Hormonal Therapies for Individuals with Intersex Conditions

  title={Hormonal Therapies for Individuals with Intersex Conditions},
  author={Garry L. Warne and Sonia R Grover and Jeffrey D. Zajac},
  journal={Treatments in Endocrinology},
Hormonal therapy forms part of the treatment of every intersex condition. For some conditions, such as salt-wasting congenital adrenal hyperplasia, hormonal replacement therapy is life saving because hormones necessary for survival (cortisol and aldosterone) are replaced. In contrast, other hormones such as androgens or mineralocorticoids are secreted in excessive amounts in congenital adrenal hyperplasia due to an enzyme imbalance, and the role of hormonal therapy is to suppress the unwanted… 
Different Clinical Presentations and Management in Complete Androgen Insensitivity Syndrome (CAIS)
Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive genetic disorder resulting from maternally inherited or de novo mutations involving the androgen receptor gene, situated in
Disorders of sex development
Even with greater understanding of the genetic causes of disorders of sex development, the complex management of these patients must be individualized, considering all aspects, informing as age-appropriate the parents and patient.
Clinical and molecular aspects of androgen insensitivity.
  • O. Hiort
  • Biology, Medicine
    Endocrine development
  • 2013
The identification of mutations in the AR gene in patients with androgen insensitivity is variable, and chances are lower the more subtle the phenotype is, so other currently unknown mechanisms must be hypothesized to lead to the respective phenotype.
Gender dysphoria associated with disorders of sex development
Patients with simple virilizing congenital adrenal hyperplasia, as well as those with CAH and severe virilization, are less likely to have psychosexual disorders than patients with other types of DSD, and early surgery seems to be a safe option for most of these patients.
Aromatase inhibitors in pediatrics
Although aromatase inhibitors appear effective in increasing adult height of boys with short stature and/or pubertal delay, safety concerns, including vertebral deformities, a decrease in serum HDL cholesterol levels and increase of erythrocytosis, are reasons for caution.
Issues in the Long-Term Management of Adolescents and Adults with DSD: Management of Gonads, Genital Reconstruction, and Late Presentation of the Undiagnosed DSD
The surgeon who once dealt primarily with DSD in infancy must be cognizant of the ever-changing guidelines and opinions related to the various disorders and engage the adolescent and young adult in the options available and the informed consent process.
Gender Incongruity in a Person with 46,XY and Complete Androgen Insensitivity Syndrome Raised as a Female.
The patient has undergone bilateral orchiectomy and has initiated treatment with topical testosterone and bisphosphonates and has yet to evaluate the effects and decide the best therapy taking into account that he has a male gender identity but complete androgen insensitivity syndrome.
Early assessment of ambiguous genitalia
Not all intersex conditions are apparent at birth (for example, complete androgen insensitivity may only become apparent in a child with a testis within an inguinal hernia, or at puberty with primary amenorrhoea and lack of androgen hair), only those presenting with genital ambiguity at birth will be considered in this article.
Gender assignment and medical history of individuals with different forms of intersexuality: evaluation of medical records and the patients' perspective.
The majority of participants had genital surgery to correct the appearance of their genitalia and/or to enable sexual functioning and the diagnostic groups differ not only in amount and invasiveness of experienced surgical and medical treatment but also in the subjective and retrospective evaluation of the treatment measures and in the amount of reported psychological distress.


Use, misuse and abuse of androgens
There is no convincing evidence that, in the absence of proven androgen deficiency, androgen therapy is effective and safe for older men per se, in men with chronic non‐gonadal disease, or for treatment of nonspecific symptoms.
Use of Androgens and Oestrogens in Adolescents - A Review of Hormone Replacement Treatment
  • M. Zacharin
  • Medicine
    Journal of pediatric endocrinology & metabolism : JPEM
  • 2000
Paediatricians are increasingly involved in new forms of care such as that of survivors of oncological treatment, where pubertal progress is complicated by growth patterns and bone quality compromised by irradiation and chemotherapy.
Gender Change from Female to Male in Classical Congenital Adrenal Hyperplasia
The most plausible factors contributing to cross-gender identity development in these patients appeared to be neither a particular genotype or endocrinotype nor a sex-typing bias on the part of the parents but a combination of a gender-atypical behavioral self-image, aGender- atypical body image, and the development of erotic attraction to women.
Psychosexual functioning in women with complete testicular feminization: is androgen replacement therapy preferable to estrogen?
It is found that androgen therapy is preferable to estrogen in gonadectomized women with complete testicular feminization with regard to psychosexual functioning, but not on coitus, masturbation, sexual thoughts, scores on desire for bodily contact, and on attention for physical appearance.
Prenatal diagnosis and treatment of congenital adrenal hyperplasia and consequences in adults.
  • B. Spiliotis
  • Medicine, Biology
    Journal of pediatric endocrinology & metabolism : JPEM
  • 2001
Prenatal dexamethasone treatment does not seem to have an adverse effect on growth, psychological development, or school performance except for increased shyness, internalizing and decreased social behavior; animal studies, however, have shown abnormalities in the fetal brain and behavior of the animals.
Long-term perspectives for 46,XY patients affected by complete androgen insensitivity syndrome or congenital micropenis.
Most patients with congenital micropenis, whether raised male or female, were satisfied with their gender and the appearance and function of the genitalia as judged by both physicians and subjects.
Androgen resistance associated with a qualitative abnormality of the androgen receptor and responsive to high dose androgen therapy.
It is postulate that the impairment of androgen receptor function was overcome in part by the large dose of androgens, making it possible to undertake surgical correction of the hypospadias.
Treatment of hypogonadal adolescent boys with long acting subcutaneous testosterone pellets
Long acting subcutaneous testosterone pellets are safe, efficacious, well tolerated, and convenient, and result in normal physical growth and improved psychological outlook in adolescent hypogonadal boys.
Changes in bone mineral density after orchidectomy and hormone replacement therapy in individuals with androgen insensitivity syndrome.
Results indicate that both oestrogen and androgen play an important role in balancing BMD in men and that androgen plays anImportant role in bone mineralization in 46,XY individuals.