears justified, even in severely masculinized 46,XX newborns with CAH (Prader stage IV or V).

Polychlorinated biphenyls (PCBs) and dioxins are known as neurotoxic compounds that may modulate sex steroid hormones. Steroid hormones play a mediating role in brain development and may influence behaviors that show sex differences, such as childhood play behavior. In this study we evaluated the effects of perinatal exposure to environmental levels of PCBs and dioxins on childhood play behavior and whether the effects showed sex differences. As part of the follow-up to the Dutch PCB/dioxin study at school age, we used the Pre-School Activity Inventory (PSAI) to assess play behavior in the Rotterdam cohort (n = 207). The PSAI assesses masculine or feminine play behavior scored on three subscales: masculine, feminine, and composite. Prenatal exposure to PCBs was defined as the sum of PCB 118, 138, 153, and 180 in maternal and cord plasma and breast milk. For breast milk we measured additional PCBs as well as 17 dioxins. Respondents returned 160 questionnaires (age 7.5 years +/- 0.4). Effects of prenatal exposure to PCBs, measured in maternal and cord plasma, on the masculine and composite scales were different for boys and girls (p <.05). In boys, higher prenatal PCB levels were related with less masculinized play, assessed by the masculine scale (p(maternal) =.042; p(cord) =.001) and composite scale (p(cord) =.011), whereas in girls higher PCB levels were associated with more masculinized play, assessed by the composite scale (p(PCBmilk) =.028). Higher prenatal dioxin levels were associated with more feminized play in boys as well as girls, assessed by the feminine scale (p =.048). These effects suggest prenatal steroid hormone imbalances caused by prenatal exposure to environmental levels of PCBs, dioxins, and other related organochlorine compounds.

In addition to procedures that bring the meatus to a more terminal position, those that bring it more ventral can be performed without negatively affecting psychosexual adjustment. Hypospadias patients were reluctant to seek advice for problems and, therefore, they should be followed through adolescence.

Purpose: We studied the degree of agreement between hypospadias patient and surgeon satisfaction with the cosmetic surgical result, and the relation between penile length, m e a d position and patient satisfaction.Materials and Methods: Cosmetic and functional results in 35 boys with hypospadias were assessed, and a standardized questionnaire was completed by patients and surgeon.Results: There was hardly any agreement between patient and surgeon satisfaction with patient penile appearance. Patients were less satisfied than the surgeon. No significant correlation was noted between penile satisfaction and penile length. Patients with a retracted meatus were less satisfied with the meatal position than those with a glanular meatus. Of the 35 patients 4 underwent repeat surgery aRer our study.Conclusions: Hypospadias surgeons should explicitly ask if patients are satisfied and they should follow patients through adolescence.KEY WORDS: penis; hypospadias; surgery, plastic Corrective surgery of hypospadias, that is reconstructing the urethra and straightening the penis, is necessary to ensure voiding in the standing position and unhampered adult sexual functioning. Another important goal of hypospadias surgery is the achievement of a cosmetic penile appearance that is as normal as possible. Others who studied long-term results of hypospadias surgery reported patient satisfaction with the penile appearance or surgeon satisfaction with the surgical result.'-6 To our knowledge only Schwobel et a1 have previously assessed hypospadias patient and surgeon satisfaction with the cosmetic r e~u l t .~ Of the 27 patients 92% judged the cosmetic result to be satisfactory or excellent and all had a satisfactory or excellent cosmetic result, as judged by Schwobel et al. 7 Unfortunately, from these data it is impossible to determine the degree of agreement between patient and surgeon satisfaction, and only the position of the urethral meatus was considered in judgingthe surgical result. These previous studies made no distinction between satisfaction with functional result and cosmetic penile appearance. It was not known if hypospadias patients were satisfied with the same aspects of penile appearance as the surgeon. It seems essential to know whether hypospadias patients and surgeons consider the same aspects of penile appearance to be important to determine if patient treatment or counseling should be adjusted. Therefore, we evaluated in a standard manner the level of agreement between hypospadias patient and surgeon satisfaction with different asof patient penile appearance, and the relation between physid ChamCtenstics (penile length and meatal position) and satisfaction with penile appearance in hypospadias patients. PATlENTS AND METHODS Patients.Of the 458 patients treated for hypospadias between 1980 and 1992 at the Sophia Children's Hospital 113 who were 9 to 18 years old at the onset of our Study Previ2Z~oronal 703ously underwent terminal procedures, which split, core or tunnel through the glans to create a true t...

The majority of hypospadias patients experienced a normal adult sex life. They were reluctant to seek advice for problems. We recommend followup of patients through adolescence.

Used standardized questionnaires to compare psychosocial functioning of 116 children and adolescents (9 to 18 years) and 73 adults (18 to 38 years) operated on for hypospadias, a congenital penile anomaly, with that of 88 and 50 age-matched comparison males, respectively, treated for an inguinal hernia. The relationships of coping with penile appearance, subject age, severity of hypospadias, number of operations, age at final surgery, and type of surgical procedure with psychosocial functioning were also investigated. Hypospadias patients did not exhibit a poorer psychosocial functioning and no significant relationships of various medical characteristics with psychosocial functioning could be discerned. Genital/body perception of hypospadias patients ages 9 to 18 years correlated positively with psychosocial functioning, albeit with low values. These findings are important for psychologists and specialists in the counseling process of hypospadias patients and their parents.

No abstract

The emotional reactions of parents and adult patients on disclosure of the clinical diagnosis of androgen insensitivity syndrome (AIS) and its later confirmation by gene mutation analysis were assessed. A semistructured interview and three questionnaires were used. Parents came from 18 different families with a total of 20 children (15 complete AIS, 5 partial AIS), 19 raised as girls, 1 as a boy. Ten adult women with complete AIS came from six families. The short-term reaction upon the clinical diagnosis was in the majority of both parents and adult patients associated with shock, grief, anger, and shame and in the mothers and adult patients with guilt. Emotional reactions were more long-lasting in mothers and adult patients than in fathers. The confirmation by DNA analysis did not alter the actual feelings of both parents. Adolescents with AIS should be informed completely – but in a step-by-step way – about their condition, since adult patients indicated that they had suffered from being not at all or misinformed about AIS in their adolescence.