Bone mineral density in complete androgen insensitivity syndrome and the timing of gonadectomy

  title={Bone mineral density in complete androgen insensitivity syndrome and the timing of gonadectomy},
  author={Thomas F J King and Winnie Zee Man Wat and Sarah M Creighton and Gerard S. Conway},
  journal={Clinical Endocrinology},
  pages={136 - 140}
Low bone mineral density (BMD) has been reported in complete androgen insensitivity syndrome (CAIS), but the impact of timing of gonadectomy is not known. We aimed to assess the relationship between age of gonadectomy and BMD in women with CAIS. 

Bone Mineral Density in Women Living with Complete Androgen Insensitivity Syndrome and Intact Testes or Removed Gonads

The maintenance of testes may represent a strategy to improve bone health in women with CAIS, but a strict follow-up to monitor the cancer risk is mandatory mainly from their 20s onwards.

Dilemmas in management of osteoporosis in patients with complete androgen insensitivity syndrome

The objective is to report a patient with CAIS status post prepubertal orchiectomy that developed early osteoporosis and to describe the lack of optimal strategies and consensus available to improve bone health in this population.

Case Report: Low Bone and Normal Lean Mass in Adolescents With Complete Androgen Insensitivity Syndrome

These limited data indicate that adolescents with CAIS have bone mass deficit, and further studies are needed to understand the extent of BMD abnormalities and the effect of gonadectomy, especially early in childhood, and to establish the optimal HRT regimen for bone accrual.

Bone mineral density, body composition and metabolic profiles in adult women with complete androgen insensitivity syndrome and removed gonads using oral or transdermal estrogens.

The results reinforce the importance of adequate hormonal treatment in women living with CAIS, suggesting a better effect from the transdermal route over the oral route.

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

Gonadal Surgery in Complete Androgen Insensitivity Syndrome: A Debate

Recommendations for gonadal removal in women living with CAIS vary in literature, and literature indications range from “the testes should be removed as soon as they are discovered” to “support leaving the testes in until after puberty has been completed and then either removing them or instituting a careful monitoring process for the early detection of seminoma".

Androgen Insensitivity Syndrome (AIS): Complete AIS (CAIS)

Bone Health in the Transgender Population

With pharmacologic testosterone, bone density in trans men remains largely unchanged although androgens have indirect effects on bone health via changes in fat and lean mass.



Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence

Adult women with complete androgen insensitivity syndrome (CAIS) are increasingly likely to defer or decline gonadectomy despite counselling about malignancy risk, and women’s reasons for deferring Gonadectomy are explored.

Osteopenia as a feature of the androgen insensitivity syndrome

Whether the adverse sequelae of androgen resistance may extend to skeletal tissue by measuring bone mineral density in six patients with androgen Insensitivity is evaluated.

The contribution of testosterone to skeletal development and maintenance: lessons from the androgen insensitivity syndrome.

It is concluded that even when compliance to exogenous estrogen use is excellent, women with complete AIS show moderate deficits in spine BMD, averaging close to 1 SD from normative means, and that with correction of BMD for bone size, skeletal deficits are magnified and include the proximal femur.

Gonadal steroid-dependent effects on bone turnover and bone mineral density in men.

Estrogens primarily regulate bone homeostasis in adult men, and testosterone and estradiol levels must decline substantially to impact the skeleton.

Comparison of bone mineral density and body proportions between women with complete androgen insensitivity syndrome and women with gonadal dysgenesis.

Taller stature in late gonadectomised CAIS women suggests an oestrogen deficiency in these women prior to gonadectomy, and increased lower to upper body ratio in GD(XY) women compared with the other groups implies that these subjects have the greatest degree of oestrogens deficiency in puberty.

Sex steroid actions in male bone.

It is proposed that estrogens influence fracture risk in aging men via direct effects on bone, whereas androgens exert an additional antifracture effect mainly via extraskeletal parameters such as muscle mass and propensity to fall.

Gonadal tumour risk in 292 phenotypic female patients with disorders of sex development containing Y chromosome or Y‐derived sequence

Evaluating tumour risk of DSD, summarize the clinical characteristics of patients with GCTs and propose management suggestions are proposed.

Genotype versus phenotype in families with androgen insensitivity syndrome.

The commonly accepted concept of dependence on fetal androgens of the development of Wolffian ducts was studied in complete androgen insensitivity syndrome (CAIS) patients and molecular observations suggest that phenotypic variation had different etiologies among these families.

The syndrome of testicular feminization in male pseudohermaphrodites.

  • J. Morris
  • Medicine
    American journal of obstetrics and gynecology
  • 1953