Short and long-term responses to metyrapone in the medical management of 91 patients with Cushing's syndrome.


OBJECTIVE:To analyse the clinical and biochemical effects of metyrapone in the treatment of Cushing's syndrome. DESIGN:An evaluation of the standard clinical practice at one institution. PATIENTS:Ninety-one patients with Cushing's syndrome: 57 pituitary-dependent Cushing's disease, 10 adrenocortical adenomas, six adrenocortical carcinomas and 18 ectopic ACTH syndrome. MEASUREMENTS:The acute response to metyrapone was assessed by measuring cortisol, 11-desoxycortisol and ACTH at 0, 1, 2, 3, 4 hours after a test dose of 750 mg of metyrapone. The longer-term effect of metyrapone was judged by measuring serum cortisol at 0900, 1200, 1500, 1800, 2100 and sometimes 2400 h and calculating a mean. RESULTS:A test dose of 750 mg of metyrapone decreased serum cortisol levels within 2 hours in all groups of patients and this effect was sustained at 4 hours. At the same time, serum 11-desoxycortisol levels increased in all patients, while plasma ACTH increased in patients with pituitary Cushing's disease and the ectopic ACTH-syndrome. Fifty-three patients with Cushing's disease were followed on short-term metyrapone therapy (1 to 16 weeks) before other more definitive therapy. Their mean cortisol levels (median 654 nmol/l, range 408-2240) dropped to the target range of less than 400 nmol/l in 40 patients (75%) on a median metyrapone dose of 2250 mg/day (range 750-6000). Metyrapone was given long term in 24 patients with Cushing's disease who had been given pituitary irradiation, for a median of 27 months (range 3-140) with adequate control of hypercortisolaemia in 20 (83%). In 10 patients with adrenocortical adenomas and six with adrenocortical carcinomas, metyrapone in a median dose of 1750 mg/day (range 750-6000) reduced their mean cortisol levels (median 847 nmol/l, range 408-2000) to less than 400 nmol/l in 13 patients (81%). In 18 patients with the ectopic ACTH-syndrome the 'mean cortisol levels', obtained from five or six samples on the test day (median 1023 nmol/l, range 823-6354) were reduced to less than 400 nmol/l in 13 patients (70%), on a median dose of 4000 mg/day (range 1000-6000). Reduction of cortisol levels was clearly associated with clinical and biochemical improvement. The medication was well tolerated. Transient hypoadrenalism and hirsutism were unusual but were the most common side-effects. CONCLUSIONS:In our experience metyrapone remains a most useful agent for controlling cortisol levels in the management of Cushing's syndrome of all types.


Clin Endocrinol (Oxf)


Clinical endocrinology


Verhelst JA,Trainer PJ,Howlett TA,Perry L,Rees LH,Grossman AB,Wass JA,Besser GM





Has Abstract


1991-08-01 00:00:00












  • Carbohydrate metabolism during long-term growth hormone treatment in children with short stature born small for gestational age.

    abstract:OBJECTIVE:To assess possible side-effects of long-term continuous growth hormone (GH) treatment on carbohydrate (CH) metabolism in children with short stature born small for gestational age. DESIGN:In a prospective, randomised double-blind, dose-response multicentre trial, the effect of GH treatment on CH metabolism w...

    journal_title:Clinical endocrinology

    pub_type: 临床试验,杂志文章,随机对照试验


    authors: Sas T,Mulder P,Aanstoot HJ,Houdijk M,Jansen M,Reeser M,Hokken-Koelega A

    更新日期:2001-02-01 00:00:00

  • Small, dense low-density lipoproteins (LDL) are predictors of cardio- and cerebro-vascular events in subjects with the metabolic syndrome.

    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


    authors: Rizzo M,Pernice V,Frasheri A,Di Lorenzo G,Rini GB,Spinas GA,Berneis K

    更新日期:2009-06-01 00:00:00

  • BRAF mutations in an Italian cohort of thyroid cancers.

    abstract:OBJECTIVE:Recently, a somatic point mutation of the BRAF gene (V599E) has been identified as the most common genetic event in papillary thyroid carcinoma (PTC) with a variable frequency (about 25-70%) in different series from USA, Japan, Portugal and Ukraine. DESIGN:In the present study, the genetic analysis of BRAF i...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Fugazzola L,Mannavola D,Cirello V,Vannucchi G,Muzza M,Vicentini L,Beck-Peccoz P

    更新日期:2004-08-01 00:00:00

  • FHIT and TSG101 in thyroid tumours: aberrant transcripts reflect rare abnormal RNA processing events of uncertain pathogenetic or clinical significance.

    abstract:OBJECTIVE:The chromosomal regions containing the two putative tumour suppressors, fragile histidine triad gene (FHIT) and tumour suppressor gene 101 (TSG101), are deleted frequently in thyroid tumours. We therefore analysed FHIT and TSG101 transcripts in a group of advanced thyroid tumours to establish their role in th...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: McIver B,Grebe SK,Wang L,Hay ID,Yokomizo A,Liu W,Goellner JR,Grant CS,Smith DI,Eberhardt NL

    更新日期:2000-06-01 00:00:00

  • Osteocalcin-insulin relationship in obese children: a role for the skeleton in energy metabolism.

    abstract:OBJECTIVE:Osteocalcin is a bone-specific protein secreted by osteoblasts and often used as a bone formation biomarker. Rodent studies have reported a hormonal role of osteocalcin on glucose metabolism, increasing insulin secretion and sensitivity and increasing energy expenditure. However, it is unknown whether osteoca...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Rochefort GY,Rocher E,Aveline PC,Garnero P,Bab I,Chappard C,Jaffré C,Benhamou CL

    更新日期:2011-08-01 00:00:00

  • Bone remodelling markers and serum cytokines in patients with hyperthyroidism.

    abstract:OBJECTIVE:This study was designed in order to evaluate bone turnover with bone formation and resorption markers in hyperthyroidism and its possible relationship with serum cytokines interleukin 6 (IL-6) and tumour necrosis-alpha (TNF-alpha), levels of thyroid hormones and thyroid autoantibodies. DESIGN AND PATIENTS:Tw...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Akalin A,Colak O,Alatas O,Efe B

    更新日期:2002-07-01 00:00:00

  • A double-blind cross-over 12-month study of L-thyroxine treatment of women with 'subclinical' hypothyroidism.

    abstract::Twenty women, who had been randomly selected from women with subclinical hypothyroidism identified in a population study were treated with L-thyroxine and placebo in a double-blind cross-over design during 2 x 6 months. Three women did not complete the study, one because she moved to another part of the country, and t...

    journal_title:Clinical endocrinology

    pub_type: 临床试验,杂志文章


    authors: Nyström E,Caidahl K,Fager G,Wikkelsö C,Lundberg PA,Lindstedt G

    更新日期:1988-07-01 00:00:00

  • Quantification and regulation of adipsin in human cerebrospinal fluid (CSF).

    abstract:CONTEXT:Data on quantification and regulation of adipsin in human cerebrospinal fluid (CSF) are sparse, and the physiological role of adipsin as an adipokine crossing the blood-brain barrier (BBB) is uncertain. OBJECTIVES:This study quantified adipsin concentrations in paired serum and CSF samples of patients undergoi...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Schmid A,Hochberg A,Berghoff M,Schlegel J,Karrasch T,Kaps M,Schäffler A

    更新日期:2016-02-01 00:00:00

  • Physiologic secretion of growth hormone and prolactin in male and female rats.

    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


    authors: Terry LC,Saunders A,Audet J,Willoughby JO,Brazeau P,Martin JB

    更新日期:1977-01-01 00:00:00

  • Pituitary Cushing's disease arising from a previously non-functional corticotrophic chromophobe adenoma.

    abstract::Severe pituitary Cushing's disease of sudden onset after 18 years of unsuccessful treatment for a previously non-functioning chromophobe adenoma is described in a middle-aged woman. Initial presentation with symptoms of optic nerve compression had been preceded by two years of amenorrhoea. Transfrontal resection of a ...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Vaughan NJ,Laroche CM,Goodman I,Davies MJ,Jenkins JS

    更新日期:1985-02-01 00:00:00

  • Hyperkalaemia and selective hypoaldosteronism in myotonic dystrophy.

    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...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


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

    更新日期:2002-02-01 00:00:00

  • Utility of I-124 PET/CT in identifying radioiodine avid lesions in differentiated thyroid cancer: a systematic review and meta-analysis.

    abstract:INTRODUCTION:Diagnostic I-123 scans have been shown to underestimate the disease burden in differentiated thyroid cancer (DTC) when compared to I-131 post-treatment scans, especially in children and patients who have had prior radioiodine (RAI) therapy and/or distant metastasis. I-124 PET/CT has been shown to be highly...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,meta分析,评审


    authors: Santhanam P,Taieb D,Solnes L,Marashdeh W,Ladenson PW

    更新日期:2017-05-01 00:00:00

  • The majority of Danish nontoxic goitre patients are ineligible for Levothyroxine suppressive therapy.

    abstract:OBJECTIVE:Levothyroxine suppressive therapy (LT4-therapy), aimed at shrinking thyroid nodules is controversial. Despite evidence of limited effect and long-term side-effects, questionnaire surveys indicate widespread use. Our aim was to determine, in consecutive nontoxic goitre patients, the proportion ineligible for L...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Fast S,Bonnema SJ,Hegedüs L

    更新日期:2008-10-01 00:00:00

  • Amniotic fluid concentrations of 3,3',5'-tri-iodothyronine (reverse T3), 3,3'-di-iodothyronine, 3,5,3'-tri-iodothyronine (T3) and thyroxine (T4) in normal and complicated pregnancy.

    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


    authors: Meinhold H,Dudenhausen JW,Wenzel KW,Saling E

    更新日期:1979-04-01 00:00:00

  • Recovery of growth hormone secretion following cabergoline treatment of macroprolactinomas.

    abstract:OBJECTIVE:Cabergoline therapy normalizes prolactin levels and reduces the size of macroprolactinomas. However there are no data indicating whether cabergoline can normalize growth hormone secretion in patients who were growth hormone deficient at the time of diagnosis of a macroprolactinoma. SUBJECTS AND METHODS:We st...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: George LD,Nicolau N,Scanlon MF,Davies JS

    更新日期:2000-11-01 00:00:00

  • Shining the light on Sunshine: a systematic review of the influence of sun exposure on type 2 diabetes mellitus-related outcomes.

    abstract::Prospective observational studies uniformly link vitamin D deficiency with the incidence of type 2 diabetes mellitus (T2DM), yet trials supplementing participants at risk of T2DM with vitamin D to reduce progression to T2DM have yielded inconsistent results. Inconsistencies between supplementation trials may be due to...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,评审


    authors: Shore-Lorenti C,Brennan SL,Sanders KM,Neale RE,Lucas RM,Ebeling PR

    更新日期:2014-12-01 00:00:00

  • Preconception management of thyroid dysfunction.

    abstract::Uncorrected thyroid dysfunction in pregnancy has well-recognized deleterious effects on foetal and maternal health. The early gestation period is one of the critical foetal vulnerability during which maternal thyroid dysfunction may have lasting repercussions. Accordingly, a pragmatic preconception strategy is key for...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,评审


    authors: Okosieme OE,Khan I,Taylor PN

    更新日期:2018-09-01 00:00:00

  • Abnormalities of thyrotrophin (TSH) evening rise and pulsatile release in haemodialysis patients: evidence for hypothalamic-pituitary changes in chronic renal failure.

    abstract::Using a sensitive enzyme amplified immunoassay for TSH, the evening rise and pulsatile release of TSH were studied in 10 men with chronic renal failure treated by haemodialysis. Compared to euthyroid male controls the evening rise of TSH was attenuated (median 0.066 vs 0.195 mU/l/h, P less than 0.01) and the rate of r...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Wheatley T,Clark PM,Clark JD,Holder R,Raggatt PR,Evans DB

    更新日期:1989-07-01 00:00:00

  • SRY-negative 46,XX testicular/ovotesticular DSD: Long-term outcomes and early blockade of gonadotropic axis.

    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


    authors: Lambert S,Peycelon M,Samara-Boustani D,Hyon C,Dumeige L,Peuchmaur M,Fiot E,Léger J,Simon D,Paye-Jaouen A,Bouligand J,Siffroi JP,Carel JC,McElreavey K,El Ghoneimi A,Brachet C,Bouvattier C,Martinerie L

    更新日期:2020-12-09 00:00:00

  • Nasogastric feeding enhances nutritional status in paediatric liver disease but does not alter circulating levels of IGF-I and IGF binding proteins.

    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


    authors: Holt RI,Miell JP,Jones JS,Mieli-Vergani G,Baker AJ

    更新日期:2000-02-01 00:00:00

  • High precursor level in maternal blood results from the alternate mode of proopiomelanocortin processing in human placenta.

    abstract:OBJECTIVE:ACTH-producing non-pituitary tumours are often associated with altered precursor processing, particularly in the most aggressive ones. Since placental tissue is characterized by its ability to express the proopiomelanocortin (POMC) gene and rapid cellular proliferation, we examined whether intact POMC could b...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Raffin-Sanson ML,Massias JF,Ankotche A,Coste J,de Keyzer Y,Oliver C,Dumont C,Cabrol D,Ferré F,Bertagna X

    更新日期:1999-01-01 00:00:00

  • Symptoms of thyrotoxicosis, bone metabolism and occult atrial fibrillation in older women with mild endogenous subclinical hyperthyroidism.

    abstract:OBJECTIVE:The objective of this study was to evaluate symptoms of thyrotoxicosis, bone turnover, bone mineral density (BMD) and occult atrial fibrillation (AF) in women ≥65 years with mild endogenous subclinical hyperthyroidism (SCH). DESIGN:Cross-sectional and case-control study. PATIENTS:Signs and symptoms of thyro...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Rosario PW,Carvalho M,Calsolari MR

    更新日期:2016-07-01 00:00:00

  • A new germline VHL gene mutation in three patients with apparently sporadic pheochromocytoma.

    abstract:CONTEXT:Germline mutations in four genes (RET, VHL, SDHB and SDHD) are detected in about 17% of patients with apparently sporadic pheochromocytoma. Thus, genetic screening of all patients with this disease is suggested for a rational diagnostic approach and management. OBJECTIVE:To report the clinical, biochemical and...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: D'Elia AV,Grimaldi F,Pizzolitto S,De Maglio G,Bregant E,Passon N,Franzoni A,Verrienti A,Tamburrano G,Durante C,Filetti S,Fogolari F,Russo D,Damante G

    更新日期:2013-03-01 00:00:00

  • The PPAR-gamma activator rosiglitazone fails to lower plasma ACTH levels in patients with Nelson's syndrome.

    abstract:BACKGROUND:Peroxisomal proliferator-activated receptors (PPAR)- gamma are expressed abundantly in ACTH-secreting pituitary tumours. The PPAR-gamma activator rosiglitazone has been shown to suppress ACTH secretion in human adrenocorticotroph tumour cells in vitro, and prevent and reduce adrenocorticotroph tumour develop...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Mullan KR,Leslie H,McCance DR,Sheridan B,Atkinson AB

    更新日期:2006-05-01 00:00:00

  • Interest of Chromogranin A for diagnosis and follow-up of endocrine tumours.

    abstract:OBJECTIVE:To determine the interest of Chromogranin A (CgA) determination for diagnosis and follow-up in patients with gastroenteropancreatic endocrine tumours (GEP-ET) and multiple endocrine neoplasia type 1 (MEN-1). PATIENTS AND METHODS:CgA levels were measured with an immunoradiometric assay in 124 sporadic GEP-ET,...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Nehar D,Lombard-Bohas C,Olivieri S,Claustrat B,Chayvialle JA,Penes MC,Sassolas G,Borson-Chazot F

    更新日期:2004-05-01 00:00:00

  • The sodium-iodide symporter expression in placental tissue at different gestational age: an immunohistochemical study.

    abstract:BACKGROUND:Iodide (I(-)) is crucial for foetal thyroid function. Foetal iodide results from maternal circulating iodide and from deiodination of iodothyronines within the placenta. The Na(+)/I(-) symporter (NIS) localized in placental cells appears to be involved in iodide exchange. Low NIS expression has been reported...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Di Cosmo C,Fanelli G,Tonacchera M,Ferrarini E,Dimida A,Agretti P,De Marco G,Vitti P,Pinchera A,Bevilacqua G,Naccarato AG,Viacava P

    更新日期:2006-10-01 00:00:00

  • Effects of exercise-induced weight loss on acylated and unacylated ghrelin in overweight children.

    abstract:OBJECTIVE:Controversial data on ghrelin concentration during exercise in human subjects have been published. We tested the hypothesis that exercise could affect acylated ghrelin (AG) and unacylated ghrelin (UAG), which could partly explain the previously reported inconsistent findings on the association of exercise wit...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章,随机对照试验


    authors: Kim HJ,Lee S,Kim TW,Kim HH,Jeon TY,Yoon YS,Oh SW,Kwak H,Lee JG

    更新日期:2008-03-01 00:00:00

  • An improved method for the detection of heterozygosity of congenital virilizing adrenal hyperplasia.

    abstract::A modified short ACTH test for the detection of heterozygote carriers of 21-hydroxylase deficiency (21-OHD) was applied to twenty-one controls and fourteen parents of children with 21-OHD. The following modifications were introduced: (1) Endogenous ACTH was suppressed by dexamethasone administration prior to the test,...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Lejeune-Lenain C,Cantraine F,Dufrasnes M,Prevot F,Wolter R,Franckson JR

    更新日期:1980-06-01 00:00:00

  • Maternal hypothyroxinaemia during early pregnancy and subsequent child development: a 3-year follow-up study.

    abstract:OBJECTIVE:To evaluate the impact of maternal hypothyroxinaemia during early gestation (fT4 below the lowest tenth percentile and TSH within the reference range: 0.15-2.0 mIU/l) on infant development, together with any subsequent changes in fT4 during gestation. DESIGN:A prospective 3-year follow-up study of pregnant w...

    journal_title:Clinical endocrinology

    pub_type: 杂志文章


    authors: Pop VJ,Brouwers EP,Vader HL,Vulsma T,van Baar AL,de Vijlder JJ

    更新日期:2003-09-01 00:00:00

  • 'What should I do with a 60-year old man with a slightly low serum total testosterone concentration and normal levels of serum gonadotrophins'?

    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


    authors: Hugh Jones T

    更新日期:2010-05-01 00:00:00