Abstract:
OBJECTIVE:Genetic diagnosis available since 1993 established germinal mutations of the RET proto-oncogene at codon 634 as the main cause of inherited medullary thyroid carcinoma (MTC). International guidelines established in 1999 recommend that children with such mutations undergo a total thyroidectomy before age 5, with unspecified cervical neck dissection. Since 1993, only 41 of 275 thyroidectomies reported in RET 634 children were performed before age 5 (15%). The aim of this study was to evaluate the implementation of these guidelines in a single centre. DESIGN AND PATIENTS:Genetic diagnosis was proposed to the parents of all eight children born after 1992 from two RET 634 families. Total thyroidectomy was proposed before age 5 if the child carried a mutation. RESULTS:Genetic diagnosis was performed in all the children (aged 1-3) and thyroidectomy in the five who carried a mutation (aged 2-5). Cervical lymph node dissection varied from lymphadenectomy of central and lateral compartments in the eldest child to pickings in the youngest. There was no permanent hypoparathyroidism or recurrent nerve paralysis. C-cell hyperplasia, medullary thyroid carcinoma and lymph node metastasis were present in 5/5, 3/5 and 0/5, respectively. Undetectable pentagastrin-stimulated CT levels were achieved and maintained postoperatively in all five children (average follow-up 3.6 years). CONCLUSIONS:MEN 2 guidelines on thyroidectomy can be efficiently and safely implemented by a multidisciplinary team operating in a single centre. The lack of guidelines on cervical neck dissection remains a problem; this could be solved by determining an age under which this procedure would be deemed unnecessary.
journal_name
Clin Endocrinol (Oxf)journal_title
Clinical endocrinologyauthors
Piolat C,Dyon JF,Sturm N,Pinson S,Bost M,Jouk PS,Plantaz D,Chabre Odoi
10.1111/j.1365-2265.2006.02559.xsubject
Has Abstractpub_date
2006-07-01 00:00:00pages
118-24issue
1eissn
0300-0664issn
1365-2265pii
CEN2559journal_volume
65pub_type
杂志文章abstract:OBJECTIVES:Septic patients always develop muscle wasting, which delays the rehabilitation and contributes to the increased complications and mortality. Previous studies have implied the crucial role of central inflammation and neuropeptides in the energy balance and muscle metabolism. Insulin has been confirmed to atte...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12610
更新日期:2015-05-01 00:00:00
abstract::We describe a patient with multiple endocrine adenomatosis Type I, characterized by pituitary-dependent Cushing's Syndrome, marked hyperprolactinaemia, primary hyperparathyroidism and hyperinsulinism leading to hypoglycaemia. The patient subsequently developed an exocrine pancreatic carcinoma at the age of 32 years fr...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1983.tb02879.x
更新日期:1983-05-01 00:00:00
abstract:OBJECTIVE:We examined the obesity phenotype most strongly associated with increased plasma concentrations of sTNFR2, and compared which of the two markers, TNF-alpha or sTNFR2, better predicts indices of plasma glucose-insulin homeostasis. DESIGN, PATIENTS AND MEASUREMENTS:Plasma sTNFR2 levels were measured in a sampl...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2009.03671.x
更新日期:2010-03-01 00:00:00
abstract:OBJECTIVE:Sex steroid hormones influence bone mineral density (BMD) in women, but are less well-studied in men. We evaluated the association of serum total and free sex steroid hormones and SHBG with osteopaenia in a nationally representative sample of men aged 20-90 years. DESIGN:BMD and sex steroid hormones were mea...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2008.03300.x
更新日期:2009-01-01 00:00:00
abstract:OBJECTIVE:To evaluate the effect of hyperprolactinaemia and its treatment with dopamine-agonists on bone mass and turnover in adolescent patients compared to adults. PATIENTS:Forty patients with hyperprolactinaemia (20 with disease onset during adolescence and 20 during adulthood) and 40 healthy control subjects. DES...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2000.00902.x
更新日期:2000-03-01 00:00:00
abstract:OBJECTIVE:Serum free cortisol, rather than serum total cortisol (TC), determines glucocorticoid activity in vivo, but how the considerable inter-subject variation in ambient serum free cortisol affects the outcome of dynamic hypothalamic-pituitary-adrenal (HPA) assessment in noncritically ill subjects is unknown. DESI...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2011.04080.x
更新日期:2011-09-01 00:00:00
abstract:OBJECTIVE:To assess the efficacy and safety of prenatal dexamethasone treatment in offspring at risk for congenital adrenal hyperplasia. METHODS:MEDLINE, EMBASE, the Cochrane Library, the clinicaltrials.gov website databases were systematically searched from inception through March 2019. WMD and SMD with 95%CIs were c...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.14126
更新日期:2020-02-01 00:00:00
abstract::The excretion of FSH, LH, oestrogens and pregnanediol was monitored once weekly in urine samples collected from eight peri-menopausal women aged 44-55 years (median, 52 years). Observations were commenced between 5 and 15 weeks before the menopause and were continued for 22-30 weeks after final menstruation. Amenorrho...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1982.tb01616.x
更新日期:1982-11-01 00:00:00
abstract:CONTEXT AND OBJECTIVE:Nonfunctioning pituitary adenomas (NFPAs) are the most common subtype of pituitary tumour. Hypopituitarism is observed in NFPAs due to tumour- or treatment-related factors and may increase mortality risk. Here, we analysed the associations of hypopituitarism, hormone replacement and mortality in a...
journal_title:Clinical endocrinology
pub_type: 杂志文章,多中心研究
doi:10.1111/cen.13141
更新日期:2016-11-01 00:00:00
abstract:OBJECTIVE:Ghrelin has been implicated in energy homeostasis, body weight regulation and glucose metabolism. Level of unacylated ghrelin (UAG), but not acylated ghrelin (AG), has been suggested to increase during long-term exercise. However, the association of the level of UAG with exercise-induced changes of insulin se...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2011.04135.x
更新日期:2012-01-01 00:00:00
abstract:BACKGROUND:Nonclassical congenital adrenal hyperplasia (NC-CAH) is caused by mutations of the CYP21A2 gene. The clinical manifestations and hormonal derangements of NC-CAH are quite variable. OBJECTIVES:(i) To define the phenotype and its relation to genotype according to gender and age and (ii) to evaluate the validi...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12543
更新日期:2015-04-01 00:00:00
abstract::Alopecia is a frequent feature in hereditary resistance to (1,25(OH)2D). We sought insight into this feature by analysing data from affected members of 30 kindreds. We assessed indices of mineral metabolism in one group with normal hair compared with a group with alopecia. Hereditary resistance to 1,25(OH)2D was diagn...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1986.tb01703.x
更新日期:1986-10-01 00:00:00
abstract::The pathogenetic mechanisms underlying pituitary tumorigenesis are largely unknown. Previous reports have suggested that aggressive pituitary adenomas and/or carcinomas may be associated with genetic alterations that are distinct from those responsible for the more common and less aggressive pituitary adenomas. Here, ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2001.01322.x
更新日期:2001-10-01 00:00:00
abstract:OBJECTIVE:The purpose was to investigate whether the function of the pinealocytes is changed in patients with disturbed metabolic rate or in subjects with marked obesity. DESIGN:Venous blood was sampled every second hour between 1800 and 0800 h, and urine collected between 2200 and 0700 h. PATIENTS:Eight patients wit...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1991.tb03497.x
更新日期:1991-07-01 00:00:00
abstract::The inactivation of synthetic [3H]thyrotrophin-releasing hormone (TRH) by plasma was studied in rats treated with propylthiouracil (PTU) alone or with PTU and thyroxine. 48 h after the onset of treatment with thyroxine, the capacity of rat plasma to inactivate [3H]TRH was significantly increased. The percentage of dea...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1976.tb01961.x
更新日期:1976-07-01 00:00:00
abstract:OBJECTIVE:Three independent genome-wide association studies in white populations have reported that single nucleotide polymorphisms (SNPs) in the KIAA0350 gene are associated with susceptibility to type 1 diabetes (T1D). The gene product of KIAA0350 is predicted to be a sugar binding C-type lectin. In the present study...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2008.03437.x
更新日期:2009-07-01 00:00:00
abstract::In order to evaluate the possible influence of GABAergic neurotransmission on the arginine vasopressin (AVP) response to osmotic and pressure volumetric stimuli, the GABAergic drug sodium valproate was administered by mouth (200 or 400 mg 16 h, 8 h and just before tests) to eight normal men before osmotic (i.v. infusi...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1989.tb00437.x
更新日期:1989-04-01 00:00:00
abstract::The role of endogenous opiate peptides in puerperal hyperprolactinaemia, and in the control of TSH in hypothyroidism, has been investigated. Although exogenous opioids raise prolactin and TSH levels, 16 mg naloxone administered to women on days 2-4 of the puerperium produced no significant change in serum prolactin an...
journal_title:Clinical endocrinology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1111/j.1365-2265.1982.tb00722.x
更新日期:1982-03-01 00:00:00
abstract::A gonadotrophin response testosterone producing adrenal cortical adenoma in a 61-year-old woman is described. Oophorectomy was performed before adrenalectomy as at first an ovarian origin of excess and androgen production was suspected. The hypophyseal gonadal feedback system was studied after oophorectomy using LHRH ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1980.tb03128.x
更新日期:1980-01-01 00:00:00
abstract:OBJECTIVE:Poorly differentiated thyroid cancer (PDTC) is a rare, but aggressive thyroid cancer (TC) and a main cause of death from non-anaplastic follicular cell-derived TC. Assessing the risk of PDTC-related death and the risk of recurrence is important for clinicians. The recent American Thyroid Association (ATA) 201...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13910
更新日期:2019-08-01 00:00:00
abstract::Twenty-seven patients starting regular haemodialysis were treated with a 1 microgram daily dose of 1alpha-hydroxyvitamin D3 and concurrent aluminium hydroxide therapy to prevent hyperphosphataemia. There was an increase in plasma calcium, but no significant improvement in plasma alkaline phosphatase activity or parath...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1977.tb03372.x
更新日期:1977-12-01 00:00:00
abstract:OBJECTIVE:Scalp hair loss is often encountered in clinical practice in Japan after successful surgery for acromegaly. However, this intriguing issue has not been addressed in the literature. The aim of this study was to examine scalp hair loss after surgery for acromegaly. METHODS:Postoperative scalp hair loss was sur...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12040
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVE:Our clinical practice has been to offer treatment with cyclical disodium etidronate and high dose calcium supplements (1500-1600 mg/day) to all female patients with osteoporosis who are unable or unwilling to take hormone replacement therapy (HRT), and male osteoporotics. In a retrospective study we compared ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1994.tb02537.x
更新日期:1994-08-01 00:00:00
abstract:OBJECTIVE:Insulin and glucocorticoids are known to increase total leptin levels. However, the effects of insulin and glucocorticoids on the components of the leptin system - free leptin (FL), bound leptin (BL) and soluble leptin receptor (SR) - have not been elucidated. The aim of this study was to determine if there i...
journal_title:Clinical endocrinology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1365-2265.2001.01243.x
更新日期:2001-04-01 00:00:00
abstract:OBJECTIVE:Various studies have demonstrated coexistence of ovarian and adrenal hyperandrogenism in women. This study was designed to determine whether testosterone can increase the response of the adrenal gland to stimulation by adrenocorticotrophin (ACTH). DESIGN:Non-randomized intervention in a university teaching h...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1994.tb03010.x
更新日期:1994-05-01 00:00:00
abstract:BACKGROUND:Postsurgical regrowth or recurrence of nonfunctioning pituitary adenomas (NFAs) is not uncommon and often requires further surgery or radiotherapy (DXT). Routine postoperative DXT increases the incidence of hypopituitarism, which is associated with increased morbidity and mortality. Identification of genetic...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2004.02046.x
更新日期:2004-07-01 00:00:00
abstract::Objective African-Americans have been shown to have low prevalence of hypothyroidism. Brazil has a high ethnic admixture allowing further exploration into whether environmental factors can explain the ethnic differences. Design A survey, representative of the population of Rio de Janeiro, a large metropolitan city in ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.02816.x
更新日期:2007-06-01 00:00:00
abstract:CONTEXT:Besides medullary thyroid carcinoma and other endocrinopathies, people with Multiple Endocrine Neoplasia Type 2 (MEN2) are at risk of gastrointestinal (GI) symptoms. OBJECTIVE:To investigate the impact of GI symptoms on the daily lives of patients with MEN2. DESIGN:An online survey was conducted among patient...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.14366
更新日期:2020-11-10 00:00:00
abstract:OBJECTIVE:GH deficiency, either in children or in adults, is a clinically relevant problem. The diagnosis is based on dynamic tests of GH secretion, which are clear cut on a group basis but highly problematic for individual diagnosis. The controversy surrounding the diagnosis of GH deficiency reflects the absence of a ...
journal_title:Clinical endocrinology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1365-2265.1996.00841.x
更新日期:1996-11-01 00:00:00
abstract:OBJECTIVE:Hypercortisolaemia is associated with an increased risk of cardiovascular disease (CVD), either through a direct action on the myocardium or by increased traditional cardiovascular risk factors. The aim of this study was to investigate whether the alterations in the ECG in Cushing's disease (CD) are predictab...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2011.03975.x
更新日期:2011-05-01 00:00:00