bluuebunny/crossref_metadata_2025_split huggingface dataset:
PUBERTAL MATURATION OF THE LH STIMULATORY RESPONSE TO CLOMIPHENE CITRATE IN CONGENITAL VIRILIZING ADRENAL HYPERPLASIA*
Authors: MOREIRA AYRTON CUSTÓDIO , VERÍSSIMO JOSÉ MOACIR TABOSA , FOSS MILTON CESAR , IAZIGI NASSIM , MACIEL LEA MARIA ZANINI , PIMENTA WALKYRIA DE PAULA , RODRIGUES JOSÉ ANTUNES , SANTORO JOSÉ ROMANO 
Published: March 2008

SUMMARYTo determine the LH release response to clomiphene citrate in children with virilizing congenital adrenal hyperplasia (CAH), we examined six patients with glucocorticoid‐treated virilizing CAH, five girls and one boy, chonological age (CA) 4 years 2 months‐15 years 9 months and bone age (BA) range 8–18 years. All the patients but one, were treated with nocturnal dexamethasone. Their ages at the onset of treatment ranged from 2 years 6 months to 13 years 8 months. An additional boy (CA = 4 years 3 months and BA = 7–8 years) was studied 10 days after removal of a virilizing adrenal adenoma. Clomiphene citrate (3mg/kg/day) was administered orally in two doses/day for 8 days. Venous blood was collected on days 0, 4, 6 and 8. Two patients were also studied in consecutive years. In three patients no increase was noted over the basal levels of LH, but in the others LH rose during clomiphene administration. In two patients studied in consecutive years, there was detectable shift of LH release pattern. The basal LH levels and the sum of responses (day 4, 6 and 8) to clomiphene citrate and particularly their decimal logarithms were statistically significantly correlated with their respective BA. These results confirm that previous prolonged exposure to elevated concentrations of adrenal sex steroids, in children with treated virilizing CAH, may induce precocious maturation of the hypothalamic pubertal mechanism of LH secretion. This maturation was closely correlated with the degree of skeletal maturation.
THYROID ABNORMALITIES IN PATIENTS WITH MYOTONIC DYSTROPHY
Authors: STEINBECK KATE S. , CARTER JOHN N. 
Published: March 2008

SUMMARYMyotonic dystrophy (MD) is a systemic genetic disorder predominantly affecting musculature. Although endocrine disturbances have been described, data relating to thyroid gland involvement are sparse and conflicting. To further investigate this aspect, twenty MD patients and twenty age‐and sex‐matched controls were studied. All were clinically euthyroid and there were no significant differences between circulating levels (mean ± SD) of T4 (101±5 ± 28±2 v. 103±8 ± 27±7 nmol/1), T3 (1±86 ± 0±57 v. 2±15 ± 0±47 nmol/1), rT3 (0±39 ± 0±14 v. 0±42 ± 0±09) nmol/1) or TSH (2±6 ±0±5 v. 2±4 ± 0±8 mu/1) or between T3 resin uptakes (97 ± 8 v. 98 ± 11%). TSH responses following TRH stimulation were significantly less in the MD patients (323 ± 141 ± 529 ± 240%; P < 0±0025). The mean maximum TSH levels achieved were 6±7 ± 3±6 mu/1 (MD) and 17±1 ±7±2 mu/1 (controls); P < 0±005. Two patients had multinodular goitres (MNG) and one a single thyroid nodule. Thyroid scans were normal except for patchy uptake in the two MNG patients. There was no abnormal incidence of circulating thyroid microsomal or thyroglobulin antibodies, and thyrotrophin binding inhibitory immunoglobulin levels were normal in four patients. It is concluded that in MD: 1 palpable thyroid gland abnormalities occur with increased frequency, 15% (three/twenty) in the present study and 20% (twenty/102 case reports) from a review of the literature; 2 TSH responses to TRH are significantly reduced; 3 circulating thyroid hormone levels are usually normal.
AN ACTH‐SECRETING PITUITARY TUMOUR ARISING IN A PATIENT WITH CONGENITAL ADRENAL HYPERPLASIA
Authors: HORROCKS P. M. , FRANKS S. , HOCKLEY A. D. , ROLFE E. B. , NOORDEN SUSAN VAN , LONDON D. R. 
Published: March 2008

SUMMARYThe case reported is of a 46‐year‐old woman who had congenital adrenal hyperplasia due to a 21‐hydroxylase deficiency, and in whom there was the development of an ACTH secreting pituitary tumour. The patient was untreated with glucocorticoids until the age of 32 years when she presented with infertility. She next presented with amenorrhoea at the age of 44 years when she was found to have an enlarged pituitary fossa. Despite treatment with bromocriptine and adequate doses of dexamethasone, the tumour enlarged and required operative treatment 1 year later. Before and after operation, plasma ACTH levels were between 300 and 400 ng/1, immunocytochemistry showed staining for ACTH and other structurally related pro‐opiocortin peptides but for no other hormones, and the tumour secreted large amounts of ACTH in vitro. The report of this case is to our knowledge the first account of a feedback tumour in congenital adrenal hyperplasia and provides yet another reason why patients with this condition should be treated, and good control achieved.
AN IMPROVED METHOD FOR THE ESTIMATION OF RELATIVE BINDING CONSTANTS OF T4 AND ITS ANALOGUES WITH SERUM PROTEINS
Authors: MIDGLEY J. E. M. , WILKINS T. A. 
Published: March 2008

SUMMARYA new method is reported for the determination of the binding constants of homologous ligands relative to that of the parent ligand. The technique minimizes the effects of random and bias errors inherent in absolute methods and obviates the need to quantitate the radiolabelled tracer concentration. In addition, it is likely to be of general applicability for monovalent proteins. The method has been applied in the determination of the binding constant (relative to that of T4) of the T4‐analogue tracer used in the Amerlex® free T4 radioimmunoassay, in respect of its binding to both T4 binding globulin and prealbumin. The binding constants of the analogue (relative to T4) were approximately 3% for both proteins. These levels were judged unlikely to interfere significantly with the measurement of free T4 in serum samples from patients with very wide variations in concentration of either T4 binding globulin or prealbumin.
TISSUE CONCENTRATIONS OF LEVONORGESTREL IN WOMEN USING A LEVONORGESTREL‐RELEASING IUD
Authors: NILSSON C. G. , HAUKKAMAA M. , VIEROLA H. , LUUKKAINEN T. , ARCANGELI P. 
Published: March 2008

SUMMARYThirteen women who were scheduled for hysterectomy volunteered for the study. Nine women had a levonorgestrel‐releasing intrauterine contraceptive device inserted between 36 and 49 days prior to surgery and four women were on oral levonorgestrel treatment for 7 days prior to surgery. After the surgical removal of the uterus, samples of endometrial, myometrial, fallopian tube and fat tissue were obtained. Methods for measuring tissue concentrations of levonorgestrel were developed and the uptake of levonorgestrel by fat tissue in vitro was studied. The concentrations of levonorgestrel in myometrial, fallopian tube and fat tissue were all of the same order, of between 1 and 5 ng/g of wet weight of tissue, in both the intrauterine device group and the orally treated group. In the endometrium the levonorgestrel concentrations were many‐fold higher in the intrauterine device group. The in vitro experiments showed a rapid uptake of levonorgestrel and an unsaturability of the fat tissue by the steroid at the concentrations used. The fat tissue concentrations of levonorgestrel correlated with the plasma concentrations.
EFFECTS OF TSH ON cAMP LEVELS AND THYROID HORMONE RELEASE IN HUMAN THYROID‘AUTONOMOUS’NODULES: RELATIONSHIP WITH IODOTHYRONINE AND IODINE CONTENT IN THYROGLOBULIN
Authors: TOCCAFONDI R. , ROTELLA C. M. , TANINI ANNALISA , ATERINI S. , BORRELLI D. , And L. LODDI , ARCANGELI P. 
Published: March 2008

SUMMARYThe effect of TSH on the adenylate cyclase‐cAMP system and in vitro iodothyronine release, together with the iodothyronine and iodine content of 19s thyroglobulin, were studied in seven clinically euthyroid patients with autonomous thyroid nodules. Basal cAMP and cGMP content together with phosphodiesterase and protein‐kinase activities were normal in nodular, and suppressed in extranodular tissue. TSH‐dependent cAMP accumulation was reduced in nodular tissue, but normal in the suppressed extranodular tissue. In vitro TSH‐dependent iodothyronine release from nodular and extranodular tissue was absent. Thyroxine and iodine content of thyroglobulin extracted from nodular tissue was reduced, while triodothyronine content was normal but with a low T4/T3 ratio. In extranodular tissue T3, T4 and iodine contents were reduced. In conclusion, autonomous thyroid nodules produced a poorly iodinated thyroglobulin leading to preferential T3 secretion with increased circulating free thyroid hormones even in clinically euthyroid patients.
SALIVARY CORTISOL ASSAYS FOR ASSESSING PITUITARY‐ADRENAL RESERVE
Authors: PETERS J. R. , WALKER R. F. , And D. RIAD‐FAHMY , HALL R. 
Published: March 2008

SUMMARYCortisol concentrations were determined in matched samples of plasma and saliva from patients and healthy volunteers throughout the course of standard tests of pituitary and adrenal reserve. During insulin tolerance tests the percentage incremental changes in cortisol concentrations in saliva were strictly comparable with those in plasma and showed less inter‐subject variance. The clinical decision taken with regard to the integrity of the pituitary‐adrenal axis was the same whether plasma or salivary cortisol was measured. In the short tetracosactrin test changes in salivary cortisol reflected those in plasma and patients with loss of adrenal responsiveness would have been diagnosed as such using either measurement. In normal subjects, the circadian rhythm in salivary cortisol concentrations exactly paralleled that in plasma. Absence of the circadian rhythm in cases of hypercortisolism was seen as well in saliva as in plasma. Assays for salivary cortisol therefore provide information which is as clinically useful as that of plasma determinations. Since salivary cortisol concentrations were shown to reflect the free, biologically active fraction in plasma, salivary assay may, in selected cases, provide results of greater diagnostic significance than plasma total concentrations.
SLEEP‐ASSOCIATED GONADOTROPHIN AND OESTRADIOL PATTERNS IN GIRLS WITH PRECOCIOUS SEXUAL DEVELOPMENT
Authors: MATTHEWS M. J. , PARKER D. C. , REBAR R. W. , JONES K. L. , ROSSMAN L. , CAREY D. E. , YEN S. S. C. 
Published: March 2008

SUMMARYCirculating concentrations of gonadotrophins and oestradiol (E2) during sleep were examined in six girls aged 5–10 years with precocious sexual development. All subjects exhibited nocturnal increments of LH and/or FSH. Gonadotrophin secretion appeared to vary from pulsatile release to random oscillations. Three subjects with LH pulses of greater than 5 miu/ml and a concomitant FSH rise had a temporal elevation of E2 during sleep. The other three subjects with pulses of either LH or FSH of less than 5 miu/ml had no nocturnal rise in E2. These data suggest that the nocturnal elevation of E2 results from pituitary release of both LH and FSH, leads to premature sexual development, and may be occasioned by inappropriate timing of hypothalamic gonadotrophin‐releasing hormone (LHRH) secretion.
ACANTHOSIS NIGRICANS, HIRSUTISM, INSULIN RESISTANCE AND INSULIN RECEPTOR DEFECT
Authors: LEME CARLOS EDUARDO , WAJCHENBERG BERNARDO LÉO , LERÁRIO ANTONIO CARLOS , GOLDMAN JAYME , BORGES JOÑO LINDOLFO CUNHA 
Published: March 2008

SUMMARYA 24‐year‐old negress with the triad of acanthosis nigricans, hirsutism associated with polycystic ovaries and insulin resistance is reported. Metabolic studies were done 3 years after a bilateral ovarian wedge resection. Partial remission of the hirsutism and return of menstrual cycles occurred after surgery. Extreme resistance to endogenous and exogenous insulin was observed. Three studies of insulin receptors on circulating red blood cells (RBC) showed abnormal inhibition‐competition curves, characterized by increased percentage insulin binding at higher unlabelled insulin levels. Scatchard plots suggested an apparent increase in the number of low affinity receptors. Despite the changes in receptor‐insulin interaction, the defect does not seem to explain the insulin resistance since binding of insulin to a target tissue (RBC) appeared to be quantitatively normal at physiological insulin levels, suggesting a simultaneous post receptor defect.
THYROTROPHIN BINDING INHIBITION BY ANTI‐THYROTROPHIN RECEPTOR ANTIBODIES IN GRAVES' DISEASE WHICH IS NOT REFLECTED BY 1.6 M AMMONIUM SULPHATE PRECIPITATES
Authors: DE BRUIN T. W. A. , VAN DER HEIDE D. , QUERIDO A. 
Published: March 2008

SUMMARYIn ten patients with Graves' disease before, during and after treatment and three patients in remission, we have compared the TSH‐binding inhibition caused by serum, by 1.6 M ammonium sulphate precipitates from serum and by the fractions (19S, 7S and 4S) obtained after G‐200 gel chromatography of serum and ammonium sulphate precipitates.Five out of ten patients had a positive thyrotrophin‐binding inhibitor immunoglobulin (TBII) index before treatment, three of whom relapsed. The binding inhibition caused by 7S fractions from ammonium sulphate precipitates of the ten untreated patients could be correlated with their TBII index (r= 0.9, P<0.001).By contrast, the 7S fractions prepared directly from the sera of the same ten patients all showed marked TSH‐binding inhibition (P<0–001) when compared with normal plasma 7S, and no correlation with the TBII index could be demonstrated (r= 0.21, P>0.05).It is concluded that ammonium sulphate precipitates, which are used to calculate the TBII index, do not reflect the presence of anti‐TSH receptor antibodies in serum accurately in all patients.
TRILOSTANE AND THE NORMAL HYPOTHALAMIC‐PITUITARY‐TESTICULAR AXIS
Authors: SEMPLE C. G. , WEIR S. W. , THOMSON J. A. , BEASTALL G. H. 
Published: March 2008

SUMMARYTrilostane, a competitive inhibitor of the 3β‐hydroxysteroid dehydrogenase enzyme system, has adrenal blocking activity and has been used to treat Cushing's syndrome and other diseases. To investigate its effect on the normal human hypothalamic‐pituitary‐testicular axis, trilostane (initially 240 mg/day) was given to ten healthy adult males, the dose increasing at weekly intervals by 240 mg/day up to 960 mg/day. When chromatography was used to remove trilostane and metabolites from the assay system, serum testosterone was found to fall on trilostane therapy (P<0.01) and this was accompanied by a rise in LH (P<0.01). The responses of FSH and LH to LHRH were unaffected by treatment. It is concluded that trilostane inhibits human testicular 3β‐hydroxy‐steroid dehydrogenase and male patients on trilostane should be monitored for sexual dysfunction and impairment of testicular steroidogenesis.
HYPER‐RESPONSIVENESS OF ALDOSTERONE TO METOCLOPRAMIDE IN ALDOSTERONISM
Authors: GNIADEK T. C. , GREKIN R. J. , GROSS M. D. , VILLAREAL J. Z. 
Published: March 2008

Metoclopramide, a dopamine antagonist, stimulates aldosterone secretion in normal man. We studied the response of plasma aldosterone, plasma renin activity, cortisol, sodium, potassium and serum prolactin to a 10‐mg intravenous dose of metoclopramide in six dexamethasone suppressed patients with aldosteronism (four with adrenal adenoma, two with bilateral adrenal hyper‐plasia) and in six dexamethasone suppressed normal male volunteers. All subjects were studied on an ad libitum sodium diet. Patients were supplemented with oral potassium prior to study.Sodium and potassium were not different between groups and did not change following metoclopramide. Cortisol was suppressed to less than 3 μg/dl throughout the study in all subjects. Basal plasma renin activity was significantly lower in the patients as compared with controls (P < 0·05), and did not change in either group following metoclopramide.Basal aldosterone levels were not significantly different in patients as compared with controls, although patients did tend to have higher levels. The incremental and integrated response of aldosterone to metoclopramide in patients was significantly greater than controls (P < 0·01). The percentage increase in aldosterone was greater than controls in five of the six patients.Basal prolactin was higher in the patients than in the controls. All subjects had a significant rise in prolactin to metoclopramide (P < 0·05). Five of six patients had an increased response of prolactin when compared to the controls and the four female patients had a significantly greater rise in prolactin as compared to the normals (P < 0·01).These data suggest that increased dopaminergic activity plays a compensatory role in inhibiting aldosterone secretion in patients with aldosteronism. It appears unlikely that a decrease of dopaminergic inhibition is involved in the pathogenesis of aldosteronism due to either adenoma or bilateral hyperplasia.
VARIATIONS IN CLINICAL, HORMONAL AND SEROLOGICAL EXPRESSIONS OF CHRONIC LYMPHOCYTIC THYROIDITIS (CLT) IN CHILDREN AND ADOLESCENTS
Authors: DE PAPENDIECK LAURA GRUñEIRO , IORCANSKY SONIA , RIVAROLA M. A. , BERGADÁ C. 
Published: March 2008

SUMMARYA diagnosis of chronic lymphocytic thyroiditis (CLT) was made in forty‐two children and adolescents according to Fisher's criteria. The age of the group ranged between 4±3 and 18±5 years. On the basis of serum TSH, T4 and RAI uptake, three groups of patients were distinguished at the time of diagnosis: 1, euthyroid group (n= 3) with normal TSH and T4; 2, hypothyroid group (n= 36) with high values of TSH, where three subgroups were distinguished: (a) compensated hypothyroidism (n= 14) with normal T4, (b) overt hypothy‐roidism with high or normal RAI uptake and low T4 (n= 19), (c) overt hypothyroidism with low RAI uptake and low T4 (n= 3); 3, Hyperthyroid group (n= 3) with high T4. In CLT, variability is a feature not only of the initial presentation, but also of the subsequent evolution of the disease. Sixteen patients were followed for periods between 4 months and 6 years. Thyroid function did not change in six. Six patients with compensated or overt hypothyroidism improved, four became euthyroid with normal basal TSH and T4 values, however, all three tested had an exaggerated response to TRH. Three patients who were initially hyperthyroid progressed to a state of compensated hypothyroidism. One patient with overt hypothyroidism on presentation developed hyperthyroidism after 15 months and became hypothyroid once more 8 months later. The variable clinical course of CLT is characteristic of immunological disturbances.
ALDOSTERONE IN SUSTAINED ESSENTIAL HYPERTENSION
Authors: SAFAR M. E. , SIMON A. Ch. , DARD S. A. , PARLIER H. R. , PAULEAU N. E. , VINCENT M. L. , SASSARD J. E. 
Published: March 2008

SUMMARYPlasma aldosterone (PA), plasma renin activity (PRA), extracellular fluid volume (EFV) and hepatic blood flow were measured in forty‐four patients with sustained essential hypertension and compared with forty‐two normotensive controls of same age and sex. All patients had inulin clearances within the normal range and balanced sodium intake and urinary output. In hypertensives, PA, PRA, EFV and hepatic blood flow were within normal ranges; the log‐ratio PA: PRA was significantly elevated (P < 0·001). In normotensives, a negative relationship was observed between PA and EFV (r= ‐0·55; P <0·001) while a positive relationship was observed between PA and PRA (=+0·70; P < 0·001). In hypertensives, the two relationships were disrupted or less significant: for a given value of EFV, PA was more elevated in hypertensives than in normotensives; for a given value of PRA, PA was more elevated in hypertensives than in normotensives. The results could not be explained on the basis of a disturbance in hepatic blood flow and/or in the metabolic clearance rate of aldosterone. The study provided evidence that, in patients with sustained essential hypertension and equilibrated sodium balance, there is an excess of plasma aldosterone relative to the levels of extracellular fluid volume and plasma renin activity. The excess is probably related to an abnormality in the adrenal secretion.
T3 HYPERTHYROIDISM AND THYROID CANCER
Authors: KRUTER REGINA H. E. , LIEDTKE MONIKA , DE CASTRO J. A. SISSON , GROSS J. L. 
Published: March 2008

SUMMARYThe authors present an unusual case of T3 hyperthyroidism caused by a well‐differentiated thyroid follicular carcinoma and its large skull metastasis. They suggest iodine deficiency as being the single major factor in the aetiology of both disorders.
THE REPRODUCTIVE ENDOCRINE SYSTEM IN CYSTIC FIBROSIS: 2. CHANGES IN GONADOTROPHINS AND SEX STEROIDS FOLLOWING LHRH
Authors: REITER E. O. , STERN R. C. , ROOT A. W. 
Published: March 2008

SUMMARYHypothalamic–pituitary–gonadal function was assessed in forty‐seven patients with cystic fibrosis (CF) by the 3‐h infusion of 100 μg of synthetic gonadotrophin‐releasing factor, LHRH and the results compared with a group of children being evaluated for short stature and delayed puberty (‘controls’). Levels of gonadotrophins and sex steroids were measured prior to and during the infusion. In prepubertal boys, LH and FSH release evoked by LHRH was significantly greater (P < 0·001) in ‘control’subjects than in CF patients. In pubertal boys, LH and FSH release was also greater in ‘controls’than in CF, though to a lesser degree (P < 0·05). In pubertal girls, responses to LHRH were comparable for LH and slightly greater (P < 0·05) in ‘controls’for FSH. In the earliest pubertal groups of both sexes (male‐Tanner genitalia stage 2; females‐Tanner breast stage 2), LH secretion was similar in patients with CF and ‘control’subjects. Significant increments of testosterone and oestradiol in pubertal CF patients do not occur until 6 h after the LHRH infusion begins, in contrast to a rise at 3 h in ‘control’subjects. These data suggest that prepubertal boys with CF, who are the most impaired in height, weight and skeletal maturation, also have measurable abnormalities of LHRH‐releasable gonadotrophin secretion. Despite continued impaired weight growth, pubertal patients do attain essentially normal gonadotrophin secretory responses to LHRH administration and are similar to subjects with constitutional delayed adolescent development in reproductive endocrine physiology.
CLOMIPHENE CITRATE DOES NOT MODIFY THE EXAGGERATED THYROTROPHIN RESPONSE TO THYROTROPHIN‐RELEASING HORMONE OCCURRING IN PRIMARY TESTICULAR FAILURE
Authors: SPITZ I. M. , KEREM E. , ZYLBER‐HARAN E. , SHILO S. , LAUFER N. , LIVSHIN Y. , SCHENKER J. G. 
Published: March 2008

SUMMARYPatients with primary testicular failure have increased basal TSH levels and an exaggerated TSH response to TRH in the presence of normal circulating levels of thyroid hormones. In order to evaluate if this TSH profile is an oestrogenrelated phenomenon, sixteen patients with primary testicular failure were challenged with 200 μg TRH prior to and after the administration of clomiphene citrate. The latter was given in a dose of 100 mg/day for 4 weeks to ten patients; 200 mg/day for 4 weeks to three patients and 100 mg/day for 2 months to the final three patients.The patients demonstrated increased mean basal TSH levels with an exaggerated TSH response to TRH. Following the administration of clomiphene citrate, there were no changes in T4, T3 sephadex or total T3 levels and in basal or stimulated TSH levels. Clomiphene did produce an increase in oestradiol, testosterone, basal gonadotrophins and LH response to LHRH. Since the oestrogen antagonist, clomiphene citrate, had no effect on TSH secretion, it is unlikely that the exaggerated TSH response to TRH is mediated by oestrogens.