Abstract:
:Myotonic dystrophy (MyD) is a common genetic neuromuscular disorder in which chromosome 19 gives rise to an abnormal expansion of CTG-trinucleotide repeats. MyD is a highly variable multisystem disorder with muscular and nonmuscular abnormalities. Increasingly, endocrine abnormalities, such as gonadal, pancreatic, and adrenal dysfunction are being uncovered. Herein we present three unrelated cases with MyD with abnormally elevated serum potassium; 2 of the 3 cases presented clinically with cardiac dysrhythmias. Hyperkalaemic conditions such as renal failure, cortisol deficiency, pseudohyperkalaemia, and hyperkalaemic periodic paralysis were excluded. Further endocrine evaluation revealed baseline hypoaldosteronism associated with elevated renin activity. Perturbation of the renin-angiotensin-aldosterone system resulted in appropriately enhanced renin activity but with a subnormal aldosterone response, which appeared to be due to adrenal hyporesponsiveness. The treatment of all cases with fludrocortisone was without effect. Whether the apparent mineralocorticoid abnormality in MyD is due to associated hormonal perturbations (i.e. excessive ACTH responsiveness. elevated cytokines, elevated atrial natriuretic hormone, etc.), adrenal atrophy, and/or a manifestation of the underlying kinase dysfunction is uncertain, but merits further evaluation in view of the clinical consequence of hyperkalaemia.
journal_name
Clin Endocrinol (Oxf)journal_title
Clinical endocrinologyauthors
Misra D,DeSilva S,Fellerman H,Dufour DR,Streeten DH,Nylen ESdoi
10.1046/j.0300-0664.2001.01452.xkeywords:
subject
Has Abstractpub_date
2002-02-01 00:00:00pages
271-5issue
2eissn
0300-0664issn
1365-2265pii
1452journal_volume
56pub_type
杂志文章abstract::The fundamental question in assessing an older man with a slightly low total testosterone and normal gonadotrophin levels is to determine whether or not he has clinical hypogonadism. Hypogonadism is defined as a clinical syndrome complex, which comprises both symptoms as well as biochemical testosterone deficiency. As...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2009.03737.x
更新日期:2010-05-01 00:00:00
abstract::Growth hormone and prolactin are secreted episodically in man and experimental animals. To investigate physiologic mechanisms of GH and PRL secretion, a series of experiments were performed in individual, unanaesthetized male and female rats. GH secretion in the male rat is characterized by intermittent surges that oc...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1977.tb03335.x
更新日期:1977-01-01 00:00:00
abstract:OBJECTIVE:Pituitary responsiveness to GnRH varies throughout the normal menstrual cycle. We have investigated whether there are differences in the ovarian mechanisms which regulate gonadotrophin secretion between the follicular and the luteal phase of the cycle. DESIGN:Normally ovulating women were studied during the ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.1997.3461141.x
更新日期:1997-12-01 00:00:00
abstract::Amniotic fluid concentrations of 3,3',5'-tri-iodothyronine (rT3), 3,3'-Di-iodothyronine (3,3'-T2), 3,5,3'-tri-iodothyronine (T3) and T4 were studied in 384 women during normal and complicated pregnancy. An inverse correlation was observed between decreasing rT3 and increasing 3,3'-T2 concentrations in amniotic fluid w...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1979.tb02090.x
更新日期:1979-04-01 00:00:00
abstract:CONTEXT:Childbearing is considered to be a significant risk factor for developing overweight and obesity. Physical activity might influence weight change via hormonal changes. OBJECTIVE:To test the hypothesis that higher levels of moderate-to-vigorous intensity physical activity (MVPA) are positively associated with m...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12593
更新日期:2015-01-01 00:00:00
abstract::The initial presentation of macroprolactinoma with visual field impairment, especially in males, is well recognized. Successful treatment with dopamine agonist therapy is characterized by a reduction in hyperprolactinaemia and often rapid and progressive resolution of the visual impairment. A small proportion of patie...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2000.01039.x
更新日期:2000-10-01 00:00:00
abstract:OBJECTIVE:When the gonadotrophin levels increase at midcycle, more basic isoforms of FSH and LH appear in the circulation. However, when these gonadotrophins increase at menopause more acidic forms appear. The present study was done to see whether chronic 17 beta-oestradiol (E2) administration to post-menopausal women ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1994.tb02513.x
更新日期:1994-06-01 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::The growth hormone-insulin-like growth factor 1 (GH-IGF-1) axis plays an important role in modulating the peripheral metabolism of glucocorticoids mainly through its effect on the isoenzyme 11 beta-hydroxysteroid dehydrogenase 1 (11beta-HSD1) which, in vivo, functions as a reductase catalysing the conversion of cortis...
journal_title:Clinical endocrinology
pub_type: 杂志文章,评审
doi:10.1111/j.1365-2265.2007.02763.x
更新日期:2007-04-01 00:00:00
abstract:OBJECTIVE:Patients with acromegaly demonstrate impaired quality of life (QoL), but data on long-term QoL changes in treated acromegaly are limited. This study evaluates and identifies factors that influence QoL in patients with long-term biochemical remission. DESIGN:The study consists of a cross-sectional arm compari...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.13331
更新日期:2017-06-01 00:00:00
abstract:OBJECTIVE:There are no consistent data on the prevalence and bone status of normocalcaemic hypoparathyroidism (NHYPO) as defined by normal adjusted calcium and low PTH level. Our aim was to determine the prevalence and the metabolic bone profile of NHYPO in older women, assessing its evolution over time. The second obj...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.12732
更新日期:2015-06-01 00:00:00
abstract:OBJECTIVE:Gitelman's syndrome, recognized as a variant of Bartter's syndrome, is characterized by hypokalaemic metabolic alkalosis in combination with hypomagnesaemia and hypocalciuria. Overlapping biochemical features in Gitelman's syndrome and Bartter's syndrome has been observed. Here, we investigated the clinical, ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2009.03649.x
更新日期:2010-02-01 00:00:00
abstract::Three different forms of testosterone (T) replacement therapy were compared; they were the intramuscular injection of mixed testosterone esters 250 mg; the subcutaneous implantation of 6 X 100 mg pellets of fused testosterone; and the oral administration of testosterone undecanoate (TU) 80 mg twice daily. Six hypogona...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1984.tb03448.x
更新日期:1984-08-01 00:00:00
abstract:OBJECTIVE:We examined the charge heterogeneity and carbohydrate complexity of hCG in healthy pregnant Asian subjects and Asian women with gestational thyrotoxicosis to assess whether particular glycoforms of hCG are associated with the thyrotoxicosis of pregnancy. DESIGN:Blood was taken at 8-16 weeks of gestation from...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2001.01316.x
更新日期:2001-07-01 00:00:00
abstract::This study was to examine indirectly the effect of endogenous progesterone, a known competitor for 5 alpha-reductase, on androgen metabolism in target organs in hirsute women. Serum levels of progesterone, testosterone (T), androstenedione (A), dihydrotestosterone (DHT) and 5 alpha-androstane 3 alpha 17 beta-diol (3 a...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1984.tb03225.x
更新日期:1984-10-01 00:00:00
abstract:OBJECTIVE:Patients on long-term dialysis may develop secondary hyperparathyroidism (SHPT), which causes varying degrees of bone mass loss. This condition is treated with parathyroidectomy (PTX). We investigated whether serial serum bone turnover markers could predict changes in bone mineral density (BMD) after PTX. DE...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2011.04265.x
更新日期:2012-05-01 00:00:00
abstract:BACKGROUND:Type 1 diabetes mellitus (T1DM) is associated with skeletal fragility. While previous meta-analyses have demonstrated an increased risk of fracture in individuals with T1DM, little is known about fracture risk in T1DM, in the absence of age-related confounders. AIMS:To determine the risk of fracture in youn...
journal_title:Clinical endocrinology
pub_type: 杂志文章,meta分析,评审
doi:10.1111/cen.13761
更新日期:2018-09-01 00:00:00
abstract:OBJECTIVE:Women with polycystic ovary syndrome (PCOS) have an increased risk of cardiovascular disease. The contribution of lipid abnormalities to this higher risk, in particular atherogenic modifications of low density lipoprotein (LDL) such as a shift towards smaller LDL, has not been properly explored. We aimed to e...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2001.01245.x
更新日期:2001-04-01 00:00:00
abstract:OBJECTIVE:Complications of childhood cirrhosis include abnormal growth and malnutrition, associated with abnormalities in circulating IGFs and IGFBPs. Controlled studies suggest that intensive enteral feeding enhances nutritional status. The aim was to ascertain whether nasogastric feeding improves nutritional status i...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1046/j.1365-2265.2000.00934.x
更新日期:2000-02-01 00:00:00
abstract::Endocrine studies, using the four hypothalamic releasing hormones, gonadotrophin releasing hormone, thyrotrophin releasing hormone, corticotrophin releasing hormone and growth hormone releasing hormone, were performed in 33 males after admission with a major head injury. The test was repeated 7 days later in all 33 pa...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1988.tb00257.x
更新日期:1988-08-01 00:00:00
abstract::We designed a systematic study of patients with Cushing's disease to compare the results of acute experiments with cyproheptadine, sodium valproate and bromocriptine with the results of chronic treatment with sodium valproate. In 13 patients the plasma cortisol response to single doses of 2.5 mg bromocriptine, 6 mg cy...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1986.tb03621.x
更新日期:1986-12-01 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:OBJECTIVES:The recent American Thyroid Association management guidelines suggest cervical ultrasonography (US) surveillance in patients with differentiated thyroid cancer (DTC). This study was conducted to identify predictive factors that can determine whether subsequent fine needle aspiration guided by ultrasound (US-...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2010.03915.x
更新日期:2011-02-01 00:00:00
abstract:OBJECTIVE:Observational studies have suggested positive associations between serum 25-hydroxyvitamin D (25(OH)D) levels and muscular strength, balance and quality of life. Our aim was to examine whether high-dose vitamin D supplementation would improve these measures as compared to standard-dose vitamin D, as well as t...
journal_title:Clinical endocrinology
pub_type: 杂志文章,随机对照试验
doi:10.1111/cen.13353
更新日期:2017-07-01 00:00:00
abstract::We studied day/night (D:N) patterns of urinary sodium excretion and the 24 hour ambulatory electrocardiogram in seven normal subjects before and during the administration of T4. Thyroxine increased thyroid hormone levels within the normal range and inhibited the plasma TSH response to TRH. This was associated with a s...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1983.tb02881.x
更新日期:1983-05-01 00:00:00
abstract:OBJECTIVE AND DESIGN:The immediate early gene locus AP-1, incorporating the cellular oncogenes c-fos and c-jun (and their oncoprotein products Fos and Jun respectively) play a key role in regulating cell growth and differentiation. The myc-gene is also known to promote cell growth. In order to investigate the possible ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1994.tb02453.x
更新日期:1994-01-01 00:00:00
abstract:OBJECTIVE:Small, dense low-density lipoproteins (LDL) are a feature of the metabolic syndrome (MS) but their predictive role still remains to be established. We performed a 2-year follow-up study in 124 subjects with MS (63 +/- 6 years), as defined by the American Heart Association/National Heart, Lung and Blood Instit...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.2008.03407.x
更新日期:2009-06-01 00:00:00
abstract::Serum thyroxine was consistently unmeasurable by radioimmunoassay in an elderly patient with myxoedema after successful treatment with oral thyroxine. Abnormal binding of thyroxine was suspected and shown to be due to the presence in serum of antibodies of the IgG variety. The characteristics of these antibodies with ...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/j.1365-2265.1981.tb02741.x
更新日期:1981-07-01 00:00:00
abstract:OBJECTIVE:SRY-negative 46,XX testicular and ovotesticular disorders/differences of sex development (T/OTDSD) represent a very rare and unique DSD condition where testicular tissue develops in the absence of a Y chromosome. To date, very few studies have described the phenotype, clinical and surgical management and long...
journal_title:Clinical endocrinology
pub_type: 杂志文章
doi:10.1111/cen.14389
更新日期:2020-12-09 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