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Cushing Response

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81. Cushing syndrome due to ACTH-secreting pheochromocytoma aggravated by a glucocorticoid-driven positive-feedback loop. (PubMed)

Cushing syndrome due to ACTH-secreting pheochromocytoma aggravated by a glucocorticoid-driven positive-feedback loop. Pheochromocytoma is a catecholamine-producing tumor that originates from adrenal chromaffin cells and is capable of secreting various hormones, including ACTH.A 56-year-old female presented with Cushingoid appearance and diabetic ketoacidosis. Endocrinological examinations demonstrated ectopic ACTH production with hypercortisolemia and excess urinary cortisol accompanied (...) by elevated plasma and urine catecholamines. Computed tomography revealed a large left adrenal tumor with bilateral adrenal enlargement. Metaiodobenzylguanidine scintigraphy revealed abnormal accumulation in the tumor, which was eventually diagnosed as pheochromocytoma with ectopic ACTH secretion with subsequent manifestation of Cushing's syndrome. Ectopic ACTH secretion and catecholamine production were blocked by metyrapone treatment, whereas dexamethasone paradoxically increased ACTH secretion. Left

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2015 Journal of Clinical Endocrinology and Metabolism

82. Dilated Cardiomyopathy Revealing Cushing Disease: A Case Report and Literature Review. (PubMed)

Dilated Cardiomyopathy Revealing Cushing Disease: A Case Report and Literature Review. Cardiovascular impairments are frequent in Cushing's syndrome and the hypercortisolism can result in cardiac structural and functional changes that lead in rare cases to dilated cardiomyopathy (DCM). Such cardiac impairment may be reversible in response to a eucortisolaemic state.A 43-year-old man with a medical past of hypertension and history of smoking presented to the emergency department with global (...) heart failure. Coronary angiography showed a significant stenosis of a marginal branch and cardiac MRI revealed a nonischemic DCM. The left ventricular ejection fraction (LVEF) was estimated as 28% to 30%. Clinicobiological features and pituitary imaging pointed toward Cushing's disease and administration of adrenolytic drugs (metyrapone and ketoconazole) was initiated. Despite the normalization of cortisol which had been achieved 2 months later, the patient presented an acute heart failure

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2015 Medicine

83. The USP8 mutational status may predict drug susceptibility in corticotroph adenomas of Cushing's disease. (PubMed)

The USP8 mutational status may predict drug susceptibility in corticotroph adenomas of Cushing's disease. Somatic mutations in the ubiquitin-specific peptidase USP8 gene were recently detected in one- to two-third(s) of corticotroph adenomas of Cushing's disease (CD). These mutations may lead to the deubiquitination of EGFR, thereby increasing EGFR signaling, which has been implicated in ACTH hypersecretion.Our objective was to determine the impact of USP8 mutations on the clinicopathological (...) mutations may predict favorable responses to the somatostatin analog pasireotide, which exhibits high affinity for SSTR5. In contrast, non-mutated aggressive tumors such as CCA may respond better to the alkylating agent temozolomide because of their significantly weak expression of MGMT.© 2016 European Society of Endocrinology.

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2015 European Journal of Endocrinology

84. Targeted Therapy With Gefitinib in Patients With USP8-mutated Cushing's Disease

. Targeted Therapy With Gefitinib in Patients With USP8-mutated Cushing's Disease The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02484755 Recruitment Status : Unknown Verified June 2015 by Zhaoyun Zhang, Huashan Hospital. Recruitment status was: Recruiting First Posted : June 30, 2015 Last Update Posted (...) : June 30, 2015 Sponsor: Huashan Hospital Information provided by (Responsible Party): Zhaoyun Zhang, Huashan Hospital Study Details Study Description Go to Brief Summary: The USP8 gene and its downstream target, epidermal growth factor receptor (EGFR), is a potential therapeutic target of Cushing disease. The EGFR inhibitor, Gefitinib, has been shown to reduce the production of ACTH both in vitro and in vivo, especially in USP8-mutated corticotrophin adenomas. The investigators hypothesize

2015 Clinical Trials

85. Study of Efficacy and Safety of Osilodrostat in Cushing's Syndrome

and Safety of Osilodrostat in Cushing's Syndrome The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02468193 Recruitment Status : Completed First Posted : June 10, 2015 Last Update Posted : December 10, 2018 Sponsor: Novartis Pharmaceuticals Information provided by (Responsible Party): Novartis ( Novartis (...) Study of Efficacy and Safety of Osilodrostat in Cushing's Syndrome Study of Efficacy and Safety of Osilodrostat in Cushing's Syndrome - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Study of Efficacy

2015 Clinical Trials

86. FGFR4 Polymorphic Variants Modulate Phenotypic Features of Cushing Disease. (PubMed)

FGFR4 Polymorphic Variants Modulate Phenotypic Features of Cushing Disease. Cushing disease is a potentially lethal condition resulting from hormone excess, usually due to a small pituitary tumor that fails to respond to negative feedback inhibition. A minority of patients develop larger, more aggressive tumors of the same lineage but with modest hormone excess. Here we show that a common polymorphism in the fibroblast growth factor receptor 4 (FGFR4) transmembrane domain yields receptor (...) isoforms with distinct properties that mediate these biological differences. Forced expression of the major FGFR4-G388 variant allele supports pY-signal transducer and activator of transcription (STAT3) responses. In contrast, expression of the minor FGFR4-R388 allele enhances STAT3 serine phosphorylation, driving cellular growth. In addition, FGFR4-R388 enhances glucocorticoid receptor phosphorylation and nuclear translocation. Consistent with these findings, glucocorticoid administration resulted

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2014 Molecular Endocrinology

87. Efficacy of medical treatment in Cushing's disease: a systematic review. (PubMed)

Efficacy of medical treatment in Cushing's disease: a systematic review. Reported rates of response to medical therapies used in Cushing's disease (CD) vary widely. The aim of this review is to analyse systematically the efficacy of medical therapies for CD and to assess the strength of the supporting evidence.Systematic PubMed searches identified studies of medical treatment in CD. The GRADE criteria were imposed to assess the strength of evidence supporting each medication.Fifteen studies (...) ). Response rates were 25-50% for cabergoline (four studies) and 45% for ketoconazole (one study). Among studies that included patients with other forms of Cushing's syndrome, response rates were 53-88% for ketoconazole (three studies), 70% for mitotane (one study), 57% for metyrapone (one study) and 38-60% for mifepristone. Again, all of these medications are supported by a 'low' level of evidence.There is a paucity of high-quality studies of medical therapy in CD, with only one medication achieving

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2014 Clinical endocrinology

88. Cushing's syndrome due to a bronchial ACTH-secreting carcinoid successfully treated with radio frequency ablation (RFA). (PubMed)

Cushing's syndrome due to a bronchial ACTH-secreting carcinoid successfully treated with radio frequency ablation (RFA). The ectopic production of ACTH is responsible for approximately 10% of cases of Cushing's syndrome. Whenever possible, once hypercortisolemia is under control with medical therapy, the final treatment consists of surgical excision of the tumor. We report a case of a patient with high surgical risk and poor response to medical therapy in which hypercortisolemia has been (...) successfully treated with radiofrequency ablation of the bronchial carcinoid tumor.A 43-year-old woman came to our hospital because of severe and rapidly worsening signs and symptoms of hypercortisolism over the previous 3 months. Hormonal tests suggested the presence of Cushing's syndrome due to ectopic ACTH production. Imaging studies detected an 8-mm pulmonary nodule with fluorine-18-fluorodeoxyglucose uptake localized in the middle right lobe. The patient started therapy with ketoconazole with poor

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2014 Journal of Clinical Endocrinology and Metabolism

89. High-resolution<sup>18</sup>F-fluorodeoxyglucose positron emission tomography and magnetic resonance imaging for pituitary adenoma detection in Cushing disease. (PubMed)

High-resolution18F-fluorodeoxyglucose positron emission tomography and magnetic resonance imaging for pituitary adenoma detection in Cushing disease. OBJECT High-resolution PET (hrPET) performed using a high-resolution research tomograph is reported as having a resolution of 2 mm and could be used to detect corticotroph adenomas through uptake of(18)F-fluorodeoxyglucose ((18)F-FDG). To determine the sensitivity of this imaging modality, the authors compared(18)F-FDG hrPET and MRI (...) detection of pituitary adenomas in Cushing disease (CD). METHODS Consecutive patients with CD who underwent preoperative(18)F-FDG hrPET and MRI (spin echo [SE] and spoiled gradient recalled [SPGR] sequences) were prospectively analyzed. Standardized uptake values (SUVs) were calculated from hrPET and were compared with MRI findings. Imaging findings were correlated to operative and histological findings. RESULTS Ten patients (7 females and 3 males) were included (mean age 30.8 ± 19.3 years; range 11-59

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2014 Journal of Neurosurgery

90. ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome: Clinicopathologic Study of 11 Cases and Review of the Literature. (PubMed)

ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome: Clinicopathologic Study of 11 Cases and Review of the Literature. Adrenocorticotropic hormone (ACTH)-secreting pancreatic neuroendocrine tumors (PanNETs), although rare, are responsible for about 15% of ectopic Cushing syndrome (CS). They represent a challenging entity because their preoperatory diagnosis is frequently difficult, and clear-cut morphologic criteria useful to differentiate them from other types of PanNETs have

2014 American Journal of Surgical Pathology

91. Case report: nocardia infection associated with ectopic cushings. (PubMed)

Case report: nocardia infection associated with ectopic cushings. Cushing's syndrome results from exposure to excess glucocorticoids. Ectopic Cushings is endogenous ACTH dependant form of Cushing's associated with markedly raised ACTH and cortisol levels. This leads to an impaired immune response, setting the stage for occurrence of opportunistic infections. Nocardiosis is a gram positive bacterial infection caused by aerobic actinomycetes in genus Nocardia. We report a series of patients (...) diagnosed with ectopic Cushings, having pneumonia with Nocardia spp. In one of these cases, the manifestations of Cushing's disappeared with treatment for Nocardia.Two middle aged men of Asian descent presented to the Endocrine clinic: the first with history of exertional shortness of breath, and weight loss for 1 year, the other with facial swelling, disturbed sleep and lethargy for a month. The third case was a young Asian male who presented with progressive weakness & weight loss for 2 months. All

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2014 BMC Endocrine Disorders

92. Effects of Metyrapone in Patients With Endogenous Cushing's Syndrome

of Metyrapone in Patients With Endogenous Cushing's Syndrome (PROMPT) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02297945 Recruitment Status : Unknown Verified February 2017 by HRA Pharma. Recruitment status was: Recruiting First Posted : November 21, 2014 Last Update Posted : February 15, 2017 Sponsor (...) : HRA Pharma Information provided by (Responsible Party): HRA Pharma Study Details Study Description Go to Brief Summary: The purpose of this prospective, international phase III/IV study is to assess the efficacy and safety of metyrapone in patients with endogenous Cushing's syndrome during up to 36 weeks of treatment. The ability of metyrapone (250 mg capsules) to normalize urinary free cortisol (UFC) levels will be assessed during up to 36 weeks (9 months) of treatment. Condition or disease

2014 Clinical Trials

93. Long Term Safety and Efficacy of Pasireotide s.c. in Patients With Cushing's Disease

adding more. Long Term Safety and Efficacy of Pasireotide s.c. in Patients With Cushing's Disease The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier: NCT02310269 Recruitment Status : Recruiting First Posted : December 8, 2014 (...) Last Update Posted : August 20, 2018 See Sponsor: Novartis Pharmaceuticals Information provided by (Responsible Party): Novartis ( Novartis Pharmaceuticals ) Study Details Study Description Go to Brief Summary: This is a non-interventional, multinational, multi-center post-marketing study, to further document the safety and efficacy of pasireotide s.c. administered in routine clinical practice in patients with Cushing's disease. Patients with Cushing's disease and treated with pasireotide s.c

2014 Clinical Trials

94. The Factors Associated With the Recurrence in Patients With Cushing Disease

. The Factors Associated With the Recurrence in Patients With Cushing Disease The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02233335 Recruitment Status : Completed First Posted : September 8, 2014 Last Update Posted : September 8, 2014 Sponsor: National Taiwan University Hospital Information provided (...) by (Responsible Party): National Taiwan University Hospital Study Details Study Description Go to Brief Summary: Cushing syndrome refers the manifestations induced by chronic glucocorticoid excess and may arise from various causes. Iatrogenic Cushing syndrome accounts for most patients, when they are given exogenous glucocorticoid treatment. In contrast, spontaneous Cushing syndrome results from endogenous glucocorticoid over-secretion. Although Cushing disease is rare, it is the most common cause

2014 Clinical Trials

95. Cardiac Steatosis in Cushing's Syndrome

by (Responsible Party): Assistance Publique - Hôpitaux de Paris Study Details Study Description Go to Brief Summary: This study aims at evaluating the myocardial triglyceride content and cardiac structure and function, using 1H magnetic resonance spectroscopy and cardiac magnetic resonance imaging, in patients with Cushing's syndrome before and after treatment and in age-, sex- and BMI-matched healthy volunteers. The investigators make the hypothesis that Cushing's syndrome patients compared to healthy (...) Cardiac Steatosis in Cushing's Syndrome Cardiac Steatosis in Cushing's Syndrome - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Cardiac Steatosis in Cushing's Syndrome (CORTICOEUR) The safety and scientific

2014 Clinical Trials

96. Study of Management of Pasireotide-induced Hyperglycemia in Adult Patients With Cushing's Disease or Acromegaly

studies (100). Please remove one or more studies before adding more. Study of Management of Pasireotide-induced Hyperglycemia in Adult Patients With Cushing's Disease or Acromegaly The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02060383 Recruitment Status : Completed First Posted : February 12, 2014 (...) Last Update Posted : January 8, 2019 Sponsor: Novartis Pharmaceuticals Information provided by (Responsible Party): Novartis ( Novartis Pharmaceuticals ) Study Details Study Description Go to Brief Summary: The study aims to demonstrate that pasireotide-induced hyperglycemia can be effectively and safely managed in majority of patients, including those with diabetes at start of pasireotide treatment. Condition or disease Intervention/treatment Phase Cushing's Disease Acromegaly Drug: Pasireotide

2014 Clinical Trials

97. Safety and Efficacy of LCI699 for the Treatment of Patients With Cushing's Disease

adding more. Safety and Efficacy of LCI699 for the Treatment of Patients With Cushing's Disease The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT02180217 Recruitment Status : Active, not recruiting First Posted : July 2, 2014 Last Update Posted : March 28, 2019 Sponsor: Novartis Pharmaceuticals (...) Information provided by (Responsible Party): Novartis ( Novartis Pharmaceuticals ) Study Details Study Description Go to Brief Summary: The study aims to confirm long-term efficacy and safety of LCI699 for the treatment of patients with Cushing's disease. It is a pivotal trial intended to support the registration of LCI699 for the treatment of patients with Cushing's disease in the EU, Japan, and other countries. This is a phase lll, multi-center, double-blind, randomized withdrawal study of LCI699

2014 Clinical Trials

98. Treatment of Cushing's Disease With R-roscovitine

provided by (Responsible Party): Shlomo Melmed, MD, Cedars-Sinai Medical Center Study Details Study Description Go to Brief Summary: The investigators hypothesize that R-roscovitine will suppress pituitary corticotroph tumor ACTH production and normalize urinary free cortisol levels in patients with Cushing disease. To date, R-roscovitine has been evaluated in several Phase I and II studies and has shown early signs of anti-cancer activity in approximately 240 patients. Condition or disease (...) Proceedings, Abs 2060. 2005. Yeo et al. J Clin Oncol 2009 27‐15s (Suppl abstr 6026). Layout table for additonal information Responsible Party: Shlomo Melmed, MD, Sr. Vice President of Academic Affairs, Cedars-Sinai Medical Center ClinicalTrials.gov Identifier: Other Study ID Numbers: Pro35720 First Posted: June 11, 2014 Last Update Posted: November 7, 2018 Last Verified: November 2018 Keywords provided by Shlomo Melmed, MD, Cedars-Sinai Medical Center: Cushings disease R-roscovitine Additional relevant

2014 Clinical Trials

99. Adrenal Venous Sampling in Patients With Overt or Subclinical Cushings Syndrome, and Bilateral Adrenal Tumors

(100). Please remove one or more studies before adding more. Adrenal Venous Sampling in Patients With Overt or Subclinical Cushings Syndrome, and Bilateral Adrenal Tumors (AVS) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier (...) : NCT02543697 Recruitment Status : Recruiting First Posted : September 7, 2015 Last Update Posted : September 8, 2016 See Sponsor: Haukeland University Hospital Information provided by (Responsible Party): Haukeland University Hospital Study Details Study Description Go to Brief Summary: Patients with tumors in both adrenal glands and slightly elevated cortisol (subclinical Cushings syndrome) are offered to go through an adrenal venous sampling to try to quantify if the overproduction of cortisol is from

2015 Clinical Trials

100. Long-Term Outcome of Bilateral Laparoscopic Adrenalectomy Measured by Disease-Specific Questionnaire in a Unique Group of Patients with Cushing's Syndrome. (PubMed)

used a disease-specific questionnaire covering all major clinicopathologic characteristics of CS. We compared the outcome from LBA to a control group of 60 patients who had thyroidectomy (matched for age, gender, and time of surgery, 2:1 control-to-CS).Twenty-eight patients (20 women and 8 men) underwent LBA for CS. Of them, 24 patients (86 %) provided responses to our questionnaire. Ninety-two percent of patients' responses indicated a significant improvement of general Cushing's physical features (...) Long-Term Outcome of Bilateral Laparoscopic Adrenalectomy Measured by Disease-Specific Questionnaire in a Unique Group of Patients with Cushing's Syndrome. Laparoscopic bilateral adrenalectomy (LBA) is recommended for patients with bilateral adrenal disease and occult or unresectable ectopic Cushing's syndrome (CS). There are limited data on long-term outcomes after LBA, partly due to the lack of disease-specific tools for the measurement of impact on patients' health and quality of life.We

2015 Annals of Surgical Oncology

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