Hyperkalaemia and selective hypoaldosteronism in myotonic dystrophy.


: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.


Clin Endocrinol (Oxf)


Clinical endocrinology


Misra D,DeSilva S,Fellerman H,Dufour DR,Streeten DH,Nylen ES





Has Abstract


2002-02-01 00:00:00














  • Inhibin B levels in azoospermic subjects with cytologically characterized testicular pathology.

    abstract:BACKGROUND AND OBJECTIVE:Inhibin B, a heterodimeric glycoprotein of gonadal origin, is the most important circulating form of inhibin in human males and an inverse relationship between inhibin B and FSH plasma levels was been recently observed. Azoospermia represents the end-point of different kinds of testicular damag...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Foresta C,Bettella A,Petraglia F,Pistorello M,Luisi S,Rossato M

    更新日期:1999-06-01 00:00:00

  • Clinical utility of telomerase for the diagnosis of malignant well-differentiated endocrine tumours.

    abstract:OBJECTIVE:The distinction between benign and malignant well-differentiated endocrine tumours is hard to achieve. The aim of the present study was to determine whether detection of telomerase or quantification of human telomerase reverse transcriptase protein subunit (hTERT) differ between benign and malignant endocrine...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Vezzosi D,Bouisson M,Escourrou G,Laurell H,Selves J,Seguin P,Pradayrol L,Caron P,Buscail L

    更新日期:2006-01-01 00:00:00

  • Phenotypic and molecular characteristics in eleven Chinese patients with 5α-reductase Type 2 deficiency.

    abstract:CONTEXT:Steroid 5α-reductase type 2 deficiency (5α-RD2) is a male-limited, autosomal recessive inherited disease. Affected 46, XY individuals usually present with ambiguous genitalia at birth. An early and precise diagnosis is of great value to the long-term prognosis of the disease. OBJECTIVE:To describe the clinical...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Zhu H,Liu W,Han B,Fan M,Zhao S,Wang H,Lu Y,Pan C,Chen F,Chen M,Song H,Cheng K,Qiao J

    更新日期:2014-11-01 00:00:00

  • Body fat predicts an increase and limb muscle strength predicts a decrease in leptin in older adults over 2·6 years.

    abstract:OBJECTIVE:Obesity is characterized by hyperleptinaemia, which is associated with diabetes, hypertension and coronary heart disease. The aim of this study was to determine if body fat and muscle measures predict the natural increase in leptin over 2·6 years in older adults. METHODS:A total of 190 subjects (50% females)...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Antony B,Jones G,Stannus O,Blizzard L,Ding C

    更新日期:2013-11-01 00:00:00

  • Growth hormone (GH) enhances anaerobic capacity: impact on physical function and quality of life in adults with GH deficiency.

    abstract:CONTEXT:Anaerobic capacity is impaired in adults with GH deficiency (GHD), adversely affecting physical function and quality of life (QoL). OBJECTIVE:To investigate whether GH replacement improves anaerobic capacity, physical function and QoL in adults with GHD. DESIGN:One-month double-blind placebo-controlled crosso...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Chikani V,Cuneo RC,Hickman I,Ho KK

    更新日期:2016-10-01 00:00:00

  • Fasting concentrations of nesfatin-1 are negatively correlated with body mass index in non-obese males.

    abstract:BACKGROUND:We recently identified a novel anorexigenic protein, nesfatin-1, which is processed from nesfatin/nucleobindin-2 (NUCB2). However, the clinical importance of this protein has not been determined. OBJECTIVE:To investigate its clinical significance in humans, we have established a new specific enzyme-linked i...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Tsuchiya T,Shimizu H,Yamada M,Osaki A,Oh-I S,Ariyama Y,Takahashi H,Okada S,Hashimoto K,Satoh T,Kojima M,Mori M

    更新日期:2010-10-01 00:00:00

  • Radioimmunoassay artifact associated with circulating anti-rabbit antibodies.

    abstract::A 30 year old woman presenting with infertility was found to have elevated serum immunoreactive prolactin, TSH and total T3. These findings were eventually attributed to assay artifact when she was shown to have anti-rabbit antibodies in her serum. The possibility of laboratory artifact should be considered in clinica...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Jeffcoate WJ,Selby C,Powell RJ

    更新日期:1988-01-01 00:00:00

  • Osmoregulation of thirst and vasopressin secretion in Kallmann's syndrome.

    abstract::It has been suggested that abnormalities of thirst and vasopressin secretion commonly coexist with Kallmann's syndrome. Out-patient plasma osmolality, plasma sodium and 24-hour urine volume were similar in 10 patients with Kallmann's syndrome and 10 matched controls. Six patients underwent dynamic testing of osmoregul...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Thompson CJ,White MC,Baylis PH

    更新日期:1989-05-01 00:00:00

  • Prediction of recurrence of nonfunctioning pituitary tumours by loss of heterozygosity analysis.

    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


    authors: Buch HN,Raskauskiene D,Bahar A,Bicknell EJ,Farrell WE,Clayton RN

    更新日期:2004-07-01 00:00:00

  • Blockage of the positive feedback effect of oestradiol during prolonged administration of clomiphene citrate to normal women.

    abstract::The effect of clomiphene citrate on the occurrence and timing of the endogenous LH surge in superovulated cycles is unclear. To study further this event, five normally ovulating women were treated with clomiphene citrate 100 mg per day in two different cycles, that is, for 5 days in one cycle (days 2 to 6, CC-5) and f...

    journal_title:Clinical endocrinology

    pub_type: 临床试验,杂志文章,随机对照试验


    authors: Messinis IE,Templeton A

    更新日期:1988-11-01 00:00:00

  • The glutamine 27 glutamic acid polymorphism of the beta2-adrenoceptor gene is associated with abdominal obesity and greater risk of impaired glucose tolerance in men but not in women: a population-based study in Spain.

    abstract:OBJECTIVE:Given the important role of the beta2-adrenoceptor (beta2-AR) in lipid mobilization and the lack of studies in Southern European countries, the aim of this study was to investigate the role of the glutamine 27 glutamic acid (Gln27Glu) beta2-AR polymorphism in the susceptibility to obesity and its metabolic co...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: González Sánchez JL,Proenza AM,Martínez Larrad MT,Ramis JM,Fernández Pérez C,Palou A,Serrano Ríos M

    更新日期:2003-10-01 00:00:00

  • The basal plasma glucose: a simple relevant index of maturity-onset diabetes.

    abstract::The 'basal' plasma glucose, defined as the stable overnight concentration, has been assessed as an index of diabetes control by comparison with plasma glucose measurements during 24-h profiles in diet-treated maturity-onset diabetic patients and normal subjects. The basal plasma glucose correlated with the total (r=0....

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Holman RR,Turner RC

    更新日期:1981-03-01 00:00:00

  • Reference interval for albumin-adjusted calcium based on a large UK population.

    abstract:CONTEXT:Primary hyperparathyroidism is a common condition and results in hypercalcaemia, especially in older women. Thus, it is critical to obtain a robust estimate for the upper limit of the reference interval for albumin-adjusted serum calcium in the general population. The current reference interval in use in the UK...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Schini M,Hannan FM,Walsh JS,Eastell R

    更新日期:2021-01-01 00:00:00

  • Autoimmune thyroid disease and chronic urticaria.

    abstract::We report six cases of autoimmune thyroid disease associated with chronic urticaria and briefly review the literature, including the histopathological nature of such lesions, and their aetiology and pathogenesis. In view of the prevalence of thyroid disease in patients with chronic urticaria, screening measurements of...

    journal_title:Clinical endocrinology

    pub_type: 信件


    authors: Monge C,Demarco P,Burman KD,Wartofsky L

    更新日期:2007-09-01 00:00:00

  • Serum prolactin concentration in patients taking neuroleptic drugs.

    abstract:OBJECTIVE:Non-tumour causes of hyperprolactinaemia, including prolactin-elevating drugs, must be excluded. There is a general view that such drugs are unlikely to raise serum PRL above 3000 mU/I, but the literature is confusing. We report 8 patients receiving treatment with neuroleptic drugs, whose serum PRL concentrat...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Pollock A,McLaren EH

    更新日期:1998-10-01 00:00:00

  • Autonomous nodular hyperplasia of the adrenal cortex: tertiary hypercortisolism?

    abstract::Two cases of Cushing's syndrome are reported in which apparently autonomous adrenal adenomata were associated with the presence of pituitary tumours. In one case the patient was apparently cured by unilateral adrenalectomy, although she was noted to have radiological evidence of an intrasellar tumour; serum cortisol w...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Sturrock ND,Morgan L,Jeffcoate WJ

    更新日期:1995-12-01 00:00:00

  • Insulin-like growth factor-II: its role in metabolic and endocrine disease.

    abstract::Insulin-like growth factor-II (IGF-II) is a widely expressed 7·5 kDa mitogenic peptide hormone. Although it is abundant in serum, understanding of its physiological role is limited compared with that of IGF-I. IGF-II regulates foetal development and differentiation, but its role in adults is less well understood. Evid...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,评审


    authors: Livingstone C,Borai A

    更新日期:2014-06-01 00:00:00

  • Studies in constitutionally tall adolescents: somatostatin decrease associated with growth hormone increase after TRH injection.

    abstract::The paradoxical GH response to TRH observed in certain adolescents with constitutional tall stature is associated with a 60% fall in plasma levels of somatostatin. Conversely, those tall subjects who do not show a GH increase after TRH, or during a saline control infusion maintain steady levels of somatostatin in plas...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Garcia Blanco M,Evain Brion D

    更新日期:1984-10-01 00:00:00

  • Iodine status in UK-An accidental public health triumph gone sour.

    abstract::The improvement in iodine status among the UK population from the 1930s onwards has been described as an 'accidental public health triumph' despite the lack of any iodine fortification program. However, iodine deficiency in the UK has re-emerged in vulnerable groups and is likely due to a combination of changing farmi...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,评审


    authors: Woodside JV,Mullan KR

    更新日期:2020-11-29 00:00:00

  • Inhibin as a marker for hydatidiform mole: a comparative study with the determinations of intact human chorionic gonadotrophin and its free beta-subunit.

    abstract:OBJECTIVE:The purpose of the study was to evaluate plasma inhibin as a marker of hydatidiform mole and to compare the results with intact human chorionic gonadotrophin (hCG) and its free beta-subunit. DESIGN:Serial determinations of the plasma concentrations of inhibin, intact human chorionic gonadotrophin and its fre...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Badonnel Y,Barbé F,Legagneur H,Poncelet E,Schweitzer M

    更新日期:1994-08-01 00:00:00

  • Both intrauterine growth restriction and postnatal growth influence childhood serum concentrations of adiponectin.

    abstract:OBJECTIVE:Insulin resistance has been linked to intrauterine growth restriction; adiponectin is a strong determinant of insulin sensitivity. We aimed at studying the contributions of birthweight and insulin sensitivity to circulating adiponectin in children born small for gestational age (SGA). DESIGN:Cross-sectional,...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: López-Bermejo A,Casano-Sancho P,Fernández-Real JM,Kihara S,Funahashi T,Rodríguez-Hierro F,Ricart W,Ibañez L

    更新日期:2004-09-01 00:00:00

  • Plasma oestrone-fatty acid ester levels are correlated with body fat mass in humans.

    abstract:OBJECTIVE:The metabolites of steroidal hormones, including sulphate, glucuronide, and fatty acid (FA) ester derivatives, have received little attention, although these steroid derivatives are essential components in the global assessment of steroid metabolism. The study of FA-derivatives could, in obesity, contribute s...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Fernández-Real JM,Sanchis D,Ricart W,Casamitjana R,Balada F,Remesar X,Alemany M

    更新日期:1999-02-01 00:00:00

  • Effects of growth hormone therapy on the developmental changes of follicle stimulating hormone and insulin-like growth factor-I serum concentrations in Turner's syndrome.

    abstract:OBJECTIVE:The aim was to investigate whether, in the absence of gonads, GH could bring forward the age of neuroendocrine activation resulting in onset of puberty. DESIGN:In girls with Turner's syndrome, we evaluated the effects of GH therapy on developmental changes in FSH serum concentrations used as an indicator of ...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Bourguignon JP,Gérard A,Deby-Dupont G,Franchimont P

    更新日期:1993-07-01 00:00:00

  • Polycystic ovary syndrome has no independent effect on vascular, inflammatory or thrombotic markers when matched for obesity.

    abstract:INTRODUCTION:Previous studies investigating cardiovascular (CV) risk in obese women with polycystic ovary syndrome (PCOS) have been potentially confounded by not adequately accounting for body weight. OBJECTIVE:To assess if PCOS increases CV risk independently in young obese women by examining carotid intima-media wal...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Kahal H,Aburima A,Ungvari T,Rigby AS,Dawson AJ,Coady AM,Vince RV,Ajjan RA,Kilpatrick ES,Naseem KM,Atkin SL

    更新日期:2013-08-01 00:00:00

  • Bone mineral density and fractures in congenital adrenal hyperplasia: Findings from the dsd-LIFE study.

    abstract:BACKGROUND:In patients with congenital adrenal hyperplasia (CAH) type and doses of glucocorticoids used as well as sex hormone secretion during puberty have important actions on bone mineral density (BMD) in adulthood. AIM:To evaluate BMD in adult CAH patients depending on current glucocorticoid therapy and on androge...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Riehl G,Reisch N,Roehle R,Claahsen van der Grinten H,Falhammar H,Quinkler M

    更新日期:2020-04-01 00:00:00

  • Calcium and parathyroid hormone behaviour after exogenous secretin infusion in man.

    abstract::The response of serum calcium, PTH, CT and gastrin to an infusion of secretin (3 CU/kg/h) over a period of 90 min was studied in ten healthy males and two female patients with hypoparathyroidism. In the healthy males serum calcium, after a transient fall, significantly increased at 45 min after the onset of the infusi...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Lo Cascio V,Piubello W,Cominacini L,Vantini I,Galvanini G,Ederle A,Cavallini G,Scuro LA

    更新日期:1982-04-01 00:00:00

  • The effect of propylthiouracil on subsequent radioactive iodine therapy in Graves' disease.

    abstract:OBJECTIVE:Antithyroidal drugs (ATD) are used in the management of Graves' disease either as primary therapy for several months while awaiting remission of the disease or as pretreatment for several weeks prior to definitive radioactive iodine therapy (RAI). We have reported previously that pretreatment with propylthiou...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Hancock LD,Tuttle RM,LeMar H,Bauman J,Patience T

    更新日期:1997-10-01 00:00:00

  • Growth hormone pretreatment in man blocks the response to growth hormone-releasing hormone; evidence for a direct effect of growth hormone.

    abstract::The effect of pretreatment with biosynthetic methionyl human GH (hGH) on the GH response to GHRH has been studied in normal subjects. Eight volunteers were given either 4 IU hGH or placebo s.c. 12-hourly for 72 h before a GHRH test, or a single s.c. dose of 4 IU hGH 12 h before a GHRH test. Somatomedin-C (Sm-C) levels...

    journal_title:Clinical endocrinology

    pub_type: 临床试验,杂志文章


    authors: Ross RJ,Borges F,Grossman A,Smith R,Ngahfoong L,Rees LH,Savage MO,Besser GM

    更新日期:1987-01-01 00:00:00

  • Effects of exercise training on maternal hormonal changes in pregnancy.

    abstract:CONTEXT & OBJECTIVE:A recent paper by our group reported that regular aerobic exercise during pregnancy led to lower foetal IGF-I and IGF-II concentrations and a modest reduction in offspring birth weight when compared with the offspring of nontraining control participants. Maternal hormonal alterations in response to ...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,随机对照试验


    authors: Hopkins SA,Baldi JC,Cutfield WS,McCowan L,Hofman PL

    更新日期:2011-04-01 00:00:00

  • ACTH-independent Cushing's syndrome in pregnancy with spontaneous resolution after delivery: control of the hypercortisolism with metyrapone.

    abstract::A 25-year-old primigravid woman presented with Cushing's syndrome at 23 weeks gestation; serum cortisol was 1090 nmol/l at 0900 h, 1230 nmol/l at 2200 h; basal urinary free cortisol excretion was 3680 nmol/24 h, and 8830 nmol/24 h after dexamethasone 8 mg daily for 48 hours; plasma ACTH was < 1.1 pmol/l. CT scan of th...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Close CF,Mann MC,Watts JF,Taylor KG

    更新日期:1993-09-01 00:00:00