This review paper highlights important diagnostic and therapeutic concerns for girls with Complete Androgen Insensitivity Syndrome (CAIS). CAIS is an androgen receptor defect disorder associated with vaginal and uterine agenesis in women with a 46,XY karyotype. The major clinical issues surrounding this syndrome include timing of gonadectomy, hormone replacement, vaginal dilation, and attention...

CONTEXT Gonadectomy is avoided whenever possible in boys with 45,X/46,XY. However, no clinical markers are currently available to guide clinicians in predicting gonadal tumor risk or hormone production. OBJECTIVE The objective of the study was to test the hypothesis that gonadal histology and risk for development of a malignant germ cell tumor are reflected by the clinical presentation of a 4...

OBJECTIVES To compare bone mineral density (BMD) and body proportions between women with complete androgen insensitivity syndrome (CAIS) and with gonadal dysgenesis (GD). SETTING Adult Disorders of Sexual Development and Ovarian Failure Clinics at University College London Hospitals. DESIGN Retrospective cross-sectional study of three groups of women aged 17-58 years with varying degrees of...

The occurrence of thyroid tumors induced by A'-methyl-A'-nitrosourea (MM ) and low iodine diet in Long-Evans (LE) rats was studied with special reference to sex difference, effect of gonadectomy, and estradiol administration. Rats of experimental groups 1-6 were given i. v. injections of 40 mg of MNU/kg of body weight at SO days of age and fed on low iodine diet from 28 days of age to the end o...

Insulin resistance has been shown to be associated with increased blood pressure (BP). The sex hormones estrogen and testosterone have opposing effects in the development of increased BP. Since testosterone has been implicated in increased BP following insulin resistance, we have tried to dissect out the effects of insulin resistance on endothelium-dependent vasorelaxation in the presence and a...

OBJECTIVE Most patients with NR5A1 (SF-1) mutations and poor virilization at birth are sex-assigned female and receive early gonadectomy. Although studies in pituitary-specific Sf-1 knockout mice suggest hypogonadotropic hypogonadism, little is known about endocrine function at puberty and on germ cell tumor risk in patients with SF-1 mutations. This study reports on the natural course during p...

Patients preparing to undergo gonadotoxic medical therapy or radiation therapy or gonadectomy should be provided with prompt counseling regarding available options for fertility preservation. Fertility preservation can best be provided by comprehensive programs designed and equipped to confront the unique challenges facing these patients.

Effects of adrenal corticosteroids on reproductive and endocrine functions of the gonads are well known, but reciprocal effects of gonadal hormones on the hypothalamo-pituitary-adrenal (HPA) axis and on adrenocortical steroidogenesis in particular have received much less attention. We investigated effects of gonadectomy and testosterone (T) replacement on adrenocortical cell function in a year-...