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Cushing's syndrome

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13 papers 25 to 100 followers
By Ehud Ur Professor and Head, Endocrinology, UBC, Vancouver
André Lacroix, Feng Gu, Wilson Gallardo, Rosario Pivonello, Yerong Yu, Przemysław Witek, Marco Boscaro, Roberto Salvatori, Masanobu Yamada, Libuse Tauchmanova, Michael Roughton, Shoba Ravichandran, Stephan Petersenn, Beverly M K Biller, John Newell-Price
BACKGROUND: Cushing's disease is a rare debilitating endocrine disorder for which few prospective interventional studies have been done. We report results of the first phase 3 trial assessing long-acting intramuscular pasireotide in patients with Cushing's disease. METHODS: In this phase 3 clinical trial we recruited patients aged 18 years or older with persistent, recurrent, or de-novo (non-surgical candidates) Cushing's disease who had a mean urinary free cortisol (mUFC) concentration (from three 24 h samples) of 1·5-5·0 times the upper limit of normal (ULN), a normal or greater than normal morning plasma adrenocorticotropic hormone concentration, and a pituitary source of Cushing's syndrome, from 57 sites across 19 countries...
January 2018: Lancet Diabetes & Endocrinology
Aaron Hodes, Maya B Lodish, Amit Tirosh, Jerrold Meyer, Elena Belyavskaya, Charalampos Lyssikatos, Kendra Rosenberg, Andrew Demidowich, Jeremy Swan, Nichole Jonas, Constantine A Stratakis, Mihail Zilbermint
PURPOSE: Hair cortisol evaluation has been used to help detect patients with suspected Cushing syndrome. Our goal was to correlate segmental hair cortisol with biochemical testing in patients with Cushing syndrome and controls. This study was a prospective analysis of hair cortisol in confirmed Cushing syndrome cases over 16 months. METHODS: Thirty-six subjects (26.5 ± 18.9 years, 75% female, and 75% Caucasian) were analyzed by diurnal serum cortisol, 24 h urinary free cortisol corrected for body surface area (UFC/BSA), and 24 h urinary 17-hydroxysteroids corrected for creatinine (17OHS/Cr)...
April 2017: Endocrine
Irina Bancos, Fares Alahdab, Rachel K Crowley, Vasileios Chortis, Danae A Delivanis, Dana Erickson, Neena Natt, Massimo Terzolo, Wiebke Arlt, William F Young, M Hassan Murad
OBJECTIVE: Beneficial effects of adrenalectomy on cardiovascular risk factors in patients with subclinical Cushing's syndrome (SCS) are uncertain. We sought to conduct a systematic review and meta-analysis with the following objectives: (i) determine the effect of adrenalectomy compared with conservative management on cardiovascular risk factors in patients with SCS and (ii) compare the effect of adrenalectomy on cardiovascular risk factors in patients with SCS vs those with a nonfunctioning (NF) adrenal tumor...
December 2016: European Journal of Endocrinology
Maria Fleseriu, Amir H Hamrahian, Andrew R Hoffman, Daniel F Kelly, Laurence Katznelson
Recurrence of hypercortisolemia after initial treatment of Cushing disease (CD) is more common than previously thought, with a third of patients suffering a recurrence over their lifetime. Awareness of this high rate and delayed timeline (sometimes decades) of potential recurrence is critical and patients with CD should be monitored at regular intervals throughout their lives. In this manuscript, we review the complex evaluation needed for defining CD remission versus persistent disease after surgery, and focus on challenges in diagnosing early recurrent hypercortisolemia...
December 2016: Endocrine Practice
D A Vassiliadi, M Balomenaki, A Asimakopoulou, E Botoula, M Tzanela, S Tsagarakis
CONTEXT: Cushing's disease (CD) has a significant relapse rate after successful transsphenoidal surgery (TSS). Many CD patients respond aberrantly to the desmopressin test (DT). Disappearance of this response after surgery may suggest complete removal of abnormal corticotrophs and a lower possibility of recurrence. OBJECTIVE: The utility of postoperative DT to predict long-term outcome compared to the widely used postoperative cortisol level. DESIGN: Retrospective analysis...
December 2016: Journal of Clinical Endocrinology and Metabolism
Johannes A Romijn
Ultimately, almost all patients who are appropriately treated for pituitary tumours enter a chronic phase with control or cure of hormonal excess, adequate treatment of pituitary insufficiency and relief of mass effects. This phase is associated with improvement of initial signs and symptoms, but also with the persistent consequences of the initial disease and associated treatments. Pituitary insufficiency is a common denominator in many of these patients, and is associated with a reduction in quality of life, despite adequate endocrine substitution...
September 2016: Nature Reviews. Endocrinology
Delphine Vezzosi, Florence Tenenbaum, Laure Cazabat, Frédérique Tissier, Marie Bienvenu, Carmen A Carrasco, Marie Laloi-Michelin, Gaëlle Barrande, Hervé Lefebvre, Sylvie Hiéronimus, Antoine Tabarin, Xavier Bertagna, Paul Legmann, Marie-Christine Vantyghem, Jérôme Bertherat
CONTEXT: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent Cushing's syndrome that may occur in an isolated form or as part of Carney complex. The diagnosis of this disease can be difficult preoperatively because computed tomography (CT) scan can be normal or suggest unilateral adrenal lesion, which can impede the correct diagnosis of bilateral adrenal disease. OBJECTIVE: The aim of our study was to describe the results of preoperative imaging (adrenal [6β-(131)I]iodomethyl-19-norcholesterol] [NP-59] scintigraphy and standard adrenal CT scan) and their correlations with clinical, pathological, and genetics investigations in patients with PPNAD...
November 2015: Journal of Clinical Endocrinology and Metabolism
Andrea M Isidori, Emilia Sbardella, Maria Chiara Zatelli, Mara Boschetti, Giovanni Vitale, Annamaria Colao, Rosario Pivonello
CONTEXT: Ectopic Cushing's Syndrome (ECS) can be a diagnostic challenge with the hormonal source difficult to find. This study analyzes the accuracy of imaging studies in ECS localization. EVIDENCE ACQUISITION: Systematic review of medical literature for ECS case series providing individual patient data on at least one conventional imaging technique (computed tomography [CT]/magnetic resonance imaging) and one of the following: 111In-pentetreotide (OCT), 131I/123I-metaiodobenzylguanidine, 18Ffluoro-2-deoxyglucose-positron emission tomography (FDG-PET), 18F-fluorodopa-PET (F-DOPA-PET), 68Ga- DOTATATE-PET/CT or 68Ga-DOTATOC-PET/CT scan (68Gallium-SSTR-PET/CT)...
September 2015: Journal of Clinical Endocrinology and Metabolism
Rosario Pivonello, Monica De Leo, Alessia Cozzolino, Annamaria Colao
Cushing's disease (CD), or pituitary-dependent Cushing's syndrome, is a severe endocrine disease caused by a corticotroph pituitary tumor and associated with increased morbidity and mortality. The first-line treatment for CD is pituitary surgery, which is followed by disease remission in around 78% and relapse in around 13% of patients during the 10-year period after surgery, so that nearly one third of patients experience in the long-term a failure of surgery and require an additional second-line treatment...
August 2015: Endocrine Reviews
John Newell-Price
No abstract text is available yet for this article.
May 2014: Journal of Clinical Endocrinology and Metabolism
Qinghua Guo, William F Young, Dana Erickson, Bradley Erickson
PURPOSE: The distinction between corticotropin (ACTH)-producing pituitary adenomas and occult ectopic ACTH neoplasms is a challenge and frequently complicated by the detection of nonfunctioning pituitary adenomas on dynamic magnetic resonance imaging (DMRI). Herein, we explored quantitative differences in DMRI measures from ACTH-producing pituitary adenomas. MATERIALS AND METHODS: Fifty-five patients with pathology confirmed ACTH-producing and 44 with nonfunctioning pituitary adenomas were analysed in this retrospective pilot study...
February 2015: Clinical Endocrinology
Agostino De Venanzi, Guilherme Asmar Alencar, Isabelle Bourdeau, Maria Candida Barisson Villares Fragoso, André Lacroix
PURPOSE OF REVIEW: Primary bilateral macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome and is more often diagnosed as bilateral adrenal incidentalomas with subclinical cortisol production. We summarize the recent insights concerning its epidemiology, diagnosis, genetics, pathophysiology, and therapeutic options. RECENT FINDINGS: Recent publications have modified our notions on the genetics and pathophysiology of bilateral macronodular adrenal hyperplasia...
June 2014: Current Opinion in Endocrinology, Diabetes, and Obesity
Eugenia Resmini, Alicia Santos, Beatriz Gómez-Anson, Olga López-Mourelo, Patricia Pires, Yolanda Vives-Gilabert, Iris Crespo, Maria J Portella, Manel de Juan-Delago, Susan M Webb
BACKGROUND: Proton magnetic resonance spectroscopy ((1) H-MRS) is a sensitive, noninvasive imaging technique capable of measuring brain metabolites in vivo. Chronic exposure to endogenous hypercortisolism in Cushing's syndrome (CS) is associated with negative effects on memory and hippocampal volumes, even after biochemical cure. OBJECTIVE: To investigate metabolites in the hippocampi of CS patients and controls, using (1) H-MRS. PATIENTS AND METHODS: Eighteen right-handed cured CS patients (age 44·8 ± 12·5 years, 12·6 ± 3·8 years of education) and 18 right-handed healthy controls, matched for age (40·0 ± 11·9) and years of education (14·4 ± 3·8), underwent 3-Tesla magnetic resonance imaging (3T MRI) and (1) H-MRS including the head of each hippocampus...
November 2013: Clinical Endocrinology
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