A review was made to understand how plasma binding protein might influence sex-hormone action in target tissues. Steroids are predominately bound to plasma proteins and only unbound steroids enter the cells. Sex-hormone-binding globulin (SHBG) binds to both the main circulating steroid T and E2 but changes in SHBG concentrations exert significant results. Increased SHBG levels increase estrogen production and decreases T activity; whereas increased androgens increase T action and inhibit SHBG production. These disturbances in hormone maintenance may lead to abnormal adult sex differentiation such as hirsutism and forms of hynaecomastia. By developing SHBG concentration measurement methods-responses of hirsutism to glucocorticoid or estrogem may be assessed. In addition the effect of thyroid hormones on SHBG may also have therapeutic implications in endocrine disease.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-2265.1974.tb03298.x

Sex-hormone-binding globulin.

An extensive literature dealing with studies on the pathophysiology of the polycystic ovary syndrome (PCO) has accumulated over the years. The great clinical and biochemical variability of PCO, as indicated in several excellent reviews over a decade ago (Goldzieher & Axelrod, 1963; Mahesh & Greenblatt, 1964; Jeffcoate, 1963; Shearman & Cox, 1966), continues as a major problem in defining this syndrome. Further, the pathogenesis of this reversible disorder remains elusive. However, recent advances in our understanding of ovarian physiology, the pathways for androgen-oestrogen secretion and metabolism, and the integrated function of the hypothalamic-hypophyseal-ovarian axis permit a rational description of the pathophysiology of the PCO syndrome. Thus, current evidence regarding the basis for the dysfunction of ovarian steroidogenesis and follicular maturation, and the aberration of gonadotropin secretion as related to the inappropriate steroid feedback system will be reviewed. The long debated issue of the possible role of adrenal androgen in the genesis and persistence of PCO syndrome will also be considered.

Review article: the polycystic ovary syndrome

Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders in which there is a deficiency of one of the enzymes necessary for cortisol synthesis. An abnormality in each of the enzymatic activities required for cortisol synthesis has been described. As a result of the disordered enzymatic step, there is decreased cortisol synthesis, increased ACTH via a negative feedback system, overproduction of the hormones prior to the enzymatic step or not requiring the deficient enzyme, and deficiency of the hormones distal to the disordered enzymatic step. Since several of the enzymatic steps are required for sex hormone synthesis by the gonad, a disordered enzymatic step in the gonad resulting in gonadal steroid hormone deficiency may also be present. This chapter presents an overview of all of the enzymatic deficiencies resulting in CAH with the most extensive review of 21-hydroxylase deficiency which is the most common, first described, and most intensively studied of the enzymatic disorders.

https://www.nejm.org/doi/pdf/10.1056/NEJMra021561

Congenital adrenal hyperplasia

1 Adrenal and gonadal androgen secretion in normal females

Polycystic ovarian disease

Testosterone production rates were found to be elevated in 13 consecutive hirsute women, confirming previous observations. To determine the origin of the excessive testosterone, percutaneous bilateral adrenal and ovarian vein catheterizations were performed, and effluent blood samples obtained for androgen measurement. Although physical examination and retrograde dye injections failed to reveal ovarian enlargement in any of these patients, testosterone and androstenedione concentrations were higher in ovarian effluents vs. adrenal venous samples. The relative contributions of adrenal and ovarian secretion, and precursor conversion to the total testosterone production rate, were estimated in each patient from kinetic considerations and by relating the effluent concentrations of testosterone and androstenedione to the cortisol secretion rate. From such calculations, it is apparent that, in 9 of the 13 women studied, the major source of androgen was ovarian (direct ovarian secretion of testosterone ...

Combined Ovarian and Adrenal Vein Catheterization To Determine the Site(s) of Androgen Overproduction in Hirsute Women

Two 19-year-old patients had bilateral anorchia, normal male phenotype and 46, XY karyotype, spontaneous but incomplete pubescence and eunuchoidal appearance, with high urinary excretion o...

https://www.nejm.org/doi/pdf/10.1056/NEJM197001292820503?listPDF=true

Bilateral anorchia with persistent testosterone production.

The volume of the sella turcica in acromegalic patients was measured before and after radiation therapy. Changes in volume were seen but did not correlate with the percent decrease in growth hormone. It was concluded that changes in sella volume cannot be used to indicate decreased tumor function after radiation.

J. Bruce Jacobs

Papers

Combined Ovarian and Adrenal Vein Catheterization To Determine the Site(s) of Androgen Overproduction in Hirsute Women

Bilateral anorchia with persistent testosterone production.

A critical evaluation of interval sella turcica measurements after irradiation for acromegaly.