Abstract:
:Puberty is the period during which we attain adult secondary sexual characteristics and reproductive capability. Its onset depends upon reactivation of pulsative GNRH, secretion from its relative quiescence during childhood, on the background of intact potential for pituitary-gonadal function. This review is intended: to highlight those current practices in diagnosis and management that are evidence based and those that are not; to help clinicians deal with areas of uncertainty with reference to physiologic first principles; by sign-posting relevant data arising from other patient groups with shared issues; to illustrate how recent scientific advances are (or should be) altering clinician perceptions of pubertal delay; and finally, to emphasise that the management of men and women presenting in advanced adult life with absent puberty cannot simply be extrapolated from paediatric practice. There is a broad spectrum of pubertal timing that varies among different populations, separated in time and space. Delayed puberty usually represents an extreme of the normal, a developmental pattern referred to as constitutional delay of growth and puberty (CDGP), but organic defects of the hypothalamo-pituitary-gonadal axis predisposing to hypogonadism may not always be initially distinguishable from it. CDGP and organic, or congenital hypogonadotrophic hypogonadism are both significantly more common in boys than girls. Moreover, around 1/3 of adults with organic hypogonadotrophic hypogonadism had evidence of partial puberty at presentation and, confusingly, some 5-10% of these subsequently may exhibit recovery of endogenous gonadotrophin secretion, including men with Kallmann syndrome. However, the distinction is crucial as expectative ('watch-and-wait') management is inappropriate in the context of hypogonadism. The probability of pubertal delay being caused by organic hypogonadism rises exponentially both with increasing age at presentation and the presence of associated 'red flag' clinical features. These 'red flags' comprise findings indicating lack of prior 'mini-puberty' (such as cryptorchidism or micropenis), or the presence of non-reproductive congenital defects known to be associated with specific hypogonadal syndromes, e.g. anosmia, deafness, mirror movements, renal agenesis, dental/digital anomalies, clefting or coloboma would be compatible with Kallmann (or perhaps CHARGE) syndrome. In children, interventions (whether in the form or treatment or simple reassurance) have been historically directed at maximising height potential and minimising psychosocial morbidity, though issues of future fertility and bone density potential are now increasingly 'in the mix'. Apubertal adults almost invariably harbour organic hypogonadism, requiring sensitive acknowledgement of underlying personal issues and the timely introduction of sex hormone replacement therapy at more physiological doses.
journal_name
Eur J Endocrinoljournal_title
European journal of endocrinologyauthors
Dunkel L,Quinton Rdoi
10.1530/EJE-13-0894subject
Has Abstractpub_date
2014-06-01 00:00:00pages
R229-39issue
6eissn
0804-4643issn
1479-683Xpii
EJE-13-0894journal_volume
170pub_type
杂志文章,评审abstract:OBJECTIVE:GH causes insulin resistance, impairs glycemic control and increases the risk of vascular diabetic complications. Sulphonylureas stimulate GH secretion and this study was undertaken to investigate the possible stimulatory effect of repaglinide and nateglinide, two novel oral glucose regulators, on critical st...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1470133
更新日期:2002-07-01 00:00:00
abstract::The expression of the neuropeptide Y (NPY) gene varies considerably in human pheochromocytomas, but the mechanisms for this variation have not been clarified. To investigate the regulation pattern of the NPY gene in human pheochromocytomas, we screened 16 pheochromocytomas and 9 normal adrenal tissues with Northern bl...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1410431
更新日期:1999-10-01 00:00:00
abstract:OBJECTIVE:An increased tendency towards thromboembolic events is observed in patients with Cushing's syndrome. There are much fewer publications available about thromboembolic complications in patients with subclinical Cushing's syndrome (SCS). Therefore, a question arises whether hemostatic disturbances appear in this...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-11-0535
更新日期:2011-12-01 00:00:00
abstract::Ipilimumab (Yervoy; Medarex and Bristol-Myers Squibb) is a human MAB against cytotoxic T-lymphocyte antigen 4, which enhances co-stimulation of cytotoxic T-lymphocytes, resulting in their proliferation and an anti-tumour response. It is licensed for the treatment of unresectable or metastatic malignant melanoma, while...
journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/EJE-12-0167
更新日期:2012-07-01 00:00:00
abstract::The present study focuses on the pathogenesis of increased frequency of large-vessel disease in diabetes. The diabetic arterial wall displays characteristic alterations of the extracellular matrix secreted by arterial smooth-muscle cells. The effects of insulin and growth hormone on the synthesis of sulphate-labelled ...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1340326
更新日期:1996-03-01 00:00:00
abstract::The best test for the assessment of the hypothalamic-pituitary-adrenal (HPA) axis remains a matter of controversy. We compared the performance of the short synacthen test (SST, 250 microg) with the insulin stress test (IST) to assess the reliability of the former as a first line test. Patients with pituitary disease u...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1390580
更新日期:1998-12-01 00:00:00
abstract::Maintenance of fluid homeostasis during periods of heightened physical stress can be best evaluated in humans using exercise as a model. Although it is well established that arginine vasopressin (AVP), aldosterone and atrial natriuretic peptide (ANP) are the principle hormones regulating fluid balance at rest, the pot...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-08-0064
更新日期:2008-12-01 00:00:00
abstract::The human growth hormone variant (GH-V) gene is expressed during pregnancy in the syncytiotrophoblast and, as shown recently, in the normal human testis. In addition to the classical transcript encoding for the 22 K major form, intron D-retaining processed mRNAs (GH-V2) have also been described in both tissues. In the...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1390424
更新日期:1998-10-01 00:00:00
abstract::Guidelines recommend adults with pituitary disease in whom GH therapy is contemplated, to be tested for GH deficiency (AGHD); however, clinical practice is not uniform. AIMS:1) To record current practice of AGHD management throughout Europe and benchmark it against guidelines; 2) To evaluate educational status of hea...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-20-1180
更新日期:2020-12-01 00:00:00
abstract:CONTEXT:There is evidence to suggest that IGF-I plays a role in regulating bone turnover. OBJECTIVE:To evaluate the relationships between serum concentrations of IGF-I and IGF-binding protein-3 (IGFBP-3), and bone metabolism markers in healthy children. DESIGN AND SETTING:Prospective cross-sectional study. SUBJECTS ...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-07-0402
更新日期:2007-11-01 00:00:00
abstract:OBJECTIVE:There are limited data on cardiometabolic risk factors and the prevalence of metabolic syndrome (MetS) across the different PCOS phenotypes in Caucasian population. Lipid accumulation product (LAP) is a clinical surrogate marker that could be used for evaluation of MetS in clinical practice. The aim of the st...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-16-0775
更新日期:2016-12-01 00:00:00
abstract:OBJECTIVE:Subclinical hypothyroidism occurs in a number of children with Down's syndrome (DS). The reason for the mildly elevated plasma thyrotropin (TSH) concentrations is not known. The present study investigated whether decreased TSH bioactivity plays a role in this phenomenon. DESIGN:A retrospective study of plasm...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1440001
更新日期:2001-01-01 00:00:00
abstract::In vitro studies indicate a physiological role for insulin-like growth factor I (IGF-I) in paracrine regulation of testicular function and recent clinical studies suggest a potential role for growth hormone (GH) and/or IGF-I in the treatment of hypogonadotrophic states in males. This study aimed to examine the effects...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1310405
更新日期:1994-10-01 00:00:00
abstract::The study was undertaken to investigate the interaction between the release of endothelin-1 (ET-1) and prolactin (PRL) from human decidualized endometrial cells, and the effects of transforming growth factor (TGF)-beta 1 on the release of ET-1 and PRL from human decidual cells in the early stage of pregnancy. Stromal ...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1370200
更新日期:1997-08-01 00:00:00
abstract:OBJECTIVE:Hyperthyroidism in rats produces organ hypertrophy and increases in circulating IGF-I and IGF-binding protein (IGFBP)-3. Chronic treatment with thyroxine (T(4)) during pregnancy advances parturition, blocks lactation and changes several hormone receptors in mammary gland and liver. Since IGFs are implicated i...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1460729
更新日期:2002-05-01 00:00:00
abstract::In many centres, a test dose (TD) of octreotide is administered before commencing somatostatin analogue therapy (SAT), although the merits of this procedure are uncertain. We have analysed the value of the GH response to a TD in predicting the efficacy of subsequent SAT in 47 patients with acromegaly (25 male, median ...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.1.02073
更新日期:2006-02-01 00:00:00
abstract:OBJECTIVE:To assess the influence of craniopharyngioma or consequent surgery on melatonin secretion, and the association with fatigue, sleepiness, sleep pattern and sleep quality. DESIGN:Cross-sectional study. METHODS:A total of 15 craniopharyngioma patients were individually matched to healthy controls. In this stud...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-13-1025
更新日期:2014-06-01 00:00:00
abstract:OBJECTIVES:The French Acromegaly Registry records data of acromegalic patients' since 1992 in French, Belgian (Liège), and Swiss (Lausanne) centers. We studied the prevalence of diabetes in this population looking for risk factors. Patients from one of the centers (Reims) were then analyzed more thoroughly. METHODS:Th...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-10-1050
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:Autoimmune polyendocrine syndrome type I (APS I) is a monogenic disease affecting endocrine glands and other organs due to mutations of the autoimmune regulator (AIRE) gene. There is a wide variability in clinical phenotypes in patients with APS I, which makes the diagnosis a challenge. OBJECTIVE:To screen ...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-07-0843
更新日期:2008-05-01 00:00:00
abstract:OBJECTIVE:Hormone treatment is an important part of gender reassignment therapy in gender dysphoria. Previous data about efficacy and safety are commonly based on small cohorts or they comprise former cohorts under meanwhile obsolet therapy regimes. Our objective was to investigate these topics in a large cohort of ind...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-19-0463
更新日期:2019-11-01 00:00:00
abstract::The aim of this review was to examine the evidence for age-related changes of the hypothalamic-pituitary-adrenal (HPA) axis in both physiological and pathological aging, on the basis of the many data in the literature, as well as of our personal findings. A statistically significant circadian rhythmicity of serum cort...
journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/eje.0.1440319
更新日期:2001-04-01 00:00:00
abstract:OBJECTIVE:Transient neonatal hyperthyrotropinemia (TNH) is defined as a neonatal abnormality of thyroid function, which reverts to normal at re-examination after 2 weeks of life. The thyroid function of these infants has not been sufficiently studied in terms of the risk of developing persistent hyperthyrotropinemia (P...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-13-0907
更新日期:2015-04-01 00:00:00
abstract::Sex steroids are important for the growth and maintenance of both the female and the male skeleton. However, the relative contribution of androgens versus estrogens in the regulation of the male skeleton is unclear. Experiments using mice with inactivated sex steroid receptors demonstrated that both activation of the ...
journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/EJE-09-0118
更新日期:2009-06-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to determine the frequency and characteristics of severe GH deficiency (sGHD) in patients after treatment of acromegaly by surgery alone. DESIGN AND METHODS:One hundred and eighty-six patients fulfilling the criteria for cure of acromegaly were examined by GH-releasing peptide-2 sti...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-11-0657
更新日期:2011-12-01 00:00:00
abstract::AMP-activated protein kinase (AMPK) is a cellular gauge that is activated under conditions, such as low energy, increasing energy production and reducing energy waste. Centrally, the AMPK pathway is a canonical route regulating energy homeostasis, by integrating peripheral signals, such as hormones and metabolites, wi...
journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
doi:10.1530/EJE-16-0927
更新日期:2017-05-01 00:00:00
abstract:OBJECTIVE:To compare the influence of transdermal and peroral oestrogen treatments in conjunction with cyproterone acetate (CPA) on metabolic and hormonal parameters in women with polycystic ovary syndrome (PCOS). PATIENTS AND METHODS:Twenty-four women with PCOS, aged 25.4+/-4.3 (mean+/-s.d.) years, body mass index 24...
journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1530/eje.0.1500215
更新日期:2004-02-01 00:00:00
abstract:OBJECTIVE:To assess the individuals' thyroid volume changes after the mandatory nationwide iodine fortification (IF) program in two Danish areas with different iodine intake at baseline (Copenhagen, mild iodine deficiency (ID) and Aalborg, moderate ID). DESIGN:A longitudinal population-based study (DanThyr). METHODS:...
journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-13-0918
更新日期:2014-03-08 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/EJE-18-0253
更新日期:2018-09-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.1.02011
更新日期:2005-11-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
doi:10.1530/eje.0.1320047
更新日期:1995-01-01 00:00:00