Scintigraphic localization of ovarian dysfunction.

To assess the potential role of scintigraphy in the evaluation of clinically and biochemically suspect ovarian hyperandrogenism (HA), dexamethasone suppression 131I-6 beta-iodomethyl-19-norcholesterol (NP-59) scans were performed to characterize ovarian function in nine patients. Pelvic ultrasound and/or computed tomography (CT) identified anatomic abnormalities in the adnexal region in six women in whom there was discernible pelvic accumulation(s) of NP-59. In the remaining three patients testosterone levels were normal or only slightly elevated and the NP-59 scan did not demonstrate abnormal adrenal or pelvic uptake. CT and/or ultrasound studies failed to demonstrate an abnormality in the pelvis suggesting excessive peripheral conversion or abnormal end organ sensitivity of androgen precursors as potential etiologies of their HA. In three women with androgen secreting lipoid tumors of the ovary, unilateral, pelvic NP-59 activity was noted; these tumors were subsequently resected. Two women with bilateral pelvic NP-59 uptake were later shown to have hyperthecosis with markedly asymmetric and enlarged ovaries. In one woman the extent of asymmetric NP-59 uptake was anticipated by the asymmetry of ovarian vein androgen levels at selective venous catheterization. In another woman with markedly asymmetric polycystic ovary disease, intense focal uptake of NP-59 localized to the side of the anatomically abnormal, enlarged ovary. Thus, our preliminary study reviews our experience to date and suggests that NP-59 scintigraphy may be used to localize both tumorous and nontumorous ovarian dysfunction in states of HA and virilization.

Scintigraphic evidence of adrenal cortical dysfunction in the polycystic ovary syndrome.

The polycystic ovary syndrome (PCO) is frequently associated with elevated plasma levels of adrenal androgens and/or abnormal adrenal androgen responses to hormonal stimulation and suppression. Because of the overlap in the output of hormones between the adrenal glands and the ovaries, we measured adrenal iodocholesterol accumulation as an index of adrenocortical function in 11 women with PCO confirmed by laparoscopy or culdoscopy. All patients had normal dexamethasone suppression of plasma cortisol or urinary 17-hydroxycorticosteroid excretion. 6 beta-[131I]Iodomethylnorcholesterol (NP-59) adrenal scintiscans showed bilateral and excessive adrenal cortical uptake in PCO patients [0.46 +/- 0.08% (+/- SE) of the administered dose compared to 0.21 +/- 0.01% in a group of normal women; P less than 0.05]. The elevation of adrenal cortical NP-59 uptake in PCO was quantitatively similar to that in women with ACTH-dependent Cushing's syndrome (0.64 +/- 0.11%; P greater than 0.1). These data support the presence of abnormal adrenal cortical function in patients with PCO. The adrenal dysfunction of PCO most likely represents abnormal control of adrenal androgen production/secretion.

The relationship of I-131 6 beta-iodomethyl-19-norcholesterol (NP-59) adrenal cortical uptake to indices of androgen secretion in women with hyperandrogenism.

Dexamethasone suppression (DS) adrenal cortical scintigraphy has been shown to be useful in the detection of the adrenal pathology in women with hyperandrogenism. However, a relationship between adrenal cortical uptake of I-131 6 beta-iodomethylnorcholesterol (NP-59) and the level of adrenal androgen secretion has not been established. A retrospective analysis of DS adrenal scintiscans has been performed on 39 women with hirsutism and hyperandrogenism. In 14 patients with normal patterns of imaging, in vivo adrenal gland iodocholesterol uptake, calculated using a semi-operator-independent-computer algorithm, did not correlate with the excretion of urinary 17-ketosteroids (17-KS). In contrast, in 20 patients demonstrating abnormal bilateral early imaging patterns, adrenal gland NP-59 uptake correlated significantly with the level of urinary 17-KS excretion (r = 0.65, P less than 0.05). To date seven of these 20 patients have had confirmatory procedures documenting the adrenal glands as contributing sites of androgen secretion. A similar correlation with urinary 17-KS excretion was seen in five other patients with unilateral imaging patterns (r = 0.94, P less than 0.005), due to androgen-secreting adrenal cortical adenomas. No correlation between adrenal NP-59 uptake and plasma testosterone or dehydroepiandrosterone sulphate levels was observed in any of the groups. Thus, adrenal gland uptake of NP-59 under DS reflects a measure of androgen secretion in women with androgen excess.

Dexamethasone-suppression adrenal scintigraphy in hyperandrogenism: concise communication.

To assess the contribution of adrenal-derived androgens in women with hirsutism, adrenal scintigrams under dexamethasone suppression (DS) were performed on 35 women with increasing facial or body hair and irregular or absent menses. Based upon the DS regimen chosen (8 mg/d for 2 days or 4 mg/d for 7 days before the injection of 6 beta-[131I]iodomethylnorcholesterol), three imaging patterns were identified. The first was the absence of uptake before 3 days (8-mg DS) or before 5 days (4-mg DS) after injection. This imaging pattern was seen in 17 of the 35 patients studied and was considered normal. The second pattern was bilateral uptake earlier than 3 days (8-mg DS regimen) or 5 days (4-mg DS) after injection. This was seen in 13 of the 35 patients and was interpreted as bilateral early visualization. Adrenal-vein catheterization performed on six patients with this pattern showed increased adrenal-vein testosterone. The third pattern, observed in five patients, was unilateral early visualization, which in four cases investigated to date was the result of an adrenocortical adenoma. This study confirms the adrenal cortex as a source of androgens in women with hirsutism and hyperandrogenism and demonstrates the DS adrenal scintigraphy can be utilized to identify those women in whom adrenal-derived androgens contribute to their hyperandrogenism.

Familial Turner syndrome.

Seven women in three generations of a family have been affected by Turner syndrome. Turner phenotype in this family is the result of deletion of the entire short arm of one X chromosome. The short arm deletion is transmitted by carriers of a balanced X-1 translocation. Autoradiographic findings showed that the deleted X chromosome was late labeling in those persons with Turner syndrome, whereas the normal X chromosome was late replicating in carriers of the balanced translocation. The results of Xga typing of erythrocytes suggest that the Xg locus is on the short arm of the X chromosome. Because of the clinical implications, we believe that families of persons with structural chromosomal abnormalities should be studied to exclude familial transmission.