Hertha Richter-Appelt

Summary of Consensus Statement on Intersex Disorders and Their Management

Advances in understanding of genetic control of sexual determination and differentiation, improvements in diagnostic testing and surgical genital repair, and the persistent controversies inherent to clinical management were all compelling factors that led to the organization of an international consensus conference. The goals were to acknowledge and discuss the more controversial issues in intersex management, provide management guidelines for intersex patients, and identify and prioritize questions that need additional investigation. This is a summary statement. Advances in molecular genetic causes of abnormal sexual development and heightened awareness of the ethical and patient-advocacy issues mandate reexamination of existing nomenclature for patients with intersex.1 Terminology such as “pseudohermaphroditism” is controversial, potentially pejorative to patients,2 and inherently confusing. Therefore, the term “disorders of sex development” (DSD) is proposed to indicate congenital conditions with atypical development of chromosomal, gonadal, or anatomic sex. Additional rationale for new classification is the need for modern categorization to integrate the modern molecular genetic aspects, to maximize precision when applying definitions and diagnostic labels,3 and to meet the need for psychologically sensitive yet descriptive medical terminology. Nomenclature should be flexible enough to incorporate new information, robust enough to maintain a consistent framework, use descriptive terms, reflect genetic etiology, accommodate phenotypic variation spectrum, and be useful for clinicians, scientists, patients, and families. Hence, we propose a new classification (see “Consensus Statement on Management of Intersex Disorders”4 in this months issue of Pediatrics Electronic Edition ). Three traditionally conceptualized domains of psychosexual development are gender identity (ones self-representation [ie, male or female]), gender role (sexually dimorphic behaviors within the general population, such as toy preferences, aggression, and spatial ability), and sexual orientation (direction[s] of erotic interest). Gender dissatisfaction denotes unhappiness with assigned sex and may result in gender self-reassignment. Psychosexual developmental factors relate to parental psychopathology, parent-child … Address correspondence to Peter A. Lee, MD, PhD, Department of Pediatrics, MC-H085, Penn State College of Medicine, Milton S. Hershey Medical Center, Box 850, 500 University Dr, Hershey, PA 17033-0850. E-mail: plee{at}psu.edu

Psychiatric characteristics in transsexual individuals: multicentre study in four European countries

BACKGROUND Research into the relationship between gender identity disorder and psychiatric problems has shown contradictory results. AIMS To investigate psychiatric problems in adults fulfilling DSM-IV-TR criteria for a diagnosis of gender identity disorder. METHOD Data were collected within the European Network for the Investigation of Gender Incongruence using the Mini International Neuropsychiatric Interview - Plus and the Structured Clinical Interview for DSM-IV Axis II Disorders (n = 305). RESULTS In 38% of the individuals with gender identity disorder a current DSM-IV-TR Axis I diagnosis was found, mainly affective disorders and anxiety disorders. Furthermore, almost 70% had a current and lifetime diagnosis. All four countries showed a similar prevalence, except for affective and anxiety disorders, and no difference was found between individuals with early-onset and late-onset disorder. An Axis II diagnosis was found in 15% of all individuals with gender identity disorder, which is comparable to the general population. CONCLUSIONS People with gender identity disorder show more psychiatric problems than the general population; mostly affective and anxiety problems are found.

Deciding on Gender in Children with Intersex Conditions

Biologic factors such as genetic and hormonal influences contribute to gender identity, gender role behavior, and sexual orientation in humans, but this relationship is considerably modified by psychologic, social, and cultural factors. The recognition of biologically determined conditions leading to incongruity of genetically determined sex, somatic phenotype, and gender identity has led to growing interest in gender role development and gender identity in individuals with intersex conditions. Sex assignment of children with ambiguous genitalia remains a difficult decision for the families involved and subject to controversial discussion among professionals and self-help groups. Although systematic empirical data on outcomes of functioning and health-related quality of life are sparse, anecdotal evidence from case series and individual patients about their experiences in healthcare suggests traumatic experiences in some. This article reviews the earlier ‘optimal gender policy’ as well as the more recent ‘full consent policy’ and reviews published data on both surgical and psychosocial outcomes. The professional debate on deciding on sex assignment in children with intersex conditions is embedded in a much wider public discourse on gender as a social construction. Given that the empirical basis of our knowledge of the causes, treatment options, long-term outcomes, and patient preferences is insufficient, we suggest preliminary recommendations based on clinical experience, study of the literature, and interviews with affected individuals.

Sexual Quality of Life of Individuals with 46,XY Disorders of Sex Development

INTRODUCTION There has recently been a growing acceptance that it is not only heterosexual functioning of surgically adjusted genitalia which should be considered when measuring the treatment outcome of persons with disorders of sex development (DSD) but also their overall sexual quality of life (SexQoL). AIM A comprehensive cross-sectional investigation of SexQoL of persons with 46,XY DSD. METHODS Forty-seven persons with 46,XY DSD (age 17-60 years) were examined by means of a questionnaire on various aspects of SexQoL. Scores were compared to a nonclinical convenience sample consisting of 145 women. Data were analyzed separately for diagnostic subgroups. Furthermore, persons whose external genitalia had been surgically corrected were compared with persons whose genitalia had been left unaltered. MAIN OUTCOME MEASURES The Multidimensional Scale of Sexuality, the German Questionnaire on Feelings of Inadequacy in Social and Sexual Situations (FUSS), items on sexual dysfunctions according to DSM-IV-TR and self-constructed measures on sexual-activity history (e.g., previous sexual experience), sexual anxieties, and satisfaction with overall sex life and sexual function comprised the standardized assessment instruments. RESULTS Compared with the nonclinical group, persons with 46,XY DSD had more often no partner (P = 0.056), felt more insecure in social (Mdn(DSD) = 17.0, Mdn(comparison) = 12.0, P = 0.001) and sexual situations (Mdn(DSD) = 17.0, Mdn(comparison) = 11.0, P = 0.006), had more sexual problems (Mdn(DSD) = 4.0, Mdn(comparison) = 3.0, P = 0.001), and were less satisfied with overall sex life (Mdn(DSD) = 3.0, Mdn(comparison) = 4.0, P = 0.000) and sexual function (Mdn(DSD) = 4.0, Mdn(comparison) = 4.0, P = 0.000). Results were inconsistent with regard to sexual-activity history (e.g., previous sexual experience). Participants who underwent genital surgery showed less dyspareunia (P = 0.027) but more fear of injuries during intercourse (P = 0.019) than those whose genitals were left unaltered. CONCLUSIONS SexQoL of persons with 46,XY DSD may be impaired. Differences in SexQoL between diagnostic subgroups, effect of corrective genital surgery, and the influence of gender assignment will have to be further investigated in future studies.

Body Satisfaction and Physical Appearance in Gender Dysphoria

Gender dysphoria (GD) is often accompanied by dissatisfaction with physical appearance and body image problems. The aim of this study was to compare body satisfaction with perceived appearance by others in various GD subgroups. Data collection was part of the European Network for the Investigation of Gender Incongruence. Between 2007 and 2012, 660 adults who fulfilled the criteria of the DSM-IV gender identity disorder diagnosis (1.31:1 male-to-female [MtF]:female-to-male [FtM] ratio) were included into the study. Data were collected before the start of clinical gender-confirming interventions. Sexual orientation was measured via a semi-structured interview whereas onset age was based on clinician report. Body satisfaction was assessed using the Body Image Scale. Congruence of appearance with the experienced gender was measured by means of a clinician rating. Overall, FtMs had a more positive body image than MtFs. Besides genital dissatisfaction, problem areas for MtFs included posture, face, and hair, whereas FtMs were mainly dissatisfied with hip and chest regions. Clinicians evaluated the physical appearance to be more congruent with the experienced gender in FtMs than in MtFs. Within the MtF group, those with early onset GD and an androphilic sexual orientation had appearances more in line with their gender identity. In conclusion, body image problems in GD go beyond sex characteristics only. An incongruent physical appearance may result in more difficult psychological adaptation and in more exposure to discrimination and stigmatization.

Sexual Quality of Life of Individuals With Disorders of Sex Development and a 46,XY Karyotype: A Review of International Research

This article summarizes the current state of research on Sexual Quality of Life (SexQoL) of adults with 46,XY Disorders of Sex Development (DSD)/Intersexuality. An extensive literature search yield 21 studies published between 1974–2007, examining sexual aspects in individuals with 46,XY DSD. However, many of them lack methodological quality. The results are inconsistent but overall indicate that SexQoL of individuals with 46,XY DSD is impaired, particular with regard to sexual dysfunctions and sexual satisfaction. Future studies on SexQoL should focus more on qualitative aspects of sexuality and investigate medical and psychosocial risk factors such as sex-corrective surgery and parental bonding.

Sexual quality of life in epilepsy: Correlations with sex hormone blood levels

Seventy-nine consecutive inpatients of an epilepsy center (34 women, 45 men) who had either generalized epilepsy, temporal lobe epilepsy, focal epilepsy of other origin, or no epilepsy completed the Derogatis Interview for Sexual Function-Self-Report Inventory. Quantitative assessments of blood levels were performed for prolactin, total testosterone, sex hormone-binding globulin, estradiol, dehydroepiandrosterone sulfate, luteinizing hormone, and follicle-stimulating hormone. In men, increasing sex hormone-binding globulin levels and duration of epilepsy decreased sexual quality of life. Sex hormone-binding globulin level in men was related to enzyme-inducing antiepileptic drugs and age. In women, we found no associations between blood hormone levels and sexual quality of life. Our results suggest that sexual quality of life is affected by sexual hormone blood levels in men, but not in women with epilepsy. Avoiding enzyme-inducing antiepileptic drugs may lower the risk of raised sex hormone-binding globulin levels and, thus, of lowered sexual quality of life in men with epilepsy.

Measuring Gender Dysphoria: A Multicenter Examination and Comparison of the Utrecht Gender Dysphoria Scale and the Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults

This study examined two instruments measuring gender dysphoria within the multicenter study of the European Network for the Investigation of Gender Incongruence (ENIGI). The Utrecht Gender Dysphoria Scale (UGDS) and the Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults (GIDYQ-AA) were examined for their definitions of gender dysphoria and their psychometric properties, and evaluated for their congruence in assessing the construct. The sample of 318 participants consisted of 178 male-to-females (MtF) and 140 female-to-males (FtM) who were recruited from the four ENIGI gender clinics. Both instruments were significantly correlated in the group of MtFs. For the FtM group, there was a trend in the same direction but smaller. Gender dysphoria was found to be defined differently in the two instruments, which led to slightly different findings regarding the subgroups. The UGDS detected a difference between the subgroups of early and late onset of gender identity disorder in the group of MtFs, whereas the GIDYQ-AA did not. For the FtM group, no significant effect of age of onset was found. Therefore, both instruments seem to capture not only similar but also different aspects of gender dysphoria. The UGDS focusses on bodily aspects, gender identity, and gender role, while the GIDYQ-AA addresses subjective, somatic, social, and sociolegal aspects. For future research, consistency in theory and definition of gender dysphoria is needed and should be in line with the DSM-5 diagnosis of gender dysphoria in adolescents and adults.

Gender experience and satisfaction with gender allocation in adults with diverse intersex conditions (divergences of sex development, DSD)

The aims of this mixed-methods study were to: (1) describe the gender experience and level of satisfaction with gender allocation of intersex persons and (2) explore the spectrum of their gender identities. Of the 69 participants with a number of divergences of sex development (DSD), gender allocation at birth was female in 83% and male in 17%. Seventy-five per cent were satisfied with gender allocation. As adults, 81% lived in the female gender role, 12% in the male role and 7% chose other roles. Nine per cent reported gender change or reallocation. Twenty-four per cent reported an inclusive ‘mixed’ two-gender identity, including both male and female elements, and 3% reported a neither female nor male gender identity. Twenty-six per cent were highly uncertain about belonging to a specific gender, 14% received increased transgender scores on the gender identity questionnaire (GIQ). The dichotomous categorisation of gender fails to capture the gender experiences of a significant proportion of our participants. Uncertainty of belonging to the female or male gender category as well as non-binary identifications highlight the need for alternative gender categories. A reconsideration of the medical approach towards intersexuality, which is currently based on a binary categorisation, is discussed.

Why the factorial structure of the SCL-90-R is unstable: Comparing patient groups with different levels of psychological distress using Mokken Scale Analysis

Since its introduction, there has been a debate about the validity of the factorial structure of the SCL-90-R. In this study we investigate whether the lack of agreement with respect to the dimensionality can be partly explained by important variables that might differ between samples such as level of psychological distress, the variance of the SCL-90-R scores and sex. Three samples were included: a sample of severely psychiatrically disturbed patients (n=3078), a sample of persons with Gender Incongruence (GI; n=410) and a sample of depressed patients (n=223). A unidimensional pattern of findings were found for the GI sample. For the severely disturbed and depressed sample, a multidimensional pattern was found. In the depressed sample sex differences were found in dimensionality: we found a unidimensional pattern for the females, and a multidimensional one for the males. Our analyses suggest that previously reported conflicting findings with regard to the dimensional structure of the SCL-90-R may be due to at least two factors: (a) level of self-reported distress, and (b) sex. Subscale scores should be used with care in patient groups with low self-reported level of distress.