bluuebunny/crossref_metadata_2025_split huggingface dataset:
ADDISON'S DISEASE PRESENTING AS A HYPERCALCAEMIC CRISIS IN A PATIENT WITH IDIOPATHIC HYPOPARATHYROIDISM
Authors: WALKER D. A. , DAVIES M. 
Published: March 2008

SUMMARYA patient with idiopathic hypoparathyroidism complicated by Addison's disease is described. The development of adrenal insufficiency was accompanied by a hypercalcaemic crisis. The hypercalcaemia appeared to result from a reduction in the filtered load and enhanced renal tubular reabsorption of calcium. Cortisone therapy was followed by the characteristic renal tubular leak of calcium associated with hypoparathyroidism and a return of the serum calcium to normal. The possible mechanisms for these changes in calcium homeostatis are discussed.
DIFFERENT ASPECTS OF 5α‐REDUCTASE DEFICIENCY IN MALE PSEUDOHERMAPHRODITISM AND HYPOTHYROIDISM
Authors: MAUVAIS‐JARVIS P. , KUTTENN F. , MOWSZOWICZ IRÈNE , WRIGHT FRANÇOISE 
Published: March 2008

SUMMARYThe 5α‐reductase activity that mediates the transformation of testosterone to dihydrotestosterone in various anatomical sites of human beings, has been studied in different pathological conditions related to 5α‐reductase deficiency. We have studied two patients with male pseudohermaphroditism due to 5α‐reductase deficiency, four patients with the complete form of testicular feminization syndrome and four men with primary hypothyroidism. Results were compared with those obtained in seven normal men. In vivo: radioactive tracers of testosterone were administered to each subject by different routes: intravenous, oral and subcutaneous. The urinary metabolites of these labelled precursors were measured. The 5β: 5α ratios of 17‐ketosteroids (aetiocholanolone: 5α‐androsterone) and androstanediols (5β‐androstane‐3α, 17β‐diol: 5α‐androstane‐3α, 17β‐diol) were calculated in the urine recovered after each mode of administration of radioactive testosterone. When testosterone was administered subcutaneously these ratios were highly increased in one patient with male pseudohermaphroditism due to 5α‐reductase deficiency. In all the other patients, the ratios were found to be in the normal range for men. After oral administration of radioactive testosterone, both 5β: 5α ratios were very high in hypothyroid and in 5α‐reductase deficient patients. These results suggest that the defective 5α‐reductase activity observed in hypothyroid patients is only localized in the hepatic compartment. Conversely, in male pseudohermaphroditism, the 5α‐reductase defect might affect both hepatic and extra‐hepatic compartments. In vitro: the diagnosis of 5α‐reductase deficiency was confirmed in the two male pseudohermaphrodite patients after incubation with 3H testosterone of skin homogenates from the external genital area. No 5α‐reduction of testosterone occurred in the two skin specimens studied. In contrast, 5α‐reductase activity was normal in genital skin from hypothyroid and testicular feminization syndrome patients. In pubic skin, 5α‐reductase activity was absent in patients with testicular feminization syndrome. It was in the normal range in homogenates from hypothyroid patients and varied in the 5α‐reductase deficient patients. Based on these data, it may be postulated that the programming of hepatic and extrahepatic 5α‐reductase enzymes is fundamentally different. In addition, the enzyme that mediates the appearance of secondary sex characteristics seems to be androgen dependent, while the 5α‐reductases present in the external genital area and the liver are not androgen dependent.
THE EFFECTS OF A MET‐ENKEPHALIN ANALOGUE ON ACTH, β‐LPH, β‐ENDORPHIN AND MET‐ENKEPHALIN IN PATIENTS WITH ADRENOCORTICAL DISEASE
Authors: GAILLARD R. C. , GROSSMAN A. , SMITH R. , REES LESLEY H. , BESSER G. M. 
Published: March 2008

SUMMARYThirteen patients with either Addison's disease, or Cushing's disease treated by bilateral adrenalectomy, were infused with the long‐acting met‐enkephalin analogue DAMME. In patients with Addison's disease significant and pronounced falls in ACTH and N‐ and C‐terminal β‐LPH were seen; chromatography suggested that β‐endorphin fell concomitantly. Three out of four patients with Cushing's disease who had not received pituitary irradiation, also showed a decrease in plasma ACTH and N‐ and C‐terminal β‐LPH; however, no change was seen in any of the irradiated patients. The changes were naloxone reversible. The levels of plasma met‐enkephalin were normal and did not change after DAMME in any group of patients. These results are interpreted as suggesting that there are inhibitory opiate receptors controlling the release of ACTH, β‐LPH, and β‐endorphin.
DOPAMINERGIC CONTROL OF KETOGENESIS IN FASTING
Authors: BLESA‐MALPICA G. , JOHNSTON D. G. , BURRIN JACKY M. , ØRSKOV H. , HEATH A. B. , ALBERTI K. G. M. M. 
Published: March 2008

SUMMARYThe role of dopamine in starvation ketonaemia was investigated in male Wistar rats by administration of a specific dopamine receptor antagonist, metoclopramide (4 mg.kg−1.24h−1), or placebo, intragastrically during a 48‐h fast. Starvation alone caused a fall in blood glucose and gluconeogenic precursor concentrations, which was unaffected by metoclopramide administration. Circulating 3‐hydroxybutyrate and acetoacetate levels rose with fasting alone but metoclopramide impaired this ketonaemic response. After 48‐h starvation, total ketone body concentrations (mean±SEM) were 2·28 ± 0·19 mmol/l with metoclopramide therapy, 3·49 ± 0·21 mmol/l with placebo, P < 0·001. Plasma non‐esterified fatty acid levels were similar in metoclopramide‐ and placebo‐treated animals, as were circulating concentrations of insulin, glucagon and growth hormone. Metoclopramide thus decreased the ketonaemic response to starvation without an apparent change in lipolysis or circulating hormone levels, suggesting a direct role for dopamine in production of starvation ketonaemia.
FAMILIAL CONGENITAL CUSHING'S SYNDROME DUE TO BILATERAL NODULAR ADRENAL HYPERPLASIA
Authors: DONALDSON M. D. C. , GRANT D. B. , O'HARE M. J. , SHACKLETON C. H. L. 
Published: March 2008

SUMMARYTwo siblings with congenital Cushing's syndrome due to bilateral nodular adrenal hyperplasia are described. The older, a boy, presented with severe hypertension and died soon after subtotal adrenalectomy. His sister, who had clitoral enlargement and showed persistent hyponatraemia, had a two‐stage total adrenalectomy and is still alive. Investigations in the second case showed grossly elevated urinary cortisol metabolites, 17‐oxosteroids and 3β‐hydroxy‐5‐ene‐steroids. These were not suppressed by dexamethasone, and plasma ACTH was undetectable, indicating that the disorder was not due to excessive ACTH secretion. Cell culture studies on the resected adrenals failed to demonstrate an abnormal pattern of steroid synthesis in vitro, and normal trophic responses were obtained with 1–24 ACTH and monobutyryl cyclic AMP. No stimulation of steroid synthesis was obtained with a range of polypeptide hormones, and the cause of the adrenal hyperplasia remains unknown.
THE EFFECT OF WEIGHT LOSS ON SEX STEROID SECRETION AND BINDING IN MASSIVELY OBESE WOMEN
Authors: KOPELMAN P. G. , WHITE N. , PILKINGTON T. R. E. , JEFFCOATE S. L. 
Published: March 2008

SUMMARYWe have previously reported increased testosterone and androstenedione concentrations and decreased sex hormone binding globulin (SHBG) concentrations in the plasma of massively obese women. We now report that these plasma hormone concentrations return to normal in twelve of the same women after substantial weight reduction and these changes are associated with more normal menstrual cycles. We conclude that body weight and fat are important determinants of sex steroid secretion and binding and thus influence menstrual function.
BROMOCRIPTINE AND OESTROGEN MODULATION OF GONADOTROPHIN RELEASE IN NORMO‐AND HYPERPROLACTINAEMIC PATIENTS WITH AMENORRHOEA
Authors: DJURSING H. , HAGEN C. , CHRISTENSEN F. , NICKELSEN C. 
Published: March 2008

SUMMARYEffects of oestradiol, bromocriptine, and bromocriptine plus oestradiol, on basal and GnRH stimulated gonadotrophin concentrations were studied in normo‐(group 1, n= 7) and hyperprolactinaemic (group 2, n= 6) patients with secondary amenorrhoea. Before drug administration, hyper‐responsiveness of LH, but normal FSH responses to GnRH were observed in most patients. Oral administration of 2 mg oestradiol for 4 days resulted in increased 17β‐oestradiol levels in plasma in normal women (n= 6) in the early follicular phase of the cycle, and in groups 1 and 2. During oestradiol administration plasma LH concentration increased significantly and there was an increase of LH and FSH responses to GnRH in normal subjects, but not in amenorrhoeic women. In groups 1 and 2 basal FSH levels were suppressed but no change in GnRH stimulated gonadotrophin responses was seen. Bromocriptine (5 mg per day for 5 days) significantly decreased prolactin concentrations and increased 17β‐oestradiol levels in plasma in group 2 but not in group 1. The mean plasma 17β‐oestradiol concentration had increased to levels similar to those obtained during oestradiol administration alone. The mean LH response to GnRH was suppressed in group 2, but not in group 1. Basal and GnRH‐stimulated plasma FSH concentrations were not changed by bromocriptine treatment. Compared with the GnRH induced LH response during bromocriptine alone, bromocriptine treatment plus oestradiol administration resulted in a significantly increased LH response in group 2. This was not found in group 1.The present results suggest that there is an increased dopamine activity and inhibition of GnRH at the hypothalamic level and a relative dopamine deficiency at the pituitary level in hyperprolactinaemic patients. Normoprolac‐tinaemic patients with hypothalamic amenorrhoea have increased dopaminergic activity at the hypothalamic, as well as the, pituitary level, or alternatively that the LH release is not influenced by dopamine in these patients. Finally bromocriptine sensitizes LH‐secreting cells to GnRH in hyperprolactinaemic, but not in normoprolactinaemic, patients.
NON‐SUPPRESSIBLE INSULIN‐LIKE ACTIVITY DURING PREGNANCY IN WOMEN WITH DIABETES MELLITUS
Authors: HERINGTON A. C. , MARTIN F. I. R. , McBAIN J. C. 
Published: March 2008

SUMMARYSerum levels of the growth factor non‐suppressible insulin‐like activity (NSILA‐S) have been measured in eleven pregnant women with diabetes mellitus during pregnancy and immediately (2 days) after birth. A significant increase in mean levels was observed with increasing gestation, reaching a peak at the time of delivery and subsequently returning to non‐pregnant levels within 2 days. The NSILA‐S levels in diabetic pregnancies were not different from those in non‐diabetic pregnancies and there were no significant correlations between NSILA‐S and birth weight, maternal diabetic control, age or duration of diabetes or the treatment required. The physiological role of high NSILA‐S levels during pregnancy still requires elucidation.
STUDIES ON OESTROGEN STIMULATED NEUROPHYSIN IN WOMEN WITH ANOVULATION
Authors: McHUGH ROSEMARY E. , MARVIN SUSAN C. , SHIRLEY ANNE , BUTT W. R. , LYNCH S. S. , SHAW R. W. 
Published: March 2008

SUMMARYThe effects of endogenous and exogenous oestrogen on serum concentrations of oestrogen stimulated neurophysin (ESN) were studied in women with regular menstrual cycles and patients with anovulation. In ten menstrual cycles serum ESN concentrations rose steadily from 305 ± 16 ng/l (mean ± SEM) in the early follicular phase to reach maximum concentrations at about the time of ovulation (601 ± 106 ng/l). There were significant correlations between ESN and oestradiol concentrations in nine of the ten cycles (P < 0·05). The concentration in the mid luteal phase (386 ± 43 ng/l) was also significantly greater than at the beginning of the cycle (P < 0·05).Stimulation of endogenous oestrogens by treatment with clomiphene was accompanied by a release of ESN. Oestrogen concentrations were significantly lower in 5 months in which ovulation did not occur than in ten treatments in which ovulation did occur (P < 0·05). The amount of ESN produced however, was similar in the two groups.The basal concentration of ESN was within the range found in the early follicular phase of the cycle in twenty of the thirty‐seven patients with anovulation in whom it was measured. Concentrations tended to be low in patients with anorexia nervosa or hyperprolactinaemia and oestrogen concentrations were also low. Levels were high in oligomenorrhoea but oestradiol concentrations were normal.Serum ESN concentrations in oestrogen provocation and amplification tests reached maximum values in normal subjects 48 h after the administration of oestradiol benzoate: LH concentrations continued to rise until 72 h. In twenty‐one of forty‐one provocation tests on anovular patients the amount of ESN released was within the range for normal controls: twelve released less and eight released more. There was no correlation between the basal ESN values and the ESN released.There was intact positive LH feedback in sixteen of the forty‐one tests performed. In all except two tests with positive LH feedback, ESN was also released, but in the twenty‐five tests with no evidence of LH positive feedback there was normal release of ESN in twelve, reduced release in ten and excessive release in three. In the amplification tests LHRH was without effect on the ESN concentrations before or after oestrogen administration.It is concluded that either the release of LH stimulated by oestrogen is independent of the release of ESN, or it is stimulated at different threshold levels of oestrogen. ESN is more readily released than LH and the mechanism is probably less involved.
LHRH TEST IN BARTTER'S SYNDROME BEFORE AND DURING TREATMENT WITH PROSTAGLANDIN SYNTHESIS INHIBITORS
Authors: LARIZZA DANIELA , LORINI RENATA , CISTERNINO MARIANGELA , SALVATONI A. , SEVERI FRANCESCA 
Published: March 2008

SUMMARYLHRH tests were performed in two children with Bartter's syndrome before and during treatment with prostaglandin synthesis inhibitors. Before treatment, in patient 1 both basal and peak FSH and LH levels were elevated; in patient 2 basal and peak FSH levels were above the normal range, while basal and peak LH levels were normal. During treatment with an inhibitor of prostaglandin synthesis, abnormal values decreased into the normal range. These results support a possible role of endogenous prostaglandins in regulating gonadotrophin secretion in humans.
RELATION OF hCG‐STIMULATED STEROIDOGENESIS TO SERUM FSH, LH AND PROLACTIN IN MAN
Authors: MARTIKAINEN H. 
Published: March 2008

SUMMARYNine adult healthy male volunteers were given a single intramuscular injection of 5000 iu of human chorionic gonadotrophin (hCG). Peripheral blood samples were collected up to 7 days after the injection, the sera were analysed for LH, FSH and prolactin and the results were related to changes in the concentrations of testosterone and oestradiol.Serum LH concentrations tended to decrease for 4 days after the hCG injection (to 65% of the 0 h value) but returned to the starting level during the 7‐day observation period. FSH was suppressed throughout the experiment and correlated inversely with serum prolactin (r = ‐0·74, P < 0·01). The first significant decrease was seen at 48 h and the minimum concentration (28 ± 4%, SEM) at 7 days. Prolactin levels changed only slightly during the first 48 h after the injection but increased thereafter, being significantly elevated at 4 days, and reached a maximum (183 ± 22%) at 6 days after hCG adminstration, coinciding with the late phase of the testosterone response to hCG. Between testosterone and prolactin a positive correlation (r = 0·70. P < 0·01) was found during hCG stimulation.The results suggest that endogenous steroid production of the human testis after hCG administration is sufficient to induce changes in gonadotrophin and prolactin secretion. It is further concluded that the feedback mechanism of FSH is more sensitive to increased testicular steroid output following hCG injection than that of LH. It is hypothesized that pituitary prolactin secretion is stimulated by the elevated serum oestradiol concentration at 24–48 h, and the long term testosterone response, observed 2–6 days after the hCG injection, may be partly due to increased prolactin concentrations.
STUDIES ON CIRCULATING MET‐ENKEPHALIN AND β‐ENDORPHIN: NORMAL SUBJECTS AND PATIENTS WITH RENAL AND ADRENAL DISEASE
Authors: SMITH R. , GROSSMAN A. , GAILLARD R. , CLEMENT‐JONES VICKY , RATTER SALLY , MALLINSON J. , LOWRY P. J. , BESSER G. M. , REES LESLEY H. 
Published: March 2008

SUMMARYStudies were performed to define the responses of plasma met‐enkephalin to various endocrine and pathological stimuli and to determine the relationship between plasma β‐endorphin and met‐enkephalin. During insulin‐induced hypoglycaemia ACTH, β‐LPH and β‐endorphin immunoreactivity rose in parallel, but plasma met‐enkephalin did not change significantly. Sephadex G75 chromatography of samples taken at the time of the peak response (45 min) confirmed the rise in both β‐LPH and β‐endorphin. During administration of dexamethasone, 0·5mg 6 hourly for 48 h, plasma cortisol and ACTH became undetectable at 24 h, and β‐LPH and β‐endorphin fell significantly by 24 h and were undetectable by 48 h; plasma met‐enkephalin, however, showed no significant change. Nine adrenalectomized patients with Cushing's disease and four patients with Addison's disease had elevated plasma ACTH, β‐LPH and β‐endorphin but normal plasma met‐enkephalin levels. Each of ten patients with renal failure had markedly elevated plasma met‐enkephalin immunoreactivity which co‐eluted with synthetic met‐enkephalin on BioGel P4 chromatography. Trypsin and carboxypeptidase‐B digestion of the P4 chromatography fractions generated met‐enkephalin immunoreactivity in earlier fractions, indicating the presence of a potential high molecular weight met‐enkephalin precursor.The results imply that different control mechanisms govern β‐endorphin and met‐enkephalin plasma levels and that the adrenal is not the only source of plasma met‐enkephalin. The elevated levels of met‐enkephalin in renal failure may be due to impaired clearance of met‐enkephalin or of its precursor or to increased met‐enkephalin production.
Γ‐MELANOTROPHIN‐LIKE IMMUNOREACTIVITIES IN HUMAN PITUITARIES, ACTH‐PRODUCING PITUITARY ADENOMAS, AND ECTOPIC ACTH‐PRODUCING TUMOURS: EVIDENCE FOR AN ABNORMALITY IN GLYCOSYLATION IN ECTOPIC ACTH‐PRODUCING TUMOURS
Authors: TANAKA I. , NAKAI Y. , NAKAO K. , OKI S. , FUKATA J. , IMURA H. 
Published: March 2008

SUMMARYUsing gel exclusion chromatography on Bio‐Gel P‐60, γ‐melanotropin‐like immunoreactivity (γ‐MSH‐LI) in three human pituitary glands, two ACTH‐producing pituitary adenomas, and three ectopic ACTH‐producing tumours (two medullary thyroid carcinomas and one thymoma) was divided into one or two molecular weight classes. The largest component eluted near the position of mouse 16K fragment and was designated big γ‐MSH‐LI. This big γ‐MSH‐LI was present in all samples. The second one, designated intermediate γ‐MSH‐LI, eluted between the position of mouse 16K fragment and human ACTH, and was demonstrated only in two ectopic ACTH‐producing tumours. No γ‐MSH‐LI emerged at the elution position of synthetic γ3‐MSH.Affinity chromatography on concanavalin A‐agarose revealed that a significant fraction (52–68%) of γ‐MSH‐LI from human pituitary glands, ACTH‐producing pituitary adenomas, and one ectopic ACTH‐producing tumour bound to the column and was eluted with α‐methyl‐D‐mannopyranoside. In two ectopic ACTH‐producing tumours which contained big and intermediate γ‐MSH‐LI, a relatively small fraction (27–35%) of γ‐MSH‐LI bound to the column and was similarly eluted. These observations suggest that human γ‐MSH‐LI is glycosylated and that there is an abnormality in the glycosylation of γ‐MSH‐LI in some ectopic ACTH‐producing tumours.
INHERITED THYROXINE EXCESS: A SERUM ABNORMALITY DUE TO AN INCREASED AFFINITY FOR MODIFIED ALBUMIN
Authors: DOCTER R. , BOS G. , KRENNING E. P. , FEKKES D. , VISSER T. J. , HENNEMANN G. 
Published: March 2008

SUMMARYFurther analysis of sera from euthyroid subjects with dominantly‐inherited, elevated serum total thyroxine (T4) and free T4 index but with normal free T4 levels was performed as an extension of a previous study (Hennemann et al., 1979a). Scatchard analysis and isoelectric focusing of whole sera and purified serum fractions suggest that this T4 excess is due to increased T4 binding by modified serum albumin.Recognition of this syndrome and appreciation that the free T4 index does not reflect the free T4 levels is important to protect patients from the consequences of an incorrect diagnosis of thyrotoxicosis.
EVIDENCE FOR SECONDARY HYPERPARATHYROIDISM IN THE OSTEOMALACIA ASSOCIATED WITH CHRONIC LIVER DISEASE
Authors: DIBBLE J. B. , SHERIDAN P. , HAMPSHIRE R. , HARDY G. J. , LOSOWSKY M. S. 
Published: March 2008

SUMMARYPrevious reports have suggested that secondary hyperparathyroidism is extremely uncommon in hepatic osteomalacia. This, together with other findings, has led to suggestions that in chronic liver disease there may be selective resistance of bone to vitamin D or a specific bone mineralization defect unrelated to Vitamin D.To examine these possibilities, twenty‐five patients with chronic liver disease have been studied by bone biopsy, serum calcium and inorganic phosphate, plasma 25‐hydroxyvitamin D, plasma immunoreactive parathormone (iPTH), fasting urine cAMP, fasting renal tubular maximal reabsorptive capacity for phosphate (TmP/GFR) and fine grain hand x‐rays. Nine of the patients had osteomalacia on bone biopsy, eight of these had subnormal levels of plasma 25‐hydroxyvitamin D and the other had a borderline result. Based on the concensus of all the tests, five of these had evidence of secondary hyperparathyroidism. Plasma iPTH was higher in patients with osteomalacia than in patients without osteomalacia (P < 0·01) or controls (P < 0·01). Urine cAMP was higher in patients with osteomalacia than in patients without osteomalacia (P < 0·001) or controls (P < 0·01). TmP/GFR was significantly lower in patients with osteomalacia than in controls (P < 0·05) but not significantly different from patients without osteomalacia.The findings of this study indicate that hyperparathyroidism occurs in a substantial proportion of patients with the osteomalacia of chronic liver disease. Moreover, osteomalacia in chronic liver disease is clearly related to reduced levels of plasma 25‐hydroxyvitamin D. We conclude that hepatic osteomalacia is a vitamin D deficiency state and there is no need to suggest an unusual aetiology.
FAILURE OF 2‐HYDROXYOESTRONE TO LOWER PROLACTIN CONCENTRATIONS IN HYPERPROLACTINAEMIC WOMEN
Authors: FRANKS S. , LIGHTMAN S. L. , MACLUSKY N. J. , NAFTOLIN F. , LYNCH S. S. , BUTT W. R. , JACOBS H. S. 
Published: March 2008

SUMMARYThe catechol oestrogen 2‐hydroxyoestrone has been reported to lower serum prolactin concentrations acutely in normal women and it has been proposed that it may be effective in suppressing prolactin secretion in hyperprolactinaemic patients. Five women with hyperprolactinaemia and anovulation were studied. Following a control infusion, 2‐hydroxyoestrone was given at a rate of 80 μg/h for 4 h. In no patient was there a fall in prolactin levels and no changes were observed in gonadotrophin concentrations. We conclude that a short‐term infusion of 2‐hydroxyoestrone, at a dose which will produce high blood levels of the steroid, does not affect prolactin or gonadotrophin secretion in hyperprolac‐tinaemic states.