sodium-ethylxanthate and Adrenal-Hyperplasia--Congenital

Phoenix et al. (1959) reported that treating pregnant guinea pigs with testosterone had enduring effects on the sex-related behavior of their female offspring. Since then, similar enduring effects of early testosterone exposure have been found in other species, including humans, and for other behaviors that show average sex differences. In humans, the affected outcomes include gender identity, sexual orientation, and children's sex-typical play behavior. The evidence linking early testosterone exposure to sex-typed play is particularly robust, and sex-typed play is also influenced by many other factors, including socialization by parents and peers and self-socialization, based on cognitive understanding of gender. In addition to influencing behavior, testosterone and hormones produced from testosterone affect mammalian brain structure. Studies using human autopsy material have found some sex differences in the human brain similar to those seen in other species, and have reported that some brain sex differences correlate with sexual orientation or gender identity, although the causes of these brain/behavior relationships are unclear. Studies that have imaged the living human brain have found only a small number of sex differences, and these differences are generally small in magnitude. In addition, they have not been linked to robust psychological or behavioral sex differences. Future research might benefit from improved imaging technology, and attention to other brain characteristics. In addition, it might usefully explore how different types of factors, such as early testosterone exposure and parental socialization, work together in the developmental system that produces sex/gender differences in human brain and behavior.

Topics: Adrenal Hyperplasia, Congenital; Adult; Aggression; Animals; Brain; Child; Child Rearing; Empathy; Environment; Female; Forecasting; Gender Identity; Humans; Male; Neurosciences; Organ Size; Parent-Child Relations; Peer Group; Play and Playthings; Pregnancy; Prenatal Exposure Delayed Effects; Puberty; Sex; Sex Characteristics; Sexual Behavior; Spatial Navigation; Species Specificity; Testosterone

The current debate about the psychosocial management of intersex patients has significant implications for 46,XX patients with classic congenital adrenal hyperplasia (CAH) in two respects: (1) the question of genital surgery for reasons other than purely medical ones before the patient is old enough to give informed consent, and (2) the question of gender assignment in severely masculinized patients. This article reviews the status of the empirical evidence for the development of gender and sexuality in 46,XX persons with classic CAH and its implications for clinical practice.

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Adult; Child; Female; Genitalia; Humans; Infant, Newborn; Male; Sex; Sexual Behavior; Sexuality

Female patients with classic 21-hydroxylase deficiency (21-OHD) present with decreased fertility and low childbirth rates, women with a salt-wasting form of 21-OHD being most severely affected. In cases of undersubstitution with glucocorticoids, tonic androgen secretion disturbs ovulation. However, even adequately substituted females may present with apparent infertility. Despite adrenal androgen suppression, adrenal progesterone secretion can prevent thickening of the endometrium in the follicular phase. Furthermore, functional ovarian hyperandrogenism is a common finding even in women with well-controlled classic 21-OHD. Psychosexual factors may also contribute significantly to decreased childbirth rates found in these patients. Genital ambiguity may lead to a disturbed body image and the patients have been found to feel less feminine than healthy control women. The repeated psychological insult caused by frequent genital examinations and operations is also important, though its exact impact has been difficult to determine. Finally, prenatal androgen excess can cause masculinization of the central nervous system leading to boyish behavior in childhood and decreased heterosexual activity in adulthood. Some recent reports show a high rate of infertility also in men with 21-OHD. They are at risk of benign testicular tumors, adrenal rests, which can lead to permanent infertility. Also, raised adrenal androgen production leading to increased estrogen concentrations can suppress gonadotropin secretion and may lead to a hypogonadotropic state.

Topics: Adrenal Hyperplasia, Congenital; Female; Fertility; Humans; Male; Sex

Topics: Adrenal Hyperplasia, Congenital; Athletes; Disorders of Sex Development; Female; Genetic Testing; History, 20th Century; History, 21st Century; Humans; Hyperandrogenism; Intersex Persons; Male; Mutation; Polycystic Ovary Syndrome; Sex; Sex Characteristics; Sports; Testosterone

A study group has been constituted at the Hôpital des Enfants-Malades in order to analyse the relational approach with adolescent girls with various disorders and malformations of the genital tract followed since childhood. This group has been using a methodology which has already been applied to psychological aspects of other somatic diseases. Endocrinologists, gynecologists and psychoanalysts have weekly discussions about clinical cases. Their discussions are tape-recorded. The transcriptions are analysed by the whole group in order to evaluate the objective and subjective data concerning patients, families and doctors. We intend to discuss problems which appear to be the most important in relation to these disorders. Whatever the somatic problem is, these girls' future is also determined by their psychic reality and also that of their families and doctors. This psychic reality integrates and modifies the objective data as well as their environment.

Topics: Adrenal Hyperplasia, Congenital; Castration; Disorders of Sex Development; Family; Female; Genital Diseases, Female; Genitalia, Female; Humans; Ovarian Neoplasms; Physician-Patient Relations; Psychology, Adolescent; Sex

Lacking an empirically based theory of erotosexual development and health in childhood, pediatrics too easily falls back on reductionistic hypotheses of the nature versus nurture type. A new, three-term paradigm, namely, nature/critical-period/nurture, is needed to explain, for example, the phenomenology of hermaphroditism, and the differentiation of gender-identity/role (G-I/R) in individual cases. In 30 young women with a history of the early-treated, 46,XX congenital virilizing adrenal hyperplasia (CVAH) syndrome, 37% (N = 11) had a history of bisexual imagery or practice, as compared with 7% in the control patients (chi 2 = 17.7; p less than .001); and 5 of these 11 rated themselves as exclusively or predominantly lesbian. In Kinsey's sample, 15% of females reported homoerotic imagery by age 20, and 2 out of 3 of them also had homoerotic partner contact. The CVAH finding may be a function of prenatal and/or neonatal brain androgenization, but other variables, such as the history of juvenile erotosexual rehearsal play, cannot be ruled out. Among adolescents with a history of hermaphroditism, sex-reassignment applications are honored predominantly if they are made by 46,XY hermaphrodites assigned neonatally as girls and with nonfeminizing hormonal puberty, as medical and folk traditions both favor approval of such applicants more than others. Parthenogenic whiptail lizards that alternately simulate the male and female mating behavior of related diecious species provide an animal model that demonstrates the existence of both male and female sexual schemas in the same brain. In human beings, the irreducible sex differences are that males impregnate, and females menstruate, gestate, and lactate. Otherwise, sexual dimorphism that is programmed into the brain under the influence of prenatal hormones appears to be not sex-irreducible, but sex-shared and threshold-dimorphic. A complete theory of the differentiation of all the constituents of masculinity or femininity of G-I/R needs to be both multivariate and sequential in type. It must be applicable to all of the syndromes of hermaphroditism, and to the genesis of all the G-I/R phenomena, including transvestism and transsexualism, as well as to the genesis of a heterosexual G-I/R.

Topics: Adrenal Hyperplasia, Congenital; Animals; Brain; Disorders of Sex Development; Female; Gender Identity; Gonadal Steroid Hormones; Gonads; Homosexuality; Humans; Imagination; Lizards; Pediatrics; Play and Playthings; Sex; Sex Differentiation; Sexual Behavior; Virilism

A sample of 31 patients with congenital adrenal hyperplasia was matched to a control group by sex, age, height, full-scale IQ score, and urban/rural site of residence. Patients and matched controls were tested on measures of masculinity and femininity and on sex-typed tests of cognitive ability. The two groups did not differ significantly on these measures. The finding is in disagreement with earlier reports of "masculinization" of behavior in female patients with congenital adrenal hyperplasia.

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Adult; Behavior; Body Height; Child; Child, Preschool; Cognition; Female; Humans; Intelligence; Interview, Psychological; Male; Psychological Tests; Role; Self Concept; Sex; Sex Factors; Sports; Wechsler Scales

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Child; Child, Preschool; Disorders of Sex Development; Female; Humans; Male; Role; Sex; Sex Characteristics; Sex Determination Analysis; Socialization; Turner Syndrome; USSR

Topics: Adrenal Hyperplasia, Congenital; Black or African American; Child; Cortisone; Disorders of Sex Development; Environment; Female; Humans; Identification, Psychological; Mutism; Projective Techniques; Psychosexual Development; Sex; Sex Education; Speech Disorders

Topics: Adrenal Hyperplasia, Congenital; Adult; Attitude; Child Rearing; Disorders of Sex Development; Female; Humans; Identification, Psychological; Male; Mother-Child Relations; Self Concept; Sex

Topics: Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Cholesterol; Enzymes; Female; Hydrocortisone; Hypersensitivity; Infertility; Infertility, Male; Metabolism; Pre-Eclampsia; Pregnancy; Sex; Urine

Topics: Adrenal Hyperplasia, Congenital; Adrenal Insufficiency; Adrenogenital Syndrome; Character; Humans; Infant; Sex

Topics: Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Humans; Sex