Cushing Syndrome

A condition caused by prolonged exposure to excess levels of cortisol (HYDROCORTISONE) or other GLUCOCORTICOIDS from endogenous or exogenous sources. It is characterized by upper body OBESITY; OSTEOPOROSIS; HYPERTENSION; DIABETES MELLITUS; HIRSUTISM; AMENORRHEA; and excess body fluid. Endogenous Cushing syndrome or spontaneous hypercortisolism is divided into two groups, those due to an excess of ADRENOCORTICOTROPIN and those that are ACTH-independent.

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Networked: 4565 relevant articles (264 outcomes, 298 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cushing Syndrome
2.	Neoplasms (Cancer)
3.	Adenoma (Adenomas)
4.	Hypertension (High Blood Pressure)
5.	Pheochromocytoma

Experts

1.	Stratakis, Constantine A: 49 articles (01/2016 - 08/2003)
2.	Bertherat, Jérôme: 29 articles (11/2015 - 05/2002)
3.	Bertagna, Xavier: 26 articles (11/2015 - 05/2002)
4.	Nieman, Lynnette K: 25 articles (10/2015 - 07/2002)
5.	Boscaro, Marco: 24 articles (10/2015 - 01/2002)
6.	Lacroix, André: 22 articles (10/2015 - 01/2002)
7.	Sasano, Hironobu: 21 articles (10/2015 - 07/2002)
8.	Bourdeau, Isabelle: 21 articles (10/2015 - 08/2003)
9.	Grossman, Ashley B: 21 articles (10/2013 - 11/2003)
10.	Groussin, Lionel: 19 articles (11/2013 - 05/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cushing Syndrome:

1.	Adrenocorticotropic Hormone (ACTH)FDA Link 11/01/2004 - "After a complete clinical remission obtained by a transsphenoidal surgery, at the age of 18, the patient had a recurrence of ACTH-dependent hypercortisolism, and a second transsphenoidal surgery was performed. " 09/01/2009 - "Bilateral inferior petrosal sinus sampling is considered the gold standard for establishing the origin of ACTH secretion, and it is recommended in patients with ACTH-dependent Cushing's syndrome whose clinical, biochemical, or radiological studies are discordant or equivocal. " 05/01/1979 - "Plasma adrenocorticotropin, although not helpful in establishing the diagnosis, has proved valuable in differentiating the three major entities that cause hypercortisolism. " 05/01/2007 - "These agents have been effective in a limited number of patients with ACTH-dependent Cushing's syndrome. " 03/01/2012 - "Case 4, with a history of cyclic hypercortisolism, had normal to slightly elevated ACTH levels and low-normal UFC during remission. "
2.	Hydrocortisone (Cortisol)FDA LinkGeneric 05/01/2009 - "Sequential salivary cortisol measurements are also extremely helpful for the investigation of suspected cyclical Cushing's syndrome. " 01/01/2013 - "Because hypercortisolism symptoms are wide ranging, it is important to assess a variety of outcomes including both clinical factors, such as cortisol levels, and health-related quality of life (HR-QOL), to better understand the severity and impact of CD on patients and the potential efficacy of CD treatment. " 08/01/2014 - "A high prevalence of psychological morbidity and maladaptive personality as well as impaired quality of life (QoL) is observed in patients with and without hydrocortisone dependency following (cured) Cushing's syndrome. " 07/01/2013 - "Conversely, remission from hypercortisolism is followed by improvement in cardiovascular risk markers as intima-media thickness or arterial distensibility, suggesting a strong link between cortisol excess and adverse vascular remodeling. " 10/01/1995 - "Urinary free cortisol was also significantly greater in mild Cushing syndrome (540 +/- 50 nmol/d) than in patients with pseudo-Cushing states (430 +/- 41 nmol/d, p < 0.05) or normal volunteers (160 +/- 28 nmol/d, p < 0.0001). "
3.	Dexamethasone (Maxidex)FDA LinkGeneric 01/01/1989 - "Interestingly, low-dose dexamethasone test was highly valuable and the 24-hour urine 17-KS quite helpful in the differential diagnosis of two types of Cushing's syndrome. " 05/05/1993 - "The diagnostic accuracy of the dexamethasone-CRH test for Cushing's syndrome was significantly greater than the accuracy of either the low-dose dexamethasone test or the CRH test alone (P < .01). " 05/01/2001 - "Remission is defined as biochemical reversal of hypercortisolism with re-emergence of diurnal circadian rhythm, resolution of clinical features and adequate suppression on low-dose dexamethasone testing. " 11/01/1984 - "However, in 75% of the dogs with Cushing's syndrome, exogenous dexamethasone was cleared completely in the 3-hour test. " 10/01/2008 - "In this study, rats treated with daily dexamethasone (Dex) for 14 d provided a model of Cushing syndrome. "
4.	Ketoconazole (Nizoral)FDA LinkGeneric 12/01/1996 - "The most significant improvement of clinical symptoms was that the patients could sleep much more comfortably at night following the suppression of hypercortisolism by ketoconazole. " 11/04/1989 - "In view of the study results, we feel that ketoconazole is an effective treatment for the long term control of Cushing's syndrome. " 04/01/2000 - "[Therapeutic efficacy of ketoconazole in Cushing syndrome]." 02/15/1989 - "Different therapeutic efficacy of ketoconazole in patients with Cushing's syndrome." 12/01/1986 - "Ketoconazole is effective for long term control of hypercortisolism of either pituitary or adrenal origin. "
5.	Mifepristone (RU 486)FDA Link 08/01/2001 - "This case illustrates the efficacy of high-dose long-term treatment with mifepristone in refractory Cushing's syndrome. " 10/01/2015 - "This post hoc analysis included 40 clinical responders and 50 participants who received a dose of mifepristone (safety population) in the 24-week phase 3 SEISMIC (Study of the Efficacy and Safety of Mifepristone in the Treatment of Endogenous Cushing Syndrome) trial. " 10/01/2014 - "The Study of the Efficacy and Safety of Mifepristone in the Treatment of Endogenous Cushing's Syndrome (SEISMIC) was a 24-week, open-label study of mifepristone, and its long-term extension (LTE) is a multicenter U.S. " 08/01/2013 - "We review the use of mifepristone, as published in the recent Study of the Efficacy and Safety of Mifepristone in the Treatment of Endogenous Cushing's Syndrome (SEISMIC). " 08/01/2013 - "The purpose was to review the use of mifepristone in the treatment of Cushing's syndrome (CS) in the context of other recently published studies. "
6.	MetyraponeFDA Link 02/01/2001 - "Metyrapone suppression of hypercortisolism currently represents the best treatment for these rare cases." 05/01/1981 - "Prolonged remission in florid Cushing's syndrome following metyrapone treatment." 11/01/2015 - "Effectiveness of Metyrapone in Treating Cushing's Syndrome: A Retrospective Multicenter Study in 195 Patients." 01/01/2015 - "The baby girl underwent medical treatment of Cushing's syndrome with metyrapone which was followed by a rapid recovery. " 01/01/2013 - "We present a patient with refractory Cushing's syndrome successfully treated for nearly 6 years with metyrapone with minimal adverse effects. "
7.	Mitotane (Lysodren)FDA Link 09/01/2011 - "Mitotane is highly effective in the long-term management of Cushing's syndrome but has a slow onset of action. " 10/01/2000 - "Furthermore, we used mitotane with good results in Cushing's syndrome of non-malignant origin as pre-treatment before surgery or in long-term treatment for patients with poor tolerance of other adrenal inhibitors." 04/01/2014 - "The efficacy of adjuvant mitotane does not seem to be influenced by overt hypercortisolism." 03/01/1966 - "Remission in Cushing's syndrome with o,p'-DDD." 01/01/1999 - "[Effects of mitotane therapy in dogs with pituitary dependent Cushing syndrome on the adrenal gland size--an ultrasonographic study]."
8.	Corticotropin-Releasing HormoneIBA 05/22/1986 - "We conclude that the pathophysiologic features of hypercortisolism in depression and Cushing's disease are distinct in each of the disorders and that the ovine corticotropin-releasing hormone stimulation test can be helpful in their differential diagnosis." 05/22/1986 - "The purpose of this study was to evaluate whether the pituitary-adrenal responses to ovine corticotropin-releasing hormone could help to clarify the mechanism of hypercortisolism in depression and in Cushing's disease and to assist in the differential diagnosis of these disorders. " 07/05/1984 - "These studies demonstrate that ectopic secretion of corticotropin-releasing factor is a cause of Cushing's syndrome in human beings. " 06/01/2010 - "3 Tesla magnetic resonance imaging with and without corticotropin releasing hormone stimulation for the detection of microadenomas in Cushing's syndrome." 01/01/2007 - "[The value of corticotropin-releasing hormone (CRH) test for differential diagnosis of Cushing's syndrome]."
9.	Glucose (Dextrose)FDA LinkGeneric 10/01/2011 - "In conclusion, the present study showed the increased prevalence of cardiovascular risk factors in SCS patients with mild hypercortisolism related to impaired glucose/lipid metabolism. " 06/01/2015 - "Lipid abnormalities in patients with adrenal incidentalomas: role of subclinical hypercortisolism and impaired glucose metabolism." 09/23/2013 - "We clarified that GA is set lower in relation to plasma glucose levels in patients with Cushing's syndrome." 01/01/2013 - "We have previously demonstrated increased bone expression of TXNIP in patients with endogenous Cushing's syndrome (CS), and we hypothesized that TXNIP could contribute to the dysregulated glucose metabolism in CS. " 06/01/2012 - "Glucose metabolism in patients with subclinical Cushing's syndrome."
10.	Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric 03/01/2010 - "The aim of the study was to assess the ability of the DDAVP test in distinguishing between these conditions, with emphasis on subjects with mild hypercortisolism. " 01/01/2011 - "It follows, that desmopressin testing does not induce disease-specific untoward changes in coagulatory markers in patients with endogenous hypercortisolism and its use in this context appears safe. " 03/01/2010 - "Interpretation of the DDAVP test through a combination of parameters allowed effective discrimination of CD from PC, even in subjects with mild hypercortisolism." 03/01/2010 - "We performed the DDAVP test and standard diagnostic procedures for the diagnosis of Cushing's syndrome. " 04/01/2008 - "The desmopressin stimulation test in dogs with Cushing's syndrome."

Therapies and Procedures

1.	Adrenalectomy 11/01/1992 - "Left adrenalectomy resulted in complete remission of Cushing's syndrome. " 03/01/2015 - "Laparoscopic adrenalectomy seems to be beneficial in reversing several metabolic effects of hypercortisolism, with a low morbidity rate. " 01/01/2012 - "Further, unilateral adrenalectomy was effective for treating an older patient at high operative risk and a patient with subclinical Cushing's syndrome." 04/01/1997 - "Right adrenalectomy successfully relieved Cushing's syndrome. " 05/01/2007 - "Bilateral adrenalectomy for symptomatic relief of persistent hypercortisolism appears to be an effective treatment option. "
2.	Hypophysectomy 03/01/1988 - "Total hypophysectomy resulted in complete remission of Cushing's syndrome. " 08/01/1999 - "Negative sellar exploration (despite the results of endocrine evaluation indicating Cushing's disease), the high incidence of failure of total hypophysectomy, and remission of Cushing's syndrome after unsuccessful hypophysectomy and sellar irradiation suggest that the etiology of refractory Cushing's disease, in some patients, lies near the sella but not in the pituitary gland. " 05/01/2015 - "Transsphenoidal hypophysectomy is an effective treatment for dogs with pituitary-dependent hypercortisolism (PDH). " 01/01/2000 - "Five patients, whose hypercortisolism persisted after initial surgery, underwent total hypophysectomy as secondary transsphenoidal surgery, and only one of them showed endocrinological remission. " 05/01/1985 - "The third exhibited Cushing's syndrome from the autotransplants, finally cured by hypophysectomy. "
3.	Radiotherapy 01/01/2007 - "In conclusion, this study confirms that KTZ is an effective and generally well tolerated treatment for CS particularly: a) shortly before surgery, b) because of persistent hypercortisolism after surgery or awaiting the results of radiotherapy, c) as a reasonable option in patients with CS of unknown aetiology and, d) as long-term therapy in any case of unsolved hypercortisolism after failure of current treatments." 05/01/1998 - "In both patients there was progressive normalization of hypothalamo-pituitary-adrenal function and at 8 and 10 years after radiotherapy their Cushing's syndrome remained in remission clinically and biochemically. " 09/01/1998 - "Two months after the radiotherapy, we still had doubts about the prognosis regarding the persistence of the hypercortisolism. " 09/12/1981 - "In 4 additional patients an enlarged sella turcica was already found at the time they had Cushing's syndrome, and therefore they were first treated by pituitary surgery or radiotherapy. " 02/19/1960 - "[Experiences with radiotherapy of Cushing's syndrome]."
4.	Aftercare (After-Treatment) 12/01/1994 - "Corticosteroid in the blood was significantly elevated to normal level after treatment (P < 0.001), Cushing's syndrome was cleared up gradually. " 03/01/2011 - "Where reported (four studies), a similar meta-analysis was performed for those patients whose hypercortisolism was in remission after treatment compared to those patients from the same center with persistent disease. " 09/01/2002 - "After treatment, all but one of the patients were cured or controlled of their hypercortisolism. " 12/01/1996 - "The aim of this study was to ascertain the extent of mental illness in patients before and after treatment for Cushing's syndrome. " 01/01/2015 - "Cushing Syndrome is difficult to diagnose, and the comorbidities and persistent late effects of hypercortisolemia after treatment of the primary disease are challenging for the patient and the endocrinologist. "
5.	Pituitary Irradiation 09/01/1996 - "One patient needed three more operations until hypercortisolism subsided; one patient achieved remission after additional pituitary irradiation. " 05/01/1979 - "This may become the preferred treatment for pituitary Cushing's syndrome in the adult, particularly where the appropriate equipment and skills are available; for children, external pituitary irradiation seems to offer safe and effective therapy." 01/01/1967 - "Pituitary irradiation for Cushing's syndrome." 04/01/2000 - "Treatment of pituitary-dependent Cushing's syndrome: long-term results of unilateral adrenalectomy followed by external pituitary irradiation compared to transsphenoidal pituitary surgery." 01/01/1992 - "Treatment directed at relieving hypercortisolism has been instituted during pregnancy: Pituitary or adrenal surgery, chemotherapy, and pituitary irradiation have all been reported, with variable results. "