bluuebunny/crossref_metadata_2025_split huggingface dataset:
Circulating levels of soluble CD30 are increased in patients with systemic sclerosis (SSc) and correlate with serological and clinical features of the disease
Authors: GIACOMELLI R , CIPRIANI P , LATTANZIO R , DI FRANCO M , LOCANTO M , PARZANESE I , PASSACANTANDO A , CIOCCI A , TONIETTI G 
Published: November 2003

SUMMARY
Activated Th2 lymphocytes express the surface molecule CD30 and release a soluble form of the same molecule which can be detected both in vivo and in vitro. In the present study, high levels of soluble CD30 were found in the peripheral blood of patients with SSc, and a significant correlation with skin score and erythrocyte sedimentation rate (ESR) was detected. Furthermore, we observed a higher spontaneous release of soluble CD30 in the supernatants of unstimulated cultures of peripheral blood mononuclear cells from our patients compared with healthy controls. Taken together, these data suggest a possible involvement of Th2 cells in the immunopathogenesis of SSc, and the dosage of CD30 soluble in the peripheral blood may be helpful in following the outcome of the disease.
Failure of plasmapheresis, corticosteroids and thionamides to ameliorate a case of protracted amiodarone‐induced thyroiditis
Authors: Samaras Katherine , Marel Geoffrey M. 
Published: November 2003

We report a case of amiodarone‐induced thyrotoxicosis of protracted duration, unresponsive to conventional thionamide therapy, with therapy limited by severe adverse drug reactions. Other treatment modalities included high dose corticosteroids, plasmapheresis, lithium and perchlorate. Temporary amelioration was achieved following plasmapheresis; however, this and other measures were unsuccessful in controlling the thyrotoxicosis, which deteriorated to thyroid storm. Histopathologically, a degenerative, inflammatory thyroiditis was evident. We discuss the limitations of conventional drug therapy and the lack of a sustained response to plasmapheresis. The failure of high dose steroids to alter the course of illness and to completely suppress the thyroidal inflammatory process is highlighted. A potential role for renal and hepatic impairment in the observed protracted course of amiodarone‐induced thyrotoxicosis is suggested.
The identification of false positive responses to the pentagastrin stimulation test in RET mutation negative members of MEN 2A families
Authors: Marsh Debbie J. , McDowall Deborah , Hyland Valentine J. , Andrew Scott D. , Schnitzler Margaret , Gaskin Elizabeth L. , Nevell David F. , Diamond Terrence , Delbridge Leigh , Clifton‐Bligh Phillip , Robinson Bruce G. 
Published: November 2003

OBJECTIVE The pentagastrin stimulation test is the traditional test used for the identification of asymptomatic individuals in multiple endocrine neoplasia type 2A (MEN 2A) and familial medullary thyroid carcinoma (FMTC). The identification of mutations in the RET proto‐oncogene segregating with the disease phenotype in MEN 2A and FMTC families has made it possible to re‐examine the validity of using this test for the identification of affected family members.DESIGN Sequential and single pentagastrin stimulation test data were collected following the identification of RET mutation positive and RET mutation negative members of families with MEN 2A or FMTC.PATIENTS RET mutations were identified in 16 Australian and New Zealand MEN 2A or FMTC families. An analysis of 39 individuals from these families was included in this study. Thirty‐two individuals (14 males, 18 females) had previously been determined as RET mutation negative. Seven individuals (6 males, 1 female) had previously been determined as RET mutation positive. Two RET mutation negative males had thyroidectomy based on prior pentagastrin test results.MEASUREMENTS Serum calcitonin levels in response to stimulation with pentagastrin were measured at 0, 1, 2, 5 and 10 minutes post injection. Mutation analysis of the RET proto‐oncogene was performed in all individuals. In two RET mutation negative individuals from two MEN 2A families, thyroidectomy was performed and C‐cells were quantitated in order to determine the diagnosis of C‐cell hyperplasia.RESULTS There was a statistically significant difference (P < 0.013) between RET mutation negative male and female mean peak calcitonin responses of 282 ± 236 and 96 ± 62 (mean ±SD) ng/l respectively. False positive responses to pentagastrin stimulation were identified in seven individuals who were RET mutation negative in two of the 16 families. Histologic examination of the thyroid glands in the two RET mutation negative individuals who had thyroidectomy demonstrated C‐cell hyperplasia in one but not in the other.CONCLUSIONS There is considerable overlap between pentagastrin test results in individuals who are RET mutation positive and those who are RET mutation negative. These results indicate a need for routine performance of RET proto‐oncogene analysis on all individuals at risk of developing MEN 2A or FMTC and a coupling of pentagastrin test results and RET proto‐oncogene analysis in the decision to proceed with thyroidectomy.
Longitudinal evaluation of the development of salivary cortisol circadian rhythm in infancy
Authors: Santiago Luciano B. , Jorge Salim M. , Moreira Ayrton C. 
Published: November 2003

OBJECTIVE Although an outstanding characteristic of the adrenocortical function of children and adults is its circadian rhythm, little information is available about the age of appearance of such rhythm in infancy. The main obstacle has been the ethical difficulty in obtaining serial blood samples from healthy infants. We monitored the development of cortisol daily rhythm using non‐invasive salivary cortisol determination.DESIGN AND PATIENTS A longitudinal study of a group of 9 healthy infants was performed. Salivary samples were obtained in the morning, afternoon and night at 2, 4, 8, 12, 16, 20 and 24 post‐natal weeks from all infants.MEASUREMENTS Cortisol was determined by RIA in 25‐μl salivary samples. Two techniques based on assay coefficients of variation were employed to characterize a normal circadian pattern of cortisol.RESULTS Five infants (55%) established and maintained their cortisol rhythm as early as at 2, 4 and 8 weeks of age. In the remaining 4 infants the age of appearance was 12 and 20 weeks. This rhythm emerged in the group as a whole at a mean age of 8 weeks.CONCLUSIONS Our data suggest that, in most normal infants, the development of hypothalamic–pituitary–adrenal axis circadian maturation may occur at a much earlier age than previously described.
Normal human follicle development: an evaluation of correlations with oestradiol, androstenedione and progesterone levels in individual follicles
Authors: Thierry van Dessel H. J. H. M. , Schipper Izaak , Pache Thierry D. , Van Geldorp Hans , De Jong Frank H , Fauser Bart C. J. M. 
Published: November 2003

OBJECTIVE The mechanism of dominant follicle selection remains obscure.We have investigated the association between follicle diameter and follicular steroid levels in individual human ovarian follicles throughout the menstrual cycle.DESIGN Fluid from ovarian follicles (n = 326) was obtained in vivo during surgery from 55 regularly cycling women with proven fertility. Follicles were divided into dominant (diameter > 9 mm, n = 45) and non‐dominant (diameter ≤ 9 mm, n = 281) based on ultrasound measurements.MEASUREMENTS Fluid was assayed for oestradiol (E2), androstenedione (AD), and progesterone (P).RESULTS Median P and E2 levels were significantly lower (P < 0.0001) and AD levels significantly higher (P = 0.03) in non‐dominant as compared to dominant follicles. In non‐dominant follicles AD (r = 0.14, P = 0.02), but not P and E2, levels were correlated to follicular diameter, and significant changes in steroid concentrations across the menstrual cycle were absent. In dominant follicles, diameter was positively correlated with P and E2 (P < 0.001) levels, and inversely correlated with AD concentrations (P = 0.01).CONCLUSIONS Results indicate that (1) intrafollicular oestradiol concentrations rise only in follicles exceeding 9 mm in diameter and correlate with the diameter of these dominant follicles, suggesting that significant increase in aromatase enzyme activity occurs only in the dominant follicle (2) a cycle‐independent accumulation of andro‐stenedione with size occurs in non‐dominant follicles, and (3) progesterone production occurs in the largest dominant follicles only, suggesting a limited, if any, role for progesterone during follicle development.
OVERNIGHT DEXAMETHASONE PRE‐TREATMENT IMPROVES THE PERFORMANCE OF THE LYSINE‐VASOPRESSIN TEST IN THE DIAGNOSIS OF CUSHING'S SYNDROME
Authors: Araya Patricio 
Published: November 2003

OBJECTIVE  There is no endocrine test which is completely reliable for the confirmation of Cushing's syndrome and in separation of the various aetiologies. We have tested the hypothesis that overnight dexamethasone pre‐treatment should result in a better performance of the lysine‐vasopressin (LVP) test in the diagnosis of Cushing's syndrome. STUDY DESIGN AND PATIENTS  We studied 61 subjects, including 25 pituitary‐dependent and 9 pituitary independent Cushing's (7 adrenal tumours and 2 ectopic ACTH syndromes), 18 euadrenal controls, 4 depressed subjects, and 5 cushingoid patients. The subjects received 1 mg of dexamethasone orally at 2300 h and the following morning they were given 10 IU of lysine‐vasopressin im. MEASUREMENTS  Plasma cortisol (RIA) was measured at times −15, 0, 15, 30, 45, 60, 75, 90 and 120 minutes. RESULTS  The dexamethasone‐modified LVP (Dx/LVP) test resulted in four patterns of cortisol response. The dexa sensitive pattern (positive suppression and negative response to LVP) was found in euadrenal subjects; the dexa insensitive pattern (negative suppression and positive response to LVP) was seen in Cushing’s disease; a non‐responsive pattern (negative suppression and negative response to LVP) was observed only in pituitary independent Cushing's; and an indeterminate pattern (positive suppression and positive response to LVP) was equivocal, being observed in 2 control subjects, 1 patient with Cushing’s disease and 1 depressed patient. In separating control subjects from Cushing's syndromes the Dx/LVP test had 88.9% sensitivity, 100% specificity and 96.2% diagnostic accuracy; when the test was used to segregate Cushing's disease from control subjects we found 96.0% sensitivity, 100% specificity and 97.7% diagnostic accuracy. The performance variables for the Dx/LVP test in separating pituitary dependent from pituitary independent Cushing's were uniformly 100%. Depressed and cushingoid subjects did not differ from control subjects in their cortisol patterns during the test. Successful removal of the pituitary microadenoma in Cushing's disease was invariably followed by a reversal of the abnormal cortisol pattern (dexa insensitive) during the test to a dexa sensitive pattern indistinguishable from that of control subjects. CONCLUSION These results confirm our hypothesis and suggest that an improved performance of any corticotroph stimulus (oCRH, LVP, AVP or desmopressin) in the diagnosis of Cushing's syndrome should result from pre‐treatment with dexamethasone.
Acute biochemical effects of growth hormone treatment compared with conventional treatment in familial hypophosphataemic rickets
Authors: Patel L. , Clayton P. E. , Brain C. , Pelekouda E. , Addison G. M. , Price D. A. , Mughal M. Z. 
Published: November 2003

OBJECTIVE Conventional treatment of familial hypophosphataemic rickets with oral phosphate and 1α‐hydroxycholecalciferol (1αHCC) does not satisfactorily correct the metabolic or physical defects of the disease and can have adverse effects, such as nephrocalcinosis. Hyperoxaluria from increased oral phosphate intake may contribute to nephrocalcinosis. Growth hormone enhances renal tubular phosphate reabsorption and 1,25‐dihydroxycholecalciferol production in normal and in GH deficient individuals, and may thus be of benefit to patients with familial hypophosphataemic rickets. PATIENTS We have assessed the acute effects of GH on phosphate and calcium metabolism in 6 children (age 4–14 years) with familial hypophosphataemic rickets. DESIGN Each patient served as his/her own control and received the following in a sequential non‐randomized design: conventional treatment with oral phosphate 1.0–3.4 mmol/kg/day in 3–6 divided doses and 1αHCC 18–31 ng/kg/day—no treatment—GH 0.05 mg/kg daily—GH and 1αHCC—and GH with phosphate and 1αHCC. Each treatment was given for 7 days with 7 day periods of no treatment in between. MEASUREMENTS AND RESULTS Glomerular filtration rate, tubular maximum rate of phosphate reabsorption per litre of glomerular filtrate (TmP/GFR) and serum 1,25‐dihydroxycholecalciferol increased with GH. Mean 24‐hour plasma phosphate concentrations did not increase with GH but were higher in the treatment phases which included phosphate and 1αHCC (P = 0.002). Serum PTH was higher when GH was given in combination with phosphate and 1αHCC compared to other phases. Urine oxalate excretion did not differ between the treatment phases. CONCLUSIONS GH seemed to partially correct the defects in renal tubular phosphate transport and 1α‐hydroxylation of 25‐hydroxycholecalciferol. We speculate that the net effect of GH treatment was an increase in body phosphate, although this was not reflected in a change in plasma phosphate. Therefore, GH in combination with 1αHCC may act as a phosphate sparing agent, permitting treatment with lower and less frequent doses of oral phosphate and reducing adverse effects such as nephrocalcinosis.
Association of HTLV‐I with autoimmune thyroiditis in patients with adult T‐cell leukaemia (ATL) and in HTLV‐I carriers
Authors: Akamine Hiromitsu , Takasu Nobuyuki , Komiya Ichiro , Ishikawa Kazuo , Shinjyo Tetsuharu , Nakachi Ken , Masuda Masato 
Published: November 2003

OBJECTIVE Human T‐lymphotrophic virus type I (HTLV‐I) is a retrovirus that causes adult T‐cell leukaemia (ATL). This virus is associated with a variety of autoimmune disorders. The possible association between HTLV‐I infection and autoimmune thyroiditis has not been fully studied. We therefore evaluated anti‐thyroid peroxidase antibodies (TPOAb) and anti‐thyroglobulin antibodies (TGAb) in the sera of patients with ATL, carriers of HTLV‐I, and in healthy control subjects to investigate the possible association between such infection and autoimmune thyroiditis. PATIENTS AND METHODS Fifty‐two ATL patients (21 males, 31 females; mean age 56.4 years) and 50 HTLV‐I carriers (18 males, 32 females; mean age 56.7 years) were studied. The control subjects were 877 healthy adults (271 males, 606 females; mean age 54.8 years) who were negative for HTLV‐I antibody. TPOAb, TGAb, thyroxine (T4) and thyrotrophin (TSH) were measured in serum using a radioimmunoassay kit. RESULTS Positivity for thyroid autoantibodies (TPOAb and/or TGAb) was found in 21 of 52 ATL patients (40.4%), 15 of 50 HTLV‐I carriers (30.0%), and 120 of 877 control subjects (13.7%). The difference between the HTLV‐I‐infected and the control subjects was statistically significant (P < 0.005). Female control subjects had a significantly higher prevalence of positivity for thyroid autoantibodies than the males (17.3 vs 5.5%, P < 0.001). Carriers of HTLV‐I and patients with ATL of each sex showed an equally high prevalence of positivity for thyroid autoantibodies. Of the subjects who were positive for thyroid autoantibody, 7.5% of control subjects, 19.0% of ATL patients, and 40.0% of HTLV‐I carriers had hypothyroidism. A significant difference in this respect was noted between the HTLV‐I infected subjects and the control subjects (P < 0.005). CONCLUSIONS This study demonstrates a high prevalence of positivity for thyroid autoantibodies (TPOAb and/or TGAb) in the adult T‐cell leukaemia patients and the HTLV‐I carriers. The adult T‐cell leukaemia patients and the HTLV‐I carriers each had a high prevalence of hypothyroidism. There was an association between HTLV‐I infection and autoimmune thyroiditis.
Megaoesophagus in a patient with autoimmune polyglandular syndrome Type II
Authors: Fritzen R. , Bornstein S. R. , Scherbaum W. A. 
Published: November 2003

Dysphagia and vomiting are frequently present in untreated Addison's disease. These non‐specific symptoms may be due either to the metabolic disorder and myopathy or to disorders associated with Addison's disease. We describe a patient with autoimmune adrenal failure as a feature of autoimmune polyglandular syndrome (APS) type II. This patient was referred initially because of megaoesophagus. The association of megaoesophagus with Addison's disease or any of the three types of APS has not previously been described in humans. The association of megaoesophagus and adrenal failure, however, is known to occur in Allgrove's syndrome, a disease with primary manifestation in childhood characterized by adrenal failure, achalasia and alacrimia. Moreover, there are several reports on the association of megaoesophagus with adrenocortical insufficiency and other autoimmune endocrine diseases in dogs. Vomiting and dysphagia usually resolve with hormone substitution in patients with isolated Addison's disease. In our patient these symptoms disappeared in spite of the radiological persistence of megaoesophagus, which might have been overlooked if the diagnosis of Addison's disease had been made earlier. The occurrence of megaoesophagus might be more common than previously suspected and we suggest a systematic search for similar findings in other patients with autoimmune Addison's disease, even when minor dysphagia is present.
The prevalence of non‐classic adrenal hyperplasia due to 11β‐hydroxylase deficiency among hirsute women in a Turkish population
Authors: Keleştimur Fahrettin , Şahin Yılmaz , Ayata Demet , Tutuş Ahmet 
Published: November 2003

OBJECTIVE The present study was designed to determine the prevalence of 11β‐hydroxylase deficiency in adult women with hirsutism in a Turkish population. DESIGN AND PATIENTS One hundred and twenty‐four consecutive unselected hirsute patients were studied. An ACTH stimulation test was performed in the midfollicular phase of the cycle on the patients and 20 age‐matched controls by administration of a single bolus of 0.25 mg ACTH (1–24) at 0900 h. MEASUREMENTS Serum 11‐deoxycortisol levels were measured before, 30 and 60 minutes after ACTH injection. Basal free testosterone (fT), SHBG, cortisol and androstenedione (A) were also measured. The diagnosis of 11β‐hydroxylase deficiency has been presumed when the serum 11‐deoxycortisol response to ACTH stimulation exceeded three times the 95th percentile of controls. RESULTS Basal hormone levels including fT and A were significantly higher in the hirsute women than in the healthy women. SHBG was significantly lower in the hirsute patients. Basal and ACTH stimulated 11‐deoxycortisol levels were found to be significantly increased in the patients compared with the controls. Eight patients (6.5%) had an 11‐deoxycortisol response higher than three times the upper normal limit. CONCLUSIONS Using stringent diagnostic criteria, we have found that 6.5% of the hirsute women in a Turkish population could be presumed to have 11β‐hydroxylase deficiency.
The effect of a new slow‐release, long‐acting somatostatin analogue, lanreotide, in acromegaly
Authors: Al‐Maskari M. , Gebbie Jan , Kendall‐Taylor P. 
Published: November 2003

OBJECTIVE Previous studies have shown that somatostatin analogues such as octreotide and lanreotide are effective in suppressing GH and IGF‐I levels in acromegaly, but the mode of administration and the frequency of injections were inconvenient for the patients. We have evaluated the effects of a new slow‐release (SR), long‐acting formulation of lanreotide, a somatostatin analogue, on clinical, biochemical and safety responses in acromegaly. DESIGN We studied the effects of SR‐lanreotide 30mg administered intramuscularly twice or three times monthly for 6 months. Ten patients were studied, in whom acromegaly was confirmed by clinical features,mean GH>5 mU/l, and failure to suppress GH to <2 mU/l after a 75‐g oral glucose load. MEASUREMENTS Subjective improvement in clinical symptoms of acromegaly was graded and recorded. Any adverse reactions were noted. Plasma GH levels were measured every 10min for one hour from 0830–0930h; fasting IGF‐I levels were determined at 0830h; GH, glucose and insulin responses to oral glucose loading were measured at 0,30,60,90 and 120minutes from 0930 to 1130h. Baseline measurements were carried out and repeated at 3 and 6 months. Biliary ultrasonography was performed at baseline and 6 months. RESULTS GH levels in the10 patients decreased from 26.8±12.0 (mean SEM) to 12.7±7.0 mU/l at 3 months (P=0.04) and 9.8±5.0 mU/l at 6 months (P=0.06). Fasting IGF‐I levels decreased from 123.2±27.0 to 73.5±13.0nmol/l at 3 months (P=0.01), and increased slightly to 97.4±31.0nmol/l (P=0.05) but remained below baseline levels at 6 months. Five patients achieved good control (GH<5 mU/l) at 3months. In the remaining 5 patients the dose frequency was increased to every 10 days and one achieved good control. IGF‐I levels normalized in 3 and 5 patients at 3 and 6 months, respectively. Fasting insulin levels and peak insulin after an oral glucose load did not change significantly at 3 months but decreased from 11.7±2.0 to 7.8±3.3 mU/l (P=0.05) and 106.2±24.6 to 53.3±14.3 mU/l (P=0.04) at 6 months, respectively. There was no significant change in fasting glucose at 3 or 6 months. Most patients reported clinical improvement in acromegalic symptoms. No major adverse events were reported, but mild to moderate gastrointestinal symptoms were recorded after the initial injections. One patient developed asymptomatic gallstones at 6 months. CONCLUSIONS This slow‐release formulation of lanreotide given either twice or thrice monthly was well tolerated, more convenient for patients, effective in controlling and alleviating the symptoms of acromegaly, as well as suppressing GH and IGF‐I levels, and had no detrimental effects on carbohydrate tolerance in acromegaly.
The 24‐hour rhythm of oxytocin and Β‐endorphin secretion in human pregnancy
Authors: Lindow S. W. , Newham A. , Hendricks M. S. , Thompson J. W. , Van Der Spuy Z. M. 
Published: November 2003

OBJECTIVE Animal work suggests that maternal oxytocin secretion is influenced by the secretion of endogenous opioids in pregnancy. Spontaneous labour and pre‐labour uterine activity follow a 24‐hour rhythm the origin of which has not been explained but may be related to diurnal changes in oxytocin secretion. This study was performed to document the changes over a 24‐hour period in maternal oxytocin and Β‐endorphin secretion. DESIGN A 4‐hourly blood profile was undertaken for a 24‐hour period. PATIENTS Sixteen women with singleton pregnancies of more than 36 weeks gestation and 10 women with pregnancies in the mid trimester were studied. MEASUREMENTS Blood was sampled 4‐hourly for 24 hours beginning at 1200 h. Oxytocin was measured in all patients and Β‐endorphin‐like immunoreactivity was measured in 15 patients. RESULTS A simple index was defined for comparing night‐time levels to daytime levels for both oxytocin and Β‐endorphin. In all cases more than 36 weeks gestation the index was positive for oxytocin (night‐time levels were higher) and in all cases the index was negative for Β‐endorphin (night‐time levels were lower). In the mid trimester women all values of the index for oxytocin were positive but in the Β‐endorphin group equal numbers demonstrated a positive or a negative index. CONCLUSIONS Reciprocal 24‐hour rhythms were demonstrated between oxytocin and Β‐endorphin; however, it is not clear whether this relationship is causal.