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Biomedical subjects

A Tenore

Publications and source records attributed to A Tenore.

At least 55 records · Page 3Linked to original sources

Cytogenetic and endocrine findings in a female with 45,X,t(y;18) (p11;p11).

A 23-year-old phenotypic female with congenital heart disease, mental retardation and mild virilization was referred for evaluation of short stature and delayed sexual development. Endocrine studies revealed a markedly elevated serum testosterone, which was within the adult range. At laparotomy, a small uterus, normal fallopian tubes and bilateral gonadal tumors, consisting of a left gonadoblastoma and right dysgerminoma were found. Trypsin G banding of peripheral blood revealed a 45,XO, 18p+ karyotype. Q banding demonstrated intense fluorescence of the distal portion of the extra material on chromosome 18, consistent with fluorescence of Y chromosomal heterochromatin. A combination of banding techniques enabled us to determine a 45,X,t(Y;18) (p11;p11) karyotype in peripheral blood. Cultures of gonadal tissue revealed 45,X,t(Y;18)/46,XY mosaicism.

Adult↗

Thyrotropin in human breast milk.

The thyrotropin (TSH) content of human breast milk was investigated, using both direct 125I-TSH radioimmunoassay and radioimmunoassay preceded by a partial purification step of elution from concanavalin A-Sepharose 4B. 15 samples of human milk between 1 and 13 weeks post-partum were found to contain 2.0 +/- (SD) 0.9 microU TSH/ml when assayed after Con A-extraction and adjusted for recovery by interpolation of a standard curve of Con A-extracted TSH standards. Recovery of 125I-hTSH tracer from both serum standards (58.6 +/- 5.5%; n = 10) and milk (53.6 +/- 6.7%; n = 15) were comparable. TSH levels obtained post-extraction were not significantly different from those that TSH is present in human breast milk in low concentrations, comparable to those normally found in the serum of euthyroid adults.

Adult↗

Diagnosis of ectopic thyroid: value of pretracheal soft-tissue measurements.

Thyroid ectopia, dysgenesis, or agenesis has been conventionally diagnosed with the use of radioisotope scanning procedures. This report proposes the use of a conventional lateral radiograph of the neck to determine the presence or absence of the thyroid gland in its normal anatomic position. The thyroid gland is ensheathed by the pretracheal layer of the deep cervical fascia anterior to the trachea. The gland's presence or absence can be determined by calculating the ratio of the soft-tissue measurements between the anterior wall of the trachea and both the outer margin of the skin and the margin of the subcutaneous fat as determined from a lateral radiograph of the neck. This "pretracheal ratio" may be used to accurately differentiate between a lingual thyroid and other masses at the base of the tongue.

Adolescent↗

Changes in serum N-acetyl-beta-hexosaminidase levels after treatment of hypothyroid and hyperthyroid individuals with L-thyroxine and propylthiouracil.

Multiple serum samples were obtained from six hypothyroid and six hyperthyroid females, 11--17 years of age, over the course of their corrective treatment with L-thyroxine (LT4) and propylthiouracil (PTU), respectively. Sera were assayed for total N-acetyl-beta-hexosaminidase (HEX), the A (heat-labile) and B (heat-stable) isozymes, and total T4. HEX activity (total HEX A) in sera from hypothyroid (< 4 micrograms/dl T4) patients (total HEX: 518 +/- 66 nmol/60 min/ml, mean +/- S.D.; HEX A: 325 +/- 55; n = 5) was significantly lower than that of the euthyroid control group (total HEX: 638 +/- 77 (p < 0.005); HEX A: 420 +/- 76 ( p < 0.01); n = 23); no difference in HEX B levels was noted. Serum samples from patients successfully treated for hypothyroidism via oral administration of LT4 (n = 12) displayed levels of total HEX (722 +/- 113) and HEX A (491 +/- 91) significantly higher than those of the control group (p < 0.01 in both cases); again, no change in levels of HEX B was observed. HEX activity in sera from hyperthyroid (> 13 micrograms/dl T4) individuals (total HEX: 839 +/- 96; HEX A: 540 +/- 74; HEX B: 299 +/- 52; n =20) was significantly higher than that of the control group (p < 0.005 in all cases). The depression of hormone activity to the euthyroid range by PTU was accompanied ay a decrease in enzyme activity to control levels (total HEX: 632 +/- 92; HEX A: 400 +/- 55; HEX B: 232 +/- 52; n = 16). Non-parametric analysis of the data shows highly significance differences between pre- and post-treatment enzyme levels (alpha < 0.001) in both hyper- and hypothyroid groups. Alteration of thyroid status, and specifically T4 level is, therefore, indicated to be a contributing factor in the regulation of serum HEX activity in humans, as evidenced by individual responsiveness to oral administration of this hormone, or inhibitors of its peripheral metabolism.

Adolescent↗

Sex differences in serum levels of N-acetyl-beta-hexosaminidase in infancy: correlation of enzyme activity with testosterone levels.

Serum levels of N-acetyl-beta-hexosaminidase (HEX) (EC 3.2.1.30) activity in infants display a sexual dimorphism. Total HEX activity in males between 1 and 3 months of age is significantly elevated over female levels (male (M), 1535 +/- 300 nmol/60 min/ml; female (F), 1150 +/- 203, P < 0.0005), and the A (labile) isozyme constitutes a significantly lower proportion of the total activity present (M, 56.0 +/- 4.2, N = 24; F, 64.3 +/- 4.6, N = 21, P < 0.0005). These findings led us to investigate the relationship between testosterone concentration and HEX activity in serum. Samples from male (N = 36) and female (N = 33) infants between 1 and 6 months of age were included. In both sexes, a high degree of correlation (P < 0.0005) was observed between testosterone and total HEX (M, r = 0.71; F, r = 0.73), HEX A (M, r = 0.68; F, r = 0.56) and HEX B (M, r = 0.68; F, r = 0.72). An inverse relationship exists between testosterone levels and % A: M, r = -0.56; F, r = -0.38 (P < 0.0025 and 0.025, respectively). In contrast, no correlation between HEX levels and testosterone was evident in either male or female adults (r = 0.20 and 0.18, respectively). These data implicate testosterone in the regulation of HEX activity during the early months of human development.

Adult↗

Thyroidal response to peroral TSH in suckling and weaned rats.

The biological activity of perorally administered bovine thyroid-stimulating hormone (bTSH) was investigated in suckling (14-day-old) and weaned (30-day-old) rats. Animals were treated with water or bTSH given either by subcutaneous injection or by the oral route. Both suckling and weaned animals responded to subcutaneous administration of bTSH by an increase in serum thyroxine (T4) and triiodothyronine (T3) concentrations. Bovine TSH administered orally to weaned animals had no effect on thyroid hormone levels. In contrast, suckling pups responded to perorally administered bTSH with increases of T4 and T3 levels comparable to those achieved in animals that received bTSH subcutaneously. The characteristics of the gastric secretion and intestinal epithelium of the weaned animal, therefore, appear to play a role in preventing absorption of a polypeptide hormone. However, protein hormones contained in maternal milk can be transferred to the suckling in which their full biological functions can be expressed.

Administration, Oral↗

Effect of triiodothyronine injection on levels of triiodothyronine and thyroid-stimulating hormone in sera and milk of lactating rats and in sera of their sucklings; precocious development of jejunal alpha-disaccharidases in the sucklings.

Administration of high doses of triiodothyronine (T3) for 4 days to lactating rats evokes an increase in T3 levels in their sera and milk, as well as in the sera of pups suckled by them. Thyroid-stimulating hormone levels in sera of mothers and sucklings are decreased. Suckling rats of T3-treated mothers exhibit a precocious increase in the activity of jejunal sucrase and maltase as well as in activity of several liver acid beta-glycosidases.

Acetylglucosaminidase↗

Relationship of thyroid status and serum N-acetyl-beta-glucosaminidase isoenzyme activities in humans.

Serum from 28 hyperthyroid, hypothyroid, and euthyroid pre- or early puberty females was examined for N-acetyl-beta-glucosaminidase (HEX) activity. Total, isoenzyme A (labile), and isoenzyme B (stable) levels were determined for this enzyme. A high degree of correlation (r = 0.76; p less than 0.001) exists between total hexosaminidase activity and thyroid hormone levels (as reflected by the Free Thyroxine Index). Examining each isoenzyme individually, A is selectively enhanced (r = 0.84; p less than 0.0005) whereas B displays no significant change regardless of thyroid activity. In hyperthyroid individuals, levels of total hexosaminidase (730 +/- 67 units) (mean +/- S.D.) and isoenzyme A (516 +/- 46) were significantly higher than levels found in either the hypothyroid (total: 547 +/- 30; isoenzyme A: 352 +/- 31) or euthyroid (total: 620 +/- 81; isoenzyme A: 423 +/- 45) groups. However, no change was observed in levels of isoenzyme B among hypothyroid (195 +/- 19), euthyroid (197 +/- 43) and hyperthyroid (215 +/- 32) groups. These data substantiate our earlier findings in the rat, wherein thyroxine administration evoked a similar response in the liver. They are of particular interest in light of the deficiency of HEX A in variants of GM2 gangliosidosis.

Acetylglucosaminidase↗

Familial hypopituitarism with large sella turcica.

We studied endocrine function in three siblings with short stature and enlargement of the sella turcica. Sellar volumes were 5.9, 3.7 and 4.0 standard deviations above age-specific means. Computed tomography or pneumoencephalography showed full sellae without suprasellar extension. Basal thyrotropin levels were low despite hypothyroidism and increments were less than 3 micromicron per milliliter after thyrotropin-releasing hormone injection. Stimulated growth hormone levels were less than 5 ng per milliliter, declining to less than 2 ng per milliliter after thyroxine treatment. Both thyroxine and growth hormone treatments were required for rapid growth. Impaired thyrotropin responses to thyrotropin-releasing hormone distinguish these patients from most cases of idiopathic or familial deficiency of thyrotropin and growth hormone. Persistent deficiency of growth hormone during thyroxine treatment indicates that the defects is not limited to thyrotropin structure or release. The findings are compatible with either familial neoplasia of the anterior pituitary or a regulatory defect promoting anterior pituitary-cell hyperplasia and inhibiting thyrotropin and growth hormone release.

Adolescent↗

Identification of heterozygote carriers of congenital adrenal hyperplasia by radioimmunoassay of serum 17-OH progesterone.

The response to administered adrenocorticotropin (ACTH, Cortrosyn) of 26 heterozygotes (parents of children with adrenal 21-hydroxylase deficiency) and of 14 controls are compared. The mean plasma levels of 4-pregnene-3, 20-dione-17, 21-diol (17-OH progesterone) were significantly greater in the heterozygotes 60 minutes (p less than 0.02) and 90 minutes (p less than 0.05) after stimulation with Cortrosyn than in controls. There is, however, considerable overlap. The results would indicate a partial enzyme deficiency in the parents of diseased subjects. There was no significant difference in the response of plasma cortisol.

Adrenocortical Hyperfunction↗

Basal and stimulated serum growth hormone concentrations in inflammatory bowel disease.

Patients with inflammatory bowel disease (IBD) manifest growth failure which may antecede abdominal symptoms by some years. Eight of ten children with documented IBD had records of decreasing growth velocities. Investigation of growth hormone reserves showed excessive rather than impaired responses. Mean basal GH level was 6.2 +/- 0.75 (SEM) ng/ml. During sleep, the mean GH level rose to 26.0 +/- 4.7 ng/ml and following propranolol-glucagon stimulation, to 46.0 +/- 4.5 ng/ml. All values were significantly higher than levels obtained in a control population of 25 children investigated for short stature who were not GH deficient. The mean peak GH response following insulin in the IBD group (10.8 +/- 3.8 ng/ml), however, did not differ from the mean peak response in the control group (13.5 +/- 3.3 ng/ml). Growth failure in patients with IBD is not the result of GH deficiency and is not an irreversible phenomenon. On the contrary, judicious use of glucocorticoids aimed at the control of the disease usually produces compensatory growth acceleration ("catch-up growth").

Adolescent↗

Gonadotropin responses to luteinizing releasing factor in boys treated with cyclophosphamide for nephrotic syndrome.

Cyclophosphamide therapy of the nephrotic syndrome has been associated with oligo- and azoospermia and with abnormalities of testicular histology in adults and pubertal boys. In 15 prepubertal boys, no abnormalities of basal serum levels of LH, FSH, or T were found when they were studied 8 months to 7 years after cyclophosphamide therapy. Five boys were pubertal during therapy were found to have elevated mean basal values of gonadotropins with normal testosterone levels and elevated LH responses to LRF; the FSH responses to LRF were elevated in four patients. One of four boys who were prepubertal during therapy but pubertal at the time of testing had an elevated basal LH and LH response to LRF. Three boys who were prepubertal at the times of therapy and testing had normal LH responses to LRF. The LRF test may provide a means of identifying the patient who has sustained testicular injury and who may require testicular biopsy.

Adolescent↗

Characterization of somatogenic and lactogenic binding sites in isolated rat hepatocytes.

Suspensions of rat hepatocytes isolated enzymatically by the method of Berry and Friend were used to study the binding of 125I-labeled human (hGH) and bovine (bGH) growth hormones and ovine prolactin (oPRL). Displacement of these labeled hormones by their unlabeled analogues was analyzed by means of Scatchard plots and affinity constants (K) and the number of binding sites per cell (q) were calculated. Specificity of binding was studied using hGH, bGH oPRL and rat growth hormone (rGH) and rat prolactin (rPRL). Rat hepatocytes contained two types of binding sites which bound hGH. The first, somatogenic, was specific for the growth-promoting hormones bGH and rGH. The second, lactogenic, was specific for lactogenic hormones, oPRL and rPRL. Human GH, which has both lactogenic and growth-promoting properties in rodents, bound to both sites. The somatogenic binding sites were present in both males and females, and the number of sites was similar in females and in males and was not affected by hypophysectomy. The lactogenic binding sites were present only in females, and the number of lactogenic and somatogenic sites was similar (40,000/cell). The affinity of hGH for the lactogenic binding sites was less than for the somatogenic (0.37 X 10(9) vs. 1 X 10(9)M-1). The lactogenic binding sites were lost when female rats were hypophysectomized and could not be restored by estrogen treatment.

Animals↗