Abstract:
:Raised activity of the LH axis caused by activating mutations of LH receptor gene presents with precocious puberty in boys, analogous to the presentation of LH secreting pituitary adenomas (Faggiano et al., 1983; Ambrosi et al., 1990). LH "hyperactivity' in females appears to have no effect. Hyperactivity of the FSH axis caused by activating mutations of the FSH receptor gene might parallel the presentation of FSH secreting pituitary adenomas with Sertoli cell hypertrophy in men (Heseltine et al., 1989) or reversible premature ovarian failure in women (Moses et al., 1986; Okuda et al., 1989). Indeed the first such case to be described is a male who maintained testicular volume and fertility in the absence of gonadotrophins (Gromoll et al., 1996). Female precocious puberty may require hyperactivity of both gonadotrophin axes because of the "two-cell' arrangement required for ovarian oestrogen production. Mutations of the Gs alpha-subunit gene can mimic this situation in some women with the McCune-Albright syndrome (Malchoff et al., 1994). Lack of LH activity caused by defects in the LH beta molecule causes infertility in men and that resulting from inactivating mutations of the LH receptor gene causes Leydig cell agenesis in men while ovarian development in females is relatively normal. Lack of FSH activity caused by defects in the FSH beta caused infertility in a female, and that caused by inactivating mutations of the FSH receptor gene causes ovarian dysgenesis in women but only variable depression of spermatogenesis in men. Incidentally, this categorization of reproductive disorders may also be applied to the TSH axis. Pituitary adenomas and activating mutations of the TSH receptor gene (Parma et al., 1993) cause hyperthyroidism and TSH beta gene defects (Hayashizaki et al., 1989) and inactivating mutations of the TSH receptor gene (Sunthornthepvarakul et al., 1995) cause hypothyroidism. To complete the analogy with thyroid disorders, it is curious that despite structural similarities with the TSH receptor, neither LH nor FSH receptor autoantibodies have a prominent role in ovarian pathophysiology (Moncayo et al., 1989; Van Weissenbruch et al., 1991; Simoni et al., 1993). Complete gonadotrophin resistance is likely to be very rare, however, so what are we likely to find in partial gonadotrophin resistance? Might the "resistant ovary syndrome' come right in the end, with corresponding minor FSH receptor mutations? Experience with insulin and androgen resistance syndromes suggests that such a scenario is unlikely. Insulin receptor gene mutations are found in extreme Type A insulin resistance but not in moderate forms of insulin resistance (O'Rahilly et al., 1991). Androgen receptor gene mutations are found in nearly all cases of complete androgen insensitivity but rarely in partial forms (Patterson et al., 1994). Mild resistance to hormone action is rarely detectable in relatives who are heterozygous for receptor mutations which are inherited in a recessive pattern. It seems unlikely therefore, that individuals heterozygous for inactivating receptor mutations will manifest symptoms of reproductive disorders and account for common conditions. Thus, while mutation analysis provides new insights into the gender specific role of the gonadotrophins the cause of early gonadal failure in the majority of individuals remains a mystery.
journal_name
Clin Endocrinol (Oxf)journal_title
Clinical endocrinologyauthors
Conway GSdoi
10.1046/j.1365-2265.1996.8680879.xsubject
Has Abstractpub_date
1996-12-01 00:00:00pages
657-63issue
6eissn
0300-0664issn
1365-2265journal_volume
45pub_type
杂志文章,评审abstract:OBJECTIVE:Treatment by octreotide has been suggested in medullary thyroid carcinoma patients with post-surgery metastases. The purpose of this study was to evaluate if the tumoral regression could be improved by a high dose and by prolonged octreotide treatment. DESIGN:Fourteen thyroidectomized patients were studied. ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1992.tb00955.x
更新日期:1992-02-01 00:00:00
abstract::Plasma concentrations of FSH, LH, testosterone and prolactin have been studied in seventeen men, aged 19 to 38 years, with traumatic paraplegia. Plasma testosterone, FSH and LH values were normal in all patients. The median LH concentration in patients was significantly higher (P less than 0.05) than in controls but t...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1982.tb01594.x
更新日期:1982-09-01 00:00:00
abstract:OBJECTIVE:We examined the contributions of growth hormone (GH) and insulin-like growth factor-I (IGF-I) to insulin sensitivity and beta-cell function in acromegaly. DESIGN:A cross-sectional study was used with continuous infusion of glucose with model assessment to determine insulin sensitivity and beta-cell function....
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1992.tb02902.x
更新日期:1992-01-01 00:00:00
abstract:OBJECTIVE:Thyrotoxic hypokalaemic periodic paralysis (THPP) is an uncommon condition with intermittent episodes of muscle weakness and occasionally severe paralysis. THPP is a common complication of hyperthyroidism in Asian populations, and has also been reported in other ethnic groups including Caucasians. This study ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.03014.x
更新日期:2008-01-01 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:BACKGROUND:Obesity disproportionately affects African Americans (AA) (especially women), and is linked to depressed 25-hydroxyvitamin D (25-OH D) and elevated parathyroid hormone (PTH). The relationship of 25-OH D and PTH with body composition and size in AA is not well known. OBJECTIVE:To determine the relationship o...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2009.03676.x
更新日期:2010-05-01 00:00:00
abstract::In an attempt to obtain further information on the aetiology of premenstrual syndrome (PMS), the endocrine changes following enucleation of the corpus luteum in the mid-luteal phase of the cycle were studied in seven patients with PMS, and the results compared to details of seven control patients undergoing hysterecto...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1985.tb00162.x
更新日期:1985-06-01 00:00:00
abstract::The uterine cross-sectional area (UXA) of women with polycystic (PCO) or multifollicular ovaries (MFO) is significantly larger and smaller, respectively, than those of normal women during the early-mid-follicular phase of the menstrual cycle. In the present study the distribution of oestradiol in plasma from normal wo...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1987.tb00813.x
更新日期:1987-05-01 00:00:00
abstract:OBJECTIVE:Epidemiological studies on the cardiovascular risk of progestagen-only contraceptives are rare. With the present study we aimed to investigate the effect of the low-dose etonogestrel-releasing contraceptive implant Implanon on cardiovascular risk factors, including markers of inflammation. DESIGN:Longitudina...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.03046.x
更新日期:2008-03-01 00:00:00
abstract:OBJECTIVE:Mutations in SLC16A2, the gene encoding the thyroid hormone (TH)-specific transporter monocarboxylate transporter 8 (MCT8), result in a thyroid phenotype and severe mental retardation caused by neuronal TH deficiency. These mutational effects raise the question of whether polymorphic variation in SLC16A2 may ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2008.03377.x
更新日期:2009-04-01 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:BACKGROUND:Recent evidence has shown that oxidative stress may play a role in the pathogenesis of autoimmune diseases, and this is an issue of considerable research interest in the field of infiltrative ophthalmopathy. Therefore, we evaluated both the relationship between Graves' ophthalmopathy (GO) and serum levels of...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2010.03904.x
更新日期:2011-01-01 00:00:00
abstract:OBJECTIVE:Primary glucocorticoid resistance is characterized by decreased sensitivity to cortisol signalling. We have performed genetic analysis of the glucocorticoid receptor (GR) gene in 12 unrelated patients with primary cortisol resistance as defined by a pathological dexamethasone suppression test. METHODS:Exon s...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2001.01323.x
更新日期:2001-09-01 00:00:00
abstract::This study deals with serum prolactin concentrations during various conditions in the early puerperium in an attempt to investigate some characteristics of the mechanisms responsible for the regulation of the lactotropes. In nursing, in non-nursing, non-medicated and in non-nursing, bromocriptine-treated women prolact...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1981.tb00650.x
更新日期:1981-08-01 00:00:00
abstract:OBJECTIVES:The differentiation of constitutional delayed puberty (CDP) from gonadotrophin deficiency (GD) in boys at referral poses a difficult challenge. The effectiveness of the GnRH agonist (GnRH-a) test in distinguishing between the two conditions was evaluated and compared with findings of the GnRH and hCG stimula...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2002.01520.x
更新日期:2002-05-01 00:00:00
abstract::Pituitary-dependent Cushing's syndrome is rare in childhood. Two patients are reported who had unsuccessful transsphenoidal microadenomectomy and therefore underwent pituitary radiotherapy. In both patients there was progressive normalization of hypothalamo-pituitary-adrenal function and at 8 and 10 years after radiot...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1998.00385.x
更新日期:1998-05-01 00:00:00
abstract:OBJECTIVE:Mutations in the melanocortin-4 receptor gene (MC4R) are the most common monogenic form of human obesity. However, the contribution of MC4R mutations to obesity in Chinese has not been investigated. We studied the frequency of MC4R mutations in an obese southern Chinese population and the functional consequen...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2006.02573.x
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Women with previous gestational diabetes (pGDM) are at risk of developing Type 2 diabetes. Glucagon-like peptide-1 (GLP-1) potentiates the insulin response to oral glucose, and its secretion is diminished in Type 2 diabetes. The aim of the study was to see if decreased GLP-1 secretion might be ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2004.02172.x
更新日期:2005-01-01 00:00:00
abstract:OBJECTIVE:Staging systems applied to medullary thyroid cancer (MTC) rely on initial clinical and pathological features and do not consider the response to treatment. To determine whether MTC staging can be improved by incorporating the first postoperative calcitonin measurement. PATIENTS AND MEASUREMENTS:Eighty-five p...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12657
更新日期:2015-12-01 00:00:00
abstract::The biological response, as measured in the cytochemical bioassay, to the high circulating levels of immunoreactive parathyroid hormone (iPTH) in patients with pseudohypoparathyroidism type I (PSPI) is affected by the presence of an endogenous inhibitor. This is evidenced by the reduced recovery of the biological acti...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1986.tb03284.x
更新日期:1986-05-01 00:00:00
abstract::We have studied the inositol phospholipid turnover response to thyrotrophin-releasing hormone (TRH) gonadotrophin-releasing hormone (GnRH) and arginine vasopressin (AVP) in five corticotroph and six somatotroph pituitary adenomas. GnRH (100 nM) increased inositol phospholipid turnover in five of five somatotroph adeno...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1990.tb00467.x
更新日期:1990-07-01 00:00:00
abstract:OBJECTIVE:We evaluated menopausal symptoms, menstrual cycle bleeding characteristics and reproductive hormones for their associations with thyroid stimulating hormone (TSH) concentrations in women at the mid-life from five ethnic groups. METHODS:This report is from the baseline evaluation of the Study of Women's Healt...
journal_title:Clinical endocrinology
pub_type: 杂志文章,多中心研究
doi:10.1046/j.1365-2265.2003.01718.x
更新日期:2003-03-01 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:OBJECTIVE:We investigated correlations between age-related changes in circulating metabolites and arterial stiffness in impaired fasting glucose (IFG). DESIGN, SUBJECTS, MEASUREMENT:This prospective cohort study included 602 healthy, normal fasting glucose (NFG) subjects (30-65 years old) who underwent triennial medic...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12821
更新日期:2015-08-01 00:00:00
abstract:OBJECTIVE:Abnormal serum liver function tests are common in patients with untreated thyrotoxicosis, even prior to the initiation of antithyroidal medications that may worsen the severity of the abnormal serum liver biochemistries. There is a wide range of the incidence of these abnormalities in the published literature...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13312
更新日期:2017-05-01 00:00:00
abstract::In the present study, effects of domperidone, a peripheral dopamine blocking agent, on prolactin and growth hormone were examined to determine whether dopamine regulation of these hormones involved structures external to the blood-brain barrier. A clearcut elevation in prolactin was produced by domperidone. GH respons...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1981.tb00666.x
更新日期:1981-09-01 00:00:00
abstract::A single fasting level of serum prolactin was measured in each of sixty control subjects and eighty-three psychiatric patients of both sexes who had been on neuroleptic therapy for 2-4 weeks (acute treatment) or at least 5 years (chronic treatment) and who were aged either 17-45 or 48-85 years. All groups of patients ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1976.tb01953.x
更新日期:1976-05-01 00:00:00
abstract::3-M syndrome is an autosomal recessive primordial growth disorder characterized by small birth size and post-natal growth restriction associated with a spectrum of minor anomalies (including a triangular-shaped face, flat cheeks, full lips, short chest and prominent fleshy heels). Unlike many other primordial short st...
journal_title:Clinical endocrinology
pub_type: 杂志文章,评审
doi:10.1111/j.1365-2265.2012.04428.x
更新日期:2012-09-01 00:00:00
abstract:OBJECTIVE:To examine the association between glycaemic control, insulin resistance and hyperandrogenism on cardiac autonomic function in peripubertal girls with type 1 diabetes. DESIGN:Prospective, clinic-based study of 125 girls with diabetes and 46 age-matched nondiabetic girls. MEASUREMENTS:Heart rate variability ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12238
更新日期:2014-06-01 00:00:00
abstract:BACKGROUND:GH may be beneficial in treating patients with end-stage renal disease (ESRD). However, the efficacy and safety of GH could be compromised by the potential for accumulation in the circulation. OBJECTIVE:The objective was to investigate the pharmacokinetics and safety of GH treatment in ESRD patients. DESIG...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2007.02962.x
更新日期:2007-11-01 00:00:00