bluuebunny/crossref_metadata_2025_split huggingface dataset:
FAMILIAL HYPOCALCIURIC HYPERCALCAEMIA: ASSOCIATION WITH NEONATAL PRIMARY HYPERPARATHYROIDISM, AND POSSIBLE LINKAGE WITH HLA HAPLOTYPE
Authors: SOPWITH A. M. , BURNS CLAIRE , GRANT D. B. , TAYLOR G. W. , WOLF EVA , BESSER G. M. 
Published: March 2008

SUMMARYA kindred is described, six members of which fulfilled criteria for the syndrome of familial hypocalciuric hypercalcaemia (FHH). The majority of these individuals were asymptomatic: their hypercalcaemia was revealed by biochemical screening after parathyroid surgery had failed to diminish the elevated serum calcium concentration of the index case. In contrast to those members of the family diagnosed as adults, the daughter subsequently born to the index case included in her expression of FHH clinical features of severe neonatal primary hyperparathyroidism. Four hyperplastic parathyroid glands were excised, and following surgical intervention the baby improved clinically and accelerated the healing of her skeletal lesions. Her case suggests that an association does exist between FHH and neonatal primary hyperparathyroidism. Of the ten individuals at risk, inheritance of hypocalciuric hypercalcaemia was paralleled in eight cases by inheritance of the HLA haplotype All BW55 CW3 DR4. Such possible linkage has not previously been reported.
THE EFFECT OF OESTROGEN (MESTRANOL) ON THE THYROTROPHIN RESPONSE TO THYROTROPHIN RELEASING HORMONE IN POSTMENOPAUSAL OOPHORECTOMISED WOMEN
Authors: ABDALLA H. I. , BEASTALL G. H. , HART D. M. 
Published: March 2008

SUMMARYThe thyrotrophin (TSH) responses to thyrotrophin releasing hormone (TRH) in 16 oophorectomised postmenopausal women on long term treatment with mestranol 24 μg daily were compared to those in 16 oophorectomised postmenopausal women on placebo treatment. No significant difference between the groups was found in either basal TSH levels or the TSH response to TRH. It is therefore unlikely that the difference in TSH response to TRH between men and women of reproductive age is due to a direct stimulatory effect of oestrogen in women.
ANTITHYROID TREATMENT OF MATERNAL HYPERTHYROIDISM DURING LACTATION
Authors: LAMBERG B.‐A. , IKONEN E. , ÖSTERLUND K. , TERAMO K. , PEKONEN F. , PELTOLA J. , VÄLIMÄKI M. 
Published: March 2008

SUMMARYEleven pregnant women were treated for hyperthyroidism with carbimazole (CZ) and one with propylthiouracil (PTU). Based upon a previous study it was decided that lactation should be permitted if the dose required after delivery did not exceed 15 mg of CZ or 150 mg of PTU. In the patients studied here the daily dose of CZ varied from 5 to 15 mg and that of PTU was 125 mg. TSH was measured in cord blood and in the blood of the newborn infants usually after 2 and 3 weeks of lactation. Serum T4 was measured serially in the infants' blood from day 4 up to 21 day of age, at least. In all instances the TSH concentration in cord blood remained below 45 mU/1 the level used in screening for neonatal hypothyroidism. Serum TSH and T4 were all within the appropriate reference limits during the 3 weeks of study with only one exception. In the infant whose mother was treated with PTU the serum T4 measured 5 d after birth was slightly below the lower limit but later returned to normal. Since serum TSH and T4 did not deviate from the reference range in newborn infants during lactation, we conclude that breast‐feeding can be permitted if the daily dose of CZ does not exceed 15 mg (or 150 mg of PTU) and if facilities are available for measuring neonatal serum TSH and T4.
THE ROLE OF ENDOGENOUS OPIOIDS IN THE CHLORPROPAMIDE ALCOHOL FLUSH
Authors: JOHNSTON C. , WILES P. G. , MEDBAK S. , BOWCOCK S. , COOKE E. D. , PYKE D. A. , REES LESLEY H. 
Published: March 2008

SUMMARYThe response of plasma immunoreactive met‐enkephalin (IR‐met‐enkephalin) to ethanol (8 g by mouth) after chlorpropamide (250 mg daily for 14 d) was studied in three groups of non‐insulin dependent diabetics (a) six diabetics who showed chlorpropamide alcohol flush (CPAF) and in whom the reaction could be blocked by indomethacin, (b) five diabetics who showed CPAF but in whom the flush could not be blocked by indomethacin and (c) five diabetics who did not show CPAF. A rise in plasma IR‐met‐enkephalin was observed in all three groups. When the two groups of flushers were re‐tested with the addition of an infusion of naloxone a rise in plasma IR‐met‐enkephalin was still demonstrated in both groups regardless of whether the flush was blocked by naloxone. Naloxone blocked the flush only in those six subjects whose flush could be blocked by indomethacin. In five subjects, all flushers, CPAF was tested using intravenous and oral ethanol in doses producing similar plasma ethanol levels. A facial flush was induced by both intravenous and oral ethanol. In three flushers, plasma IR‐met‐enkephalin levels were measured during CPAF testing with both intravenous and oral ethanol. None showed a rise in plasma IR‐met‐enkephalin after intravenous ethanol, despite the appearance of a facial flush, whereas all showed a rise after oral ethanol. We therefore conclude that CPAF is unlikely to be caused by a rise in plasma IR‐met‐enkephalin.
APPARENT MINERALOCORTICOID EXCESS AND DEFICIENT 11β‐OXIDATION OF CORTISOL IN A YOUNG FEMALE
Authors: HARINCK H. I. J. , VAN BRUMMELEN P. , VAN SETERS A. P. , MOOLENAAR A. J. 
Published: March 2008

SUMMARYA 19‐year‐old female, known to have had hypertension and hypokalemic alkalosis since the age of 9 months, was found to have suppressed renin, negligible plasma and urinary aldosterone and low plasma levels of other known sodium‐retaining steroids. Despite the normal plasma cortisol the urinary excretion of 17‐oxosteroids and 17‐oxogenic steroids was low as was the cortisol secretion rate, suggesting a diminished metabolic clearance of cortisol. This was confirmed by the demonstration of a prolonged t1/2 of 14C‐cortisol. The abnormally high urinary excretion ratios of cortisol to cortisone, tetrahydracortisol to tetrahydrocortisone and 11‐hydroxy‐aetiocholanolone to 11‐oxy‐aetio‐cholanolone indicate that the diminished cortisol breakdown is the result of deficient 11β‐oxidation. Moreover, the urinary excretion of free cortisol was elevated, probably due to diminished tubular reabsorption of cortisol. Hypokalemic alkalosis did not respond to spironolactone, but was partly corrected by amiloride. No response to dexamethasone was observed, but dexamethasone combined with aminogluthetimide normalized blood pressure and serum K. These findings support the involvement of a sodium‐retaining, kaliuretic steroid in this rare syndrome.
INHIBITORY EFFECT OF CIMETIDINE ON L‐DOPA‐STIMULATED GROWTH HORMONE RELEASE IN NORMAL MAN
Authors: ZANOBONI A. , GALMOZZI G. , MARINONI S. , ZANOBONI‐MUCIACCIA WANDA 
Published: March 2008

SUMMARYSome evidence suggests the existence of a histaminergic influence on GH secretion in animals and man. We used cimetidine, a specific H2‐receptor antagonist, to study the possible interference of H2‐receptor blockade on plasma GH release by L‐dopa and on PRL inhibition by L‐dopa in normal man. Seven healthy normal male volunteers aged 23–36 years received a single oral dose of L‐dopa (500 mg) or an i.v. bolus of cimetidine (300 mg) or both (L‐dopa 30 min before cimetidine). Blood samples were taken at various times over 2 h and plasma GH and PRL levels measured. Cimetidine alone did not alter basal plasma GH values; L‐dopa elicited the well‐known GH releasing effect with peak values at 75 min (15.65 ± 2.8 ng/ml); cimetidine injected 30 min after L‐dopa ingestion significantly blunted the GH response to L‐dopa and peak values (4.7 ± 1.6 ng/ml) were delayed to 105 min. Cimetidine provoked a rapid rise in plasma PRL with the peak value of 15 ± 3 ng/ml at 15 min, followed by a return to near basal values in 90–120 min. L‐Dopa completely inhibited the PRL response to cimetidine. We conclude that there is an inhibitory influence of the H2‐receptor antagonist cimetidine on GH release by L‐dopa. This, together with the action of cimetidine on PRL secretion (with or without L‐dopa), suggests a possible antidopaminergic effect of H2‐receptor blockade at the level of the central nervous system.
TOTAL DEFICIENCY OF CORTICOSTEROID‐BINDING GLOBULIN
Authors: ROITMAN A. , BRUCHIS S. , BAUMAN B. , KAUFMAN H. , LARON Z. 
Published: March 2008

SUMMARYAn obese 15‐year‐old boy of Jewish Iranian origin who is the offspring of consanguineous parents was found to have very low levels of total cortisol in the plasma. Investigation of the family revealed a complete lack of cortisol‐binding‐globulin (CBG) in the proband and a sister, evidently the first cases of total CBG deficiency to be reported. The parents and a brother were found to have half the normal levels. This study indicates that CBG deficiency, a benign condition, is compatible with a codominant or recessive autosomal trait inheritance.
INFLUENCE OF DIETARY CALCIUM ON SERUM 1,25‐DIHYDROXYVITAMIN D CONCENTRATIONS IN RENAL STONE FORMERS
Authors: D'AMOUR P. , GASCON‐BARRÉ MARIELLE , DUFRESNE L. , PERREAULT J. ‐P. 
Published: March 2008

SUMMARYThe role of 1,25‐dihydroxyvitamin D (1,25(OH)2D) in the pathogenesis of idiopathic hypercalciuria was studied in 37 renal stone formers who, during two 10‐day periods, followed first a normal and then a low calcium diet. The following samples were taken during each diet; (1) 24 h urine; (2) fasting blood and urine; (3) blood and urine following a 1 g oral calcium load. Patients were divided according to serum calcium level, 24 h urinary calcium excretion on the first diet and fasting calcium excretion on the second diet. Eight patients were found to be normocalciuric (NSF), 16 had absorptive hypercalciuria (AH), five renal hypercalciuria (RH) and eight primary hyperparathyroidism. In NSF and AH, a positive correlation was found between the fasting and the 24 hour urinary calcium (r = 0.787, P<0.001), while negative correlations were found between the fasting urinary calcium and the serum parathyroid hormone (r= ‐0.703, P<0.001) or the fasting urinary cyclic AMP (r= ‐0.434, P < 0.01). Patients with RH had higher serum PTH and urinary cAMP levels for a given degree of fasting calciuria mainly on the low calcium diet. Mean serum 1,25(OH)2D was similar in NSF (43.6 ± 4.5 pg/ml), AH (43.6 ± 2.3 pg/ml) and RH (40.4 ± 4.8 pg/ml) on the first diet; increases were similar in all groups after 10 d of calcium restriction. A positive correlation was found between the serum 1,25(OH)2D concentrations and the 24 h urinary calcium excretion on the first diet in NSF (r = 0.889, P<0.001) but not in AH or RH. There was no evidence of such correlation with the low calcium diet. No correlation between the calciuric response to calcium loading and the serum concentrations of 1,25(OH)2D was found. The results suggest that serum concentrations of 1,25(OH)2D may be related to urinary calcium excretion in NSF more than in AH or RH. The factors responsible for the hyperabsorption of calcium in the latter patients remain to be elucidated.
OVARIAN STEROIDOGENESIS IN AN ADRENALECTOMIZED GIRL WITH 21‐HYDROXYLASE DEFICIENCY
Authors: ZACHMANN M. , MANELLA B. , KEMPKEN B. , KNORR‐MUERSET G. , ATARES M. , PRADER A. 
Published: March 2008

SUMMARYA girl with congenital adrenal hyperplasia due to 21‐hydroxylase deficiency could not be controlled by conventional treatment, and was adrenalectomized at age 8.5 years (bone age 13.5 years). After surgery, puberty and menarche occurred. On replacement therapy, her progress was uneventful up to the age of 16 years, when menstruations ceased and signs of virilization reappeared. Testosterone, androstenedione, and 17‐hydroxyprogesterone in plasma, and pregnanetriol in urine were high, but DHEA in plasma, and pregnenetriol and pregnanetriolone in urine were low. Oestrogens in plasma were normal. There was no steroid response to ACTH, and marked, but somewhat slow suppression by dexamethasone. HMG induced a strong rise in oestrone and oestradiol. Ethinyloestradiol reduced not only oestradiol in plasma, but also testosterone, androstenedione, and 17‐hydroxyprogesterone. With subsequent dexamethasone treatment, menstruation restarted, and the values returned to normal. It is concluded that virilization may reoccur in patients with 21‐hydroxylase deficiency even after adrenalectomy, and that the ovaries in this patient contain some tissue, which has properties of adrenal (suppressibility by dexamethasone) and ovarian tissue (suppressibility by ethinyl oestradiol, preference for Δ4‐pathway, low steroid 11‐oxygenation) at the same time.
ACUTE DOPAMINERGIC BLOCKADE BY SULPIRIDE STIMULATES β‐ENDORPHIN SECRETION IN PREGNANT WOMEN
Authors: SAMRA A. B. ABOU , PUGEAT M. , DECHAUD H. , NACHURY L. , TOURNIAIRE J. 
Published: March 2008

SUMMARYThe pars intermedia of the pituitary gland is well developed in most animal species but rudimentary in adult human subjects. Unlike the anterior pituitary, the intermediate lobe metabolizes proopiomelanocorticotrophin (POMC) mainly to β‐endorphin and to α‐melanotrophin (α‐MSH). This activity of the pars intermedia is under an inhibitory dopaminergic control. Biochemical and immunohistochemical evidence suggests the hyperplasia of the intermediate lobe during human pregnancy. The present study demonstrates that an acute dopaminergic blockade by sulpiride dramatically increases plasma concentrations of β‐endorphin in pregnant women but not in non‐pregnant women providing functional evidence for the intermediate lobe hypothesis.
DIFFERENTIAL EFFECTS OF OVINE AND HUMAN CORTICOTROPHIN‐RELEASING FACTOR IN HUMAN SUBJECTS
Authors: HERMUS A. R. M. M. , PIETERS G. F. F. M. , PESMAN G. J. , BUYS W. C. A. M. , SMALS A. G. H. , BENRAAD T. J. , KLOPPENBORG P. W. C. 
Published: March 2008

SUMMARYTen healthy subjects received 200 μg of human CRF (hCRF) and 200 μg of ovine CRF (oCRF) as an intravenous bolus injection on two different occasions. After hCRF plasma ACTH levels rose significantly (P< 0.0005, by Friedman's nonparametric analysis of variance) from a basal value of 35 ± 3 pg/ml (mean ± SEM) to a peak value of 80 ± 7 pg/ml 30 min after hCRF administration. This ACTH response was followed by a rise in plasma cortisol levels (P< 0.0005, by Friedman's test) from a baseline value of 0.32 ± 0.03 /μmol/l to a peak value of 0.56 ± 0.02 /μmol/l 60 min after hCRF. Ovine CRF elicited similar rises in the plasma ACTH and cortisol levels. However, as derived from the faster rate of decline of ACTH and cortisol after hCRF than after oCRF, human CRF had a significantly shorter duration of action than ovine CRF in humans. Human CRF not only stimulated ACTH release by the human pituitary gland but also prolactin release. After hCRF administration prolactin levels rose significantly (P< 0.005, by Friedman's test) from a basal value of 179 ± 18 mU/l to a peak value of 288 ± 34 mU/l at 10 min.
CONTRASTING EFFECTS OF SUBCUTANEOUS PULSATILE GnRH THERAPY IN CONGENITAL ADRENAL HYPOPLASIA AND KALLMANN'S SYNDROME
Authors: GORDON D. , COHEN H. N. , BEASTALL G. H. , HAY I. D. , THOMSON J. A. 
Published: March 2008

SUMMARYA patient with congenital adrenal hypoplasia (AH) and hypogonadotrophic hypogonadism was treated with pulsatile subcutaneous GnRH therapy for 16 weeks in an attempt to induce puberty. No rise in serum LH or FSH concentrations occurred despite increasing doses of GnRH (2.8 μg/pulse‐22.4 μg/pulse). In contrast a similar programme of therapy successfully initiated the biochemical changes of puberty in a patient with Kallmann's syndrome. Both patients before therapy had low basal serum LH and FSH concentrations with blunted LH and FSH responses to GnRH stimulation. After 1 week, serum LH and FSH rose into the normal adult range in the patient with Kallmann's syndrome. This study fails to confirm a previous report which suggested that intermittent low dose GnRH therapy may be of value in inducing puberty in AH. The reasons for the difference of pituitary responsiveness to GnRH in AH and Kallmann's syndrome are unclear at present.
THE INFLUENCE OF ADRENERGIC DENERVATION ON THE RESPONSE TO FEEDING OF THE GASTROENTEROPANCREATIC SYSTEM IN MAN
Authors: FRIER B. M. , CORRALL R. J. M. , ADRIAN T. E. , BLOOM S. R. , STRACHAN R. K. , HEADING R. C. 
Published: March 2008

SUMMARYThe effect of adrenergic denervation on the metabolic and hormonal responses to a standard meal was studied in six tetraplegic subjects (pre‐ganglionic sympathectomy) and compared with six normal subjects. Gastric emptying, estimated by isotope scintiscanning, was similar in both groups. Following the meal the mean blood glucose and plasma insulin rose in both groups, but were higher and remained elevated for longer in the tetraplegic subjects. There were no significant differences in the secretion of pancreatic glucagon, enteroglucagon, gastric inhibitory peptide (GIF) and neurotensin between the two groups. The mean basal and post‐prandial levels of pancreatic polypeptide and motilin were significantly higher in the tetraplegic group. This study confirms the presence of mild glucose intolerance in tetraplegic subjects which is not explicable on the basis of abnormal insulin or pancreatic glucagon secretion or abnormal gastric emptying. In addition the results provide evidence for a sympathetic inhibitory influence on the secretion of pancreatic polypeptide and motilin in man, which is probably adrenergically‐mediated.
HYPERINSULINAEMIA AND INSULIN RESISTANCE OF CIRRHOSIS: THE IMPORTANCE OF INSULIN HYPERSECRETION
Authors: PROIETTO J. , DUDLEY F. J. , AITKEN P. , ALFORD F. P. 
Published: March 2008

SUMMARYThe mechanism for the hyperinsulinaemia in cirrhosis was investigated using two different approaches. In the first study, the metabolic clearance rate of insulin was measured at steady state in 13 cirrhotic and 13 weight‐matched control subjects. With comparable insulin infusion rates (1.00 ± 0.19 versus 1.07 ± 0.15 mU/kg/min), steady‐state plasma insulin concentrations (104 ± 25 versus 87 ± 12 μU/ml; P>0.5) and MCRIRI (13.6 ± 1.6 versus 15.4 ± 2.0 ml/kg/min; P>0.5) were similar. In the second study, fasting and oral glucose stimulated C‐peptide/insulin ratios were compared in 16 cirrhotic and 18 weight matched control subjects. Although fasting glucose levels were significantly higher in the cirrhotic groups, all values were in the normal range (5.5 ± 0.3 versus 4.8 ± 0.1 mmol/l, P < 0.02). However, fasting insulin (0.171 ± 0.02 versus 0.068 ± 0.004 nmol/l) and C‐peptide (1.02 ± 0.13 versus 0.42 ± 0.02 nmol/l) were strikingly higher (P< 0.001) in cirrhotic subjects. On the other hand, fasting C‐peptide/insulin ratios were not statistically different in the two groups (6.18 ± 0.52 versus 6.77 ± 0.46; P>0.3). This suggests that beta cell hypersecretion was the principal cause of the fasting hyperinsulinaemia, rather than decreased insulin hepatic extraction. Following the glucose load in 13 of the control and seven of the cirrhotic group, the C‐peptide/insulin ratio fell in both groups but was significantly lower in the cirrhotic compared to control subjects at 30, 60 and 120 min, consistent with possible impairment of hepatic insulin extraction. We conclude that, in cirrhosis, fasting hyperinsulinaemia is due to beta cell hypersecretion, whereas at higher insulin secretion rates following a glucose load, reduced hepatic insulin extraction may also contribute to the hyperinsulinaemia.
STUDIES ON THE MECHANISMS OF SOMATOSTATIN RELEASE AFTER INSULIN INDUCED HYPOGLYCAEMIA IN MAN
Authors: WEBB SUSAN , LEVY I. , WASS J. A. H. , LLORENS A. , PENMAN ERICA , CASAMITJANA ROSER , WU PING , GAYA JOAN , MARTINEZ M. J. , RIVERA FRANCISCA 
Published: March 2008

SUMMARYInsulin‐induced hypoglycaemia, which stimulates gastric acid secretion, is associated with an increase in circulating somatostatin levels in man. In order to assess the mechanisms involved in this rise, six normal volunteers connected to a Biostator for continuous glucose monitoring were studied, on three separate occasions. On each occasion after basal blood sampling, 0.15 i.u. /kg body weight of insulin was administered i.v. and further samples were obtained intermittently over 150 min. On one occasion, dextrose was infused by the Biostator to prevent hypoglycaemia, while on the other two, a constant infusion of either normal saline or the specific H2 antagonist cimetidine was administered. Insulin plus dextrose caused no significant changes in circulating somatostatin levels, whereas insulin plus saline was associated with a marked, sustained and significant rise in all subjects; insulin plus cimetidine also produced a rise but it was delayed; the area under the curve was significantly (P<0.05) greater with insulin plus saline than with insulin plus cimetidine. These results show that in man insulin itself does not stimulate somatostatin secretion directly, but indirectly via hypoglycaemia. Further, the inhibition of gastric acid secretion with cimetidine reduces somatostatin release during insulin‐induced hypoglycaemia. This suggests that gastric acid may mediate somatostatin secretion associated with insulin‐induced hypoglycaemia.
INSULIN RESISTANCE IN CIRRHOSIS: EVIDENCE FOR A POST‐RECEPTOR DEFECT
Authors: PROIETTO J. , NANKERVIS ALISON , AITKEN PATRICIA , DUDLEY F. J. , CARUSO GLORIA , ALFORD F. P. 
Published: March 2008

SUMMARYReceptor and post‐receptor abnormalities of insulin action and their possible role in the insulin resistance of cirrhosis were examined in eight biopsy‐proven cirrhotic subjects and eight age‐weight matched healthy volunteers. To this end, oral glucose tolerance tests (OGTT), insulin dose response curves and insulin binding to circulating monocytes were determined for each subject. The dose‐response curves for the cirrhotic subjects were significantly shifted to the right compared to the control subjects, indicating the presence of insulin insensitivity (ED50 223 ± 30 versus 64 ± 8 mU/l respectively; P<0.001). The magnitude of the right shift of the insulin‐dose response curves correlated significantly (P< 0.001) with the OGTT 2 h insulin levels (r = 0.74) and the insulin areas under the OGTT curves (r = 0.86). In contrast, insulin responsiveness was marginally elevated in the cirrhotic group (maximal glucose disposal 680 ± 47 versus 574 ± 21 ml/m2/min; P<0.05). Insulin binding to circulating monocytes was normal in the cirrhotic subjects. It is concluded that the insulin resistance of cirrhosis is due to a post‐receptor defect in insulin action which reduces insulin sensitivity but not insulin responsiveness.