Abstract:
PURPOSE:Nelson's syndrome is a challenging condition that can develop following bilateral adrenalectomy for Cushing's disease, with high circulating ACTH levels, pigmentation and an invasive pituitary tumor. There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson's syndrome. METHODS:Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1 month pasireotide 300-600 μg s.c. twice-daily; (3) 6 months pasireotide long-acting-release (LAR) 40-60 mg monthly. RESULTS:Seven patients had s.c. treatment and 5 proceeded to LAR treatment. There was a significant reduction in morning plasma ACTH during treatment (mean ± SD; 1823 ± 1286 ng/l vs. 888.0 ± 812.8 ng/l during the s.c. phase vs. 829.0 ± 1171 ng/l during the LAR phase, p < 0.0001). Analysis of ACTH levels using a random intercept linear mixed-random effects longitudinal model showed that ACTH (before the morning dose of glucocorticoids) declined significantly by 26.1 ng/l per week during the 28-week of treatment (95% CI - 45.2 to - 7.1, p < 0.01). An acute response to a test dose predicted outcome in 4/5 patients. Overall, there was no significant change in tumor volumes (1.4 ± 0.9 vs. 1.3 ± 1.0, p = 0.86). Four patients withdrew during the study. Hyperglycemia occurred in 6 patients. CONCLUSIONS:Pasireotide lowers plasma ACTH levels in patients with Nelson's syndrome. A longer period of treatment may be needed to assess the effects of pasireotide on tumor volume. TRIAL REGISTRATION:Clinical Trials.gov ID, NCT01617733.
journal_name
Pituitaryjournal_title
Pituitaryauthors
Daniel E,Debono M,Caunt S,Girio-Fragkoulakis C,Walters SJ,Akker SA,Grossman AB,Trainer PJ,Newell-Price Jdoi
10.1007/s11102-017-0853-3subject
Has Abstractpub_date
2018-06-01 00:00:00pages
247-255issue
3eissn
1386-341Xissn
1573-7403pii
10.1007/s11102-017-0853-3journal_volume
21pub_type
杂志文章相关文献
Pituitary文献大全abstract::Ideal surgical treatment of craniopharyngiomas remains a major challenge for neurosurgeons. Craniopharyngiomas grow in the deep-seated hypothalamic area that is paramount for vegetative, emotional and endocrine function, and for maintaining worthwhile life. The benign histological nature of craniopharyngiomas belies t...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-008-0137-z
更新日期:2008-01-01 00:00:00
abstract::We report the case of a 60 year old male who complained of headache and blurry vision--that progressed to left ophthalmoplegia and ptosis--after receiving a dose of leuprolide for Prostate cancer therapy. Imaging showed a hemorrhagic sellar mass. The patient underwent transsphenoidal debulking, and the tissue obtained...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-009-0202-2
更新日期:2010-01-01 00:00:00
abstract:INTRODUCTION:There has been growing interest on medical therapy for the management of Cushing's disease (CD), particularly in cases of persistent or recurrent hypercortisolism. Ketoconazole, an inhibitor of adrenal steroidogenesis, is the most widely used drug, whereas cabergoline and pasireotide are the most promising...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-015-0641-x
更新日期:2015-04-01 00:00:00
abstract::We describe two young men with cystic pituitary enlargement on magnetic resonance imaging (MRI) causing hypopituitarism. The first patient presented acutely unwell with headache and vomiting associated with anterior and posterior pituitary dysfunction. The second patient presented with hypopituitarism after a long his...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1023/b:pitu.0000011177.43408.56
更新日期:2003-01-01 00:00:00
abstract:PURPOSE:Recent guidelines suggest that a single prolactin measurement is adequate to confirm hyperprolactinaemia. This may lead to unnecessary investigation of artefactual hyperprolactinaemia. Prolactin measurement drawn from an indwelling cannula after rest removes stress as a confounding variable. The objective was t...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-014-0576-7
更新日期:2015-06-01 00:00:00
abstract:INTRODUCTION:In order to perform trans-sphenoidal endoscopic pituitary surgery safely and efficiently it is important to identify anatomical and pituitary disease features on the pre-operative CT and MRI scans; thereby minimising the risk to surrounding structures and optimising outcomes. We aim to create a checklist t...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-017-0787-9
更新日期:2017-08-01 00:00:00
abstract:CONTEXT:Somatostatin (SST) and dopamine (DA) inhibit growth hormone (GH) secretion and proliferation of GH-secreting pituitary adenomas (GHomas) through binding to SSTR2 and D2R receptors. Chimeric SST-DA compounds (Dopastatins) display increased potency in inhibiting GH secretion, as compared with individual SST or DA...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-020-01113-4
更新日期:2021-01-12 00:00:00
abstract:PURPOSE:Granular cell tumors of the neurohypophysis are rare, solitary lesions, mostly presenting in the adult age. They rarely grow to a sufficient size to cause mass effect related symptoms and they may be found in most cases incidentally at autopsy. Because of their rarity as of now they have been described only as ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-016-0704-7
更新日期:2016-06-01 00:00:00
abstract:PURPOSE:Tatton-Brown-Rahman syndrome (TBRS) is a newly defined genetic entity characterized by overgrowth and intellectual disability, resulting from germline mutations in the gene encoding DNA methyltransferase 3 alpha (DNMT3A). Affected individuals with benign and malignant tumors have been reported; to our knowledge...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-019-01019-w
更新日期:2020-04-01 00:00:00
abstract::Acute lymphoblastic leukaemia (ALL) is the most common childhood malignancy. The survival rate in the Scandinavian countries is now around 85 %. ALL patients treated with cranial radiotherapy (CRT) are at risk for growth hormone deficiency (GHD), but little is known about other pituitary insufficiencies, e.g. ACTH. Ad...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0459-8
更新日期:2014-02-01 00:00:00
abstract:PURPOSE:DAX-1 (NR0B1) is an orphan nuclear receptor, which plays a critical role in development and regulation of the adrenal gland and hypothalamo-pituitary-gonadal axis. Mutations in NR0B1 lead to adrenal hypoplasia congenita (AHC), hypogonadotropic hypogonadism (HH) and azoospermia in men. Presentation is typically ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-017-0822-x
更新日期:2017-10-01 00:00:00
abstract:PURPOSE:Approximately 25 % of cases of clinically active acromegaly cases treated in our academic center between 1996 and 2000, were diagnosed in patients who had elevated plasma IGF-1 levels, but apparently "normal" 24-h mean plasma GH levels. The current study served to update the data for patients with acromegaly re...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-016-0735-0
更新日期:2016-12-01 00:00:00
abstract::A young female patient presented as an acute medical emergency with hypoglycaemia. Investigations revealed panhypopituitarism and an inflammatory pituitary mass. An antibody screen was negative for anti-neutrophil cytoplasmic antibodies with cytoplasmic distribution (cANCA). Pituitary histology showed lymphocytic infi...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-007-0016-z
更新日期:2007-01-01 00:00:00
abstract:OBJECTIVE:The purpose of this study was to define safety and efficacy of medical therapy in the treatment of nonfunctioning pituitary tumours. DESIGN:We studied thirteen patients with a clinically nonfunctioning pituitary macroadenoma for response to cabergoline treatment for 1 year. Twelve/13 patients were already op...
journal_title:Pituitary
pub_type: 临床试验,杂志文章
doi:10.1023/a:1015366923810
更新日期:2001-08-01 00:00:00
abstract::The dexamethasone (DXM) test has been widely used for diagnosing Cushing's disease (CD). The purpose of this paper is to review its diagnostic merit based on calculation of data extracted from earlier publications. Studies presenting individual values for patients with CD and normal subjects were identified through Pu...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-013-0509-x
更新日期:2014-08-01 00:00:00
abstract:PURPOSE:The pituitary gland is an unusual site for metastatic spread, but as patients with metastatic malignancy are living longer, it may become more prevalent. Compression of important anatomy adjacent to the sella may produce disabling symptoms and endocrine derangement, leading to significant morbidity. METHODS:An...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-018-0899-x
更新日期:2018-10-01 00:00:00
abstract::Non-functioning pituitary neuroendocrine tumors do not cause endocrine symptoms related to hypersecretion of adenohypophyseal hormones and are clinically characterized by symptoms due to growing sellar tumor mass. Histopathological classification of this tumor group has always been challenging due to their heterogenei...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-017-0855-1
更新日期:2018-04-01 00:00:00
abstract:PURPOSE:In a 10-week proof-of-concept study (LINC 1), the potent oral 11β-hydroxylase inhibitor osilodrostat (LCI699) normalized urinary free cortisol (UFC) in 11/12 patients with Cushing's disease. The current 22-week study (LINC 2; NCT01331239) further evaluated osilodrostat in patients with Cushing's disease. METHO...
journal_title:Pituitary
pub_type: 杂志文章,多中心研究
doi:10.1007/s11102-015-0692-z
更新日期:2016-04-01 00:00:00
abstract::A 54-years-old woman diagnosed of myotonic dystrophy (MyD) with past medical history of massive postpartum haemorrhage at age 28 and panhypopituitarism was studied. BMI and body composition were determined and we determined baseline serum IGF-I, IGFBP3, insulin and leptin levels and after the IGF-I generation test per...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1023/a:1009984505312
更新日期:1999-01-01 00:00:00
abstract::The morphology of the various pituitary cell types is highly dynamic and allows recognition of many cellular functions. Most pituitary cells show morphologic changes that reflect stimulation or inhibition by hormones. Drugs have also been shown to alter the morphology of several pituitary tumor types, allowing a measu...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1023/a:1022320732718
更新日期:2002-01-01 00:00:00
abstract:PURPOSE:Doses of growth hormone in adults with growth hormone deficiency are now lower than previously. However, it is not clear they are as effective as higher doses. The objective of this meta-analysis was to assess efficacy of low to moderate dose (LD) GH replacement on standard endpoints of GH compared to higher do...
journal_title:Pituitary
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s11102-014-0571-z
更新日期:2015-06-01 00:00:00
abstract::Anemia and other hematological abnormalities are common in patients with Sheehan's syndrome. The response of these abnormalities to replacement of thyroxine and glucocorticoids is not clear. The aim of the present study was to document the profile of hematological abnormalities and response to treatment in patients wi...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-010-0255-2
更新日期:2011-03-01 00:00:00
abstract:INTRODUCTION:Cushing's syndrome is associated with a number of clinical manifestations and co-morbidities which may not resolve even after long-term remission leading to excessive mortality. MATERIALS AND METHODS:This review summarizes the main manifestations of Cushing's syndrome (active or in remission) with particu...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-014-0631-4
更新日期:2015-04-01 00:00:00
abstract::The purpose of this study is to verify whether acute pre-treatment with alprazolam (ALP), a benzodiazepine that inhibits HPA secretion in normal subjects, could better characterize patients with subclinical Cushing's syndrome (SCS) than the 1-mg dexamethasone test (DST). In 22 patients with SCS, 10 with overt Cushing'...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0433-5
更新日期:2013-09-01 00:00:00
abstract::Double pituitary adenomas are rare in surgical specimens and the most common clinical feature in reported patients has been acromegaly. We report 3 cases of double pituitary lesions in patients who presented with Cushing's disease. In a 22-year-old man (case 1) with delayed puberty and low testosterone levels, mild hy...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1023/a:1011499609096
更新日期:2000-11-01 00:00:00
abstract:PURPOSE:To evaluate the effects of somatostatin analogs and disease activity status on the upper gastrointestinal system in patients with acromegaly. METHODS:One hundred eighty-one patients with acromegaly were retrospectively assessed. The demographic, biochemical, pathologic, and radiologic data of the patients were...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-020-01095-3
更新日期:2020-10-19 00:00:00
abstract::Primary autoimmune hypophysitis (PAH) is considered an underdiagnosed disease, due to the difficulty in reaching a definitive diagnosis. PAH clinical diagnosis remains an exclusion diagnosis. We aimed to correlate PAH neuro-radiological signs to endocrine features and evaluate their prognostic role. 24 PAH cases were ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-018-0892-4
更新日期:2018-08-01 00:00:00
abstract:PURPOSE:Secondary empty sella syndrome (SESS) following pituitary surgery remains a diagnostic and therapeutic challenge. The aim of this study was to specify the diagnostic criteria, surgical indications and results of chiasmapexy in the SESS. METHODS:Three cases from two experienced neurosurgical centers were collec...
journal_title:Pituitary
pub_type: 杂志文章,评审
doi:10.1007/s11102-020-01104-5
更新日期:2020-11-02 00:00:00
abstract::Acromegaly is associated with serious morbidity and mortality, if not well controlled. Approved somatostatin receptor ligands (SRLs) are a mainstay of medical therapy and exhibit preferential affinity for somatostatin receptor (SSTR) subtype 2. Our objective was to assess whether characteristic features of individual ...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-012-0445-1
更新日期:2013-12-01 00:00:00
abstract:PURPOSE:Patients treated for pituitary adenomas generally report a reduced quality of life (QoL). At present, the patient's perspective of QoL has not been fully addressed and this, and further insight in potential determinants of QoL in pituitary diseases is required to design strategies to improve QoL. We aimed to de...
journal_title:Pituitary
pub_type: 杂志文章
doi:10.1007/s11102-014-0561-1
更新日期:2015-02-01 00:00:00