Abstract:
INTRODUCTION:Prader-Willi syndrome (PWS) is a genetic disorder caused by the loss of expression of paternally transcribed genes in a highly imprinted region of chromosome 15q11-13. The clinical phenotype has been well characterized, mostly related to hypothalamic dysfunction. Even though central hypothyroidism has been documented in 20-30% of patients with PWS, thyroid function during the first 2 years of life has not been clearly defined. OBJECTIVE:To evaluate hypothalamic-pituitary-thyroid function in infant PWS patients. STUDY DESIGN:Eighteen patients with PWS, aged 0.16-2 years, were included in a prospective study. PWS diagnosis was based on clinical features and molecular analysis. Serum total (T) T4, free (F) T4, T3 and thyroid-stimulating hormone (TSH) were evaluated in the patients with PWS included in the study. Serum hormone values were compared to those of a large reference population of the same age. RESULTS:In 13 of 18 patients with PWS (72.2%), serum TT4 and/or FT4 levels were below the 2.5th percentile of the reference population, while in only one PWS patient serum T3 was below this cut-off. CONCLUSION:The results of this study suggest that transient or definitive thyrotropin-releasing hormone (TRH)-TSH thyroid axis dysfunction may frequently be present in infant PWS patients. Paediatricians should be aware of this dysfunction in this critical period of thyroid hormone action on neurological development.
journal_name
Clin Endocrinol (Oxf)journal_title
Clinical endocrinologyauthors
Vaiani E,Herzovich V,Chaler E,Chertkoff L,Rivarola MA,Torrado M,Belgorosky Adoi
10.1111/j.1365-2265.2010.03840.xsubject
Has Abstractpub_date
2010-10-01 00:00:00pages
546-50issue
4eissn
0300-0664issn
1365-2265pii
CEN3840journal_volume
73pub_type
杂志文章abstract::The measurement of serum intact parathyroid hormone (PTH) (1-84) over a 24-h period has shown the existence of a circadian rhythm in normal males which is absent in patients with primary hyperparathyroidism. The physiological significance of this observation is reflected in the presence of parallel changes in nephroge...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1990.tb00888.x
更新日期:1990-04-01 00:00:00
abstract:OBJECTIVES:A population-based study was undertaken to determine the mortality and morbidity for people with hypoparathyroidism compared to the general population. METHODS:In this study, patients identified with chronic hypoparathyroidism using data linkage from regional datasets were compared with five age- and gender...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13895
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:To define the symptoms, signs and treatment outcomes in a population of older patients with Cushing's disease (CD). METHODS:We analysed the clinical presentation and treatment outcomes in 45 CD patients older than 60 years, in comparison with 90 CD patients younger than 60, and a control group of 45 older p...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13799
更新日期:2018-10-01 00:00:00
abstract::Twelve female patients (mean age 60 years, range 41--74) with euthyroid multinodular goitre and a flat TRH test (median delta TSH 0.9 muIU/ml, range 0--3.5) had normal prolactin responses to TRH (median data prolactin 48 ng/ml, range 6--115). The results are comparable with those in euthyroid patients receiving full s...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1978.tb03581.x
更新日期:1978-11-01 00:00:00
abstract:OBJECTIVE:There is increasing evidence that antenatal stress has long-lasting effects on child development, but there is less accord on the mechanisms and the gestational window of susceptibility. One possible mechanism is by foetal exposure to maternal cortisol. To explore this, we investigated the relationship betwee...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.02785.x
更新日期:2007-05-01 00:00:00
abstract:OBJECTIVE:High overnight plasma growth hormone (GH) levels in insulin-dependent diabetes mellitus (IDDM) are reflected in both an increase in the GH pulse amplitude and elevated baseline GH concentrations. To determine whether these are a result of an increase in GH secretory episodes, we undertook deconvolution analys...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1993.tb00978.x
更新日期:1993-01-01 00:00:00
abstract::Twenty women with secondary hypogonadism and four normal women in the early follicular phase of the cycle were treated for 7 days with 10, 50 or 100 micrograms synthetic LHRH administered intramuscularly at 4 h intervals. Concentrations of pituitary and ovarian hormones in plasma were measured at intervals during the ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1983.tb00737.x
更新日期:1983-07-01 00:00:00
abstract::Using a sensitive enzyme amplified immunoassay for TSH, the evening rise and pulsatile release of TSH were studied in 10 men with chronic renal failure treated by haemodialysis. Compared to euthyroid male controls the evening rise of TSH was attenuated (median 0.066 vs 0.195 mU/l/h, P less than 0.01) and the rate of r...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1989.tb00452.x
更新日期:1989-07-01 00:00:00
abstract::Six healthy subjects were infused with angiotensin II and plasma concentrations of angiotensin, ACTH and cortisol were measured before, during and after the infusion. In all cases the plasma ACTH concentration fell as plasma angiotensin increased and rose again, sometimes to higher than basal levels, when the angioten...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1979.tb01359.x
更新日期:1979-02-01 00:00:00
abstract:OBJECTIVE:The insulin tolerance test (ITT) has been suggested as the gold standard for diagnosing GH deficiency (GHD). The ITT is, however, potentially hazardous. Glucose monitoring during the ITT varies between centres and there is surprisingly little information on the actual level of blood glucose nadir and the dura...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2006.02436.x
更新日期:2006-02-01 00:00:00
abstract:OBJECTIVE:The objective of this study was to evaluate symptoms of thyrotoxicosis, bone turnover, bone mineral density (BMD) and occult atrial fibrillation (AF) in women ≥65 years with mild endogenous subclinical hyperthyroidism (SCH). DESIGN:Cross-sectional and case-control study. PATIENTS:Signs and symptoms of thyro...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12979
更新日期:2016-07-01 00:00:00
abstract:CONTEXT:Germline mutations in four genes (RET, VHL, SDHB and SDHD) are detected in about 17% of patients with apparently sporadic pheochromocytoma. Thus, genetic screening of all patients with this disease is suggested for a rational diagnostic approach and management. OBJECTIVE:To report the clinical, biochemical and...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12032
更新日期:2013-03-01 00:00:00
abstract::In a prospective study of the incidence of thyrotoxicosis sera from 216 thyrotoxic patients in seven English towns were assayed for TSH-receptor antibodies. The incidence of antibody negative thyrotoxicosis correlated closely with the previous prevalence of endemic goitre in the towns (r = 0.9) indicating a high curre...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1985.tb00225.x
更新日期:1985-09-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:There are currently no sensitive and specific assays for activin B that could be utilized to study human biological fluids. The aim of this project was to develop and validate a 'total' activin B ELISA for use with human biological fluids and establish concentrations of activin B in the circula...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2009.03567.x
更新日期:2009-12-01 00:00:00
abstract:OBJECTIVES:Widespread amyloid deposition in the thyroid gland causes diffuse, clinically apparent enlargement of the thyroid (amyloid goitre: AG). The aim of this study was to clarify the abnormalities of thyroid function in patients with AG. DESIGN:Thirty patients with secondary amyloidosis were retrospectively analy...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1997.1841000.x
更新日期:1997-06-01 00:00:00
abstract:BACKGROUND:It has previously been shown that higher serum TSH is associated with increased thyroid cancer incidence and advanced-stage disease. In the healthy adult population, mean TSH increases with age. As age over 45 years is a known prognostic indicator for thyroid cancer, it is important to know whether higher TS...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2008.03489.x
更新日期:2009-09-01 00:00:00
abstract:OBJECTIVE:With increasing use of computed tomography and magnetic resonance imaging, pituitary adenomas are being discovered incidentally with increasing frequency. However, limited data are available concerning the clinical importance and natural history of such 'incicentalomas'. We have undertaken a prospective study...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1999.00748.x
更新日期:1999-07-01 00:00:00
abstract:OBJECTIVE:Traumatic brain injury (TBI) has been associated with hypopituitarism and GH deficiency. However, TBI-mediated hypopituitarism may be more frequent than previously thought. The present work, performed in patients with severe TBI at least 1 year before, had three aims: (i) to evaluate the prevalence of hypopit...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2005.02250.x
更新日期:2005-05-01 00:00:00
abstract:INTRODUCTION:The rates of sensitivity and specificity of fine needle aspiration biopsy (FNAB) for the diagnosis of thyroid malignancy differ considerably among various reported series. These values are influenced by three factors: (a) whether only clearly positive and negative results are considered, or whether the com...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1999.00847.x
更新日期:1999-10-01 00:00:00
abstract:OBJECTIVE:The development and progression of sporadic adrenocortical tumours are poorly understood. In autopsy studies adrenocortical tumours are found in between 2 and 9% of the general population. In congenital adrenal hyperplasia (CAH), decreased production of cortisol leads to increased secretion of ACTH from the p...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1999.00655.x
更新日期:1999-03-01 00:00:00
abstract::We have studied three patients (1M, 2F), age range 10.9 to 15.5 years, who had abnormal sexual maturation secondary to primary hypothyroidism. The boy had inappropriately large testes for his stage of puberty, the girls had isolated breast development and there was absence of pubertal growth acceleration. FSH, LH, TSH...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1988.tb03682.x
更新日期:1988-05-01 00:00:00
abstract::A 16-year-old female patient with an adrenal tumour was studied. Clinically she had progressive hirsutism, showed high urinary 17-oxosteroid excretion with normal plasma cortisol. Plasma C19-steroids, both unconjugated (including testosterone) and sulphate-conjugated, were greatly elevated. On surgical exploration an ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1978.tb02773.x
更新日期:1978-04-01 00:00:00
abstract:OBJECTIVE:Oestradiol sensitizes the pituitary to GnRH, while gonadotrophin surge attenuating factor (GnSAF) may oppose this action. Using the LH response to GnRH during treatment with FSH as an in vivo bioassay for GnSAF, we tested the hypothesis that the augmented LH response to GnRH in the late follicular phase is re...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12306
更新日期:2014-03-01 00:00:00
abstract::The ratios of reverse triiodothyronine (rT3) to triiodothyronine (T3) concentrations were similar (0.89 +/- 0.09) in twenty-three autonomous thyroid nodules (fourteen decompensated, nine compensated) and paranodular tissues (0.82 +/- 0.08), whereas the serum ratio was significantly lower (0.098 +/- 0.014). This is com...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1977.tb01310.x
更新日期:1977-08-01 00:00:00
abstract:BACKGROUND:A number of surgical series have been reported on the treatment of acromegaly and their results vary widely. The acceptable definition of remission has changed in recent years and it is known, though in a small series, that growth hormone levels of > 5 mU/l are still associated with an increased mortality fr...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1999.00760.x
更新日期:1999-05-01 00:00:00
abstract:OBJECTIVE:Postablation whole-body scintigraphy, which is performed 5-7 days after administration of ablation activity of radioactive iodine-131 (131 I) in patients with thyroid cancer, is considered a routine procedure for remnant ablation and a useful tool for disease staging. However, the relationship of pre-ablation...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13158
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:Obestatin is a novel hormone that is encoded by the Ghrelin gene and produced in the gut. Ghrelin is profoundly orexogenic and adipogenic, increasing food intake and body weight. This new ghrelin-associated peptide behaves as a physiological opponent of ghrelin in rodent animals, but its pathophysiological r...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.02776.x
更新日期:2007-04-01 00:00:00
abstract:BACKGROUND:Urine calcium after an overnight fast is higher in osteoporotic than in normal post-menopausal women. The question is whether this is the cause or effect of the bone-losing state. OBJECTIVE:To establish whether the elevated obligatory calcium loss in osteoporotic women is due to a raised filtered load of ca...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1994.tb03782.x
更新日期:1994-07-01 00:00:00
abstract:OBJECTIVES:Localization of ectopic ACTH-secreting tumours causing Cushing syndrome (ECS) is essential for clinical management, yet often difficult. [68 Ga]-DOTATATE PET/CT ([68 Ga]-DOTA-(Tyr3 )-octreotate)] is an FDA-approved high-resolution diagnostic tool for imaging neuroendocrine tumours. Data on the clinical utili...
journal_title:Clinical endocrinology
pub_type: 杂志文章,多中心研究
doi:10.1111/cen.14008
更新日期:2019-08-01 00:00:00
abstract:INTRODUCTION:Iodine deficiency in pregnancy may impair foetal neurological development. The UK population is generally thought to be iodine sufficient; however, recent studies have questioned this assumption. Our study aimed to explore the prevalence of iodine deficiency in a cohort of pregnant mothers from South-West ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13268
更新日期:2017-03-01 00:00:00