Biomedical subjects
Publications and source records attributed to A V Stepanas.
We report a 16-year-old phenotypic female with XY pure gonadal dysgenesis. A dysgerminoma was found in one of the dysgenetic gonads stressing the importance of prophylactic gonadectomy. Her case presents a difficult problem because as a member of a national junior training squad, she had the potential for competing at international level where her genetic sex could be a profound liability.
Thirty-two patients with medullary carcinoma of the thyroid (MCT) were studied before and after therapy. Sixteen patients had familial and 16 had the sporadic type of disease. The groups differed in several features: Patients with sporadic disease were older at diagnosis and presented with enlargements in the thyroid or lymph nodes, and one patient had neuromata; in the familial group the disease was more often bilateral and was associated at times with the MEA syndrome (parathyroid adenoma or pheochromocytomas, or both). The immunoreactive serum calcitonin (CT) level was measured before and after some form of therapy in all 32 patients. Our investigations showed: 1) Delaying treatment was clearly detrimental in this progressive disease; 2) The most effective therapy was surgery, while radiotherapy and chemotherapy were less effective; 3) Postoperatively, the CT level usually fell sharply, when the disease was thought extirpated, but the lowest nadir might be reached at from 1 month to 6 years; 4) In all such patients the CT level eventually rose from the postoperative nadir; 5) Patients with no clinical or radiological evidence of disease, had high CT levels for a mean of 3 years of observation; 6) A marked rise of CT levels in three patients preceded metastases and death; 7) Patients with abdominal, particularly liver disease had the highest CT levels; and 8) Patients who died had relatively high CT levels, corresponding in general with the extent of disease.
Ten unselected patients with gonadal dysgenesis, three with Klinefelter's and seven with Turner's syndromes, were studied. Two of the patients with Klinefelter's and two with Turner's syndromes had enlarged pituitary fossae. The other six patients had normal sized sella, but showed localized change in the sellar contour on polytomography, suggesting hyperplasia or microadenoma formation of the pituitary gland. All ten patients had abnormally high serum follicle-stimulating hormone levels both at basal and after luteinizing hormone-releasing hormone stimulation. These results suggest that hyperplasia or microadenoma of the pituitary gland may occur secondary to gonadal failure, producing enlarged volume or attenuation in the normal contour of the sella turcica. Lack of awareness of reactive pituitary changes secondary to gonadal failure may result in inappropriate surgical management of what may appear to be primary pituitary tumor.
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Prolactin (PRL), human placental lactogen (hPL), the beta-subunit of human chorionic gonadotropin (betahCG), testosterone (T), estrone (E1), and estradiol (E2) were measured in blood samples from 45 patients with testicular tumors, 27 of whom had gynecomastia at some stage of their disease. Forty-two of the 45 patients had at least one abnormal hormone level. The most common abnormality was that of plasma estrone: it was elevated in 32 out of 42 (76%) patients in whom it was measured, suggesting a useful role for E1 as a testicular tumor marker. Prognosis was notably worse in patients with embryonal carcinoma, teratocarcinoma, and choriocarcinoma, in those with gynecomastia and, particularly, galactorrhea. Such patients also had the highest incidence of hormonal abnormalities as well as the most extreme absolute values. Hormonal mechanisms were implicated in the development of gynecomastia and galactorrhea. Prolactin, betahCG, E1, and E2 levels in all permutations correlated significantly among patients with gynecomastia, but not among those without, while estrogen to testosterone ratios were elevated in patients with galactorrhea.
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A new kit for measuring total serum triiodothyronine (T3) by radioimmunoassay ('T3 RIA'; Radiochemical Centre, Amersham) was evaluated using sera from 1114 patients and normal controls. The kit performed reliably with intraassay and interassay variability figures of 3.9% and 9.3%, respectively, at 'medium' concentrations of T3. A T3 measurement could be obtained conveniently within 18 h after overnight incubation at room temperature. There were no critical steps dependent on time or temperature. Serum T3 values showed no significant sex difference. There was no significant change in mean serum T3 between the ages of 15 and 69 years, but it fell by 0.15 nmol/l for every 5 years beyond the age of 70. Mean serum T3 (+/-SD) for 335 normal euthyroid subjects ages 15-69 years was 2.11+/-0.46 nmol/l (range: mean+/-2 SD = 1.19-3.03 nmol/l). 64% of pregnant euthyroid women and 12% of those taking oral contraceptives had elevated serum T3 levels, as did all hyperthyroid patients, apart from one with T4 toxicosis. Overlapping T3 values from hyperthyroid patients and euthyroid subjects with elevated concentrations of thyroid binding proteins could be separated completely by two correction techniques which related total serum T3 to the corresponding T3 resin uptake test, viz an 'augmented free T3 index', or a map plot of T3 vs T3 resin uptake. A 25% incidence of T3 toxicosis was observed. One hyperthyroid patient with T4 toxicosis, and seven euthyroid patients with 'biochemical T4 toxicosis' were investigated. 73% of moderately and 30% of severely hypothyroid patients had normal serum T3 levels. This overlap was not reduced by applying correction techniques. Our studies demonstrate the value of serum T3 measurements in screening for and diagnosing hyperthyroidism. As T3 measurements become more widely available, it would seem reasonable to subclassify hyperthyroid patients into three types: those with 'T3/4 toxicosis', 'T3 toxicosis' and 'T4 toxicosis'.
A system of "in vitro" thyroid function testing is proposed whereby laboratory staff select the most appropriate screening test depending on the information supplied by the clinician. Total serum triiodothyronine (T3) or thyroxine (T4) are used for screening as appropriate. In borderline cases, secondary tests are performed automatically according to a flow chart. This system improves efficiency and is cost effective, saving approximately 1,800 pounds annually in a laboratory handling about 5,000 requests for thyroid function tests each year.
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