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Research and Markets(http://www.researchandmarkets.com/research/rdf6gm/pituitary_acth) has announced the addition of the “Pituitary ACTH Hypersecretion (Cushing’s Disease) – Pipeline Review, H1 2013” report to their offering.
‘Pituitary ACTH Hypersecretion (Cushing’s Disease) – Pipeline Review, H2 2013’, provides an overview of the indication’s therapeutic pipeline. This report provides information on the therapeutic development for Pituitary ACTH Hypersecretion (Cushing’s Disease), complete with latest updates, and special features on late-stage and discontinued projects. It also reviews key players involved in the therapeutic development for Pituitary ACTH Hypersecretion (Cushing’s Disease).
Scope
– A snapshot of the global therapeutic scenario for Pituitary ACTH Hypersecretion (Cushing’s Disease).
– A review of the Pituitary ACTH Hypersecretion (Cushing’s Disease) products under development by companies and universities/research institutes based on information derived from company and industry-specific sources.
– Coverage of products based on various stages of development ranging from discovery till registration stages.
– A feature on pipeline projects on the basis of monotherapy and combined therapeutics.
– Coverage of the Pituitary ACTH Hypersecretion (Cushing’s Disease) pipeline on the basis of route of administration and molecule type.
– Key discontinued pipeline projects.
– Latest news and deals relating to the products.
Companies Involved in Pituitary ACTH Hypersecretion (Cushing’s Disease) Therapeutics Development
Drug Profiles: Product Description, Mechanism of Action and R&D Progress
For more information visit http://www.researchandmarkets.com/research/rdf6gm/pituitary_acth
Filed under: pituitary, Rare Diseases | Tagged: ACTH, ACTH receptor, Adrenocorticotropic hormone, Cortendo Invest AB, cortisol, Cushing, Cushing Disease, Cushing Syndrome, Cushing's, HRA Pharma, Ipsen, Isis Pharmaceuticals, ISIS-GCCRRx, ketoconazole, LCI-699, Mechanism of Action, mifepristone, Novartis AG, pasireotide, pipeline, pituitary, Proceedings of the National Academy of Sciences of the United States of America, S.A., therapy | 1 Comment »
Case Reports in Endocrinology Volume 2013 (2013), Article ID 205208, 3 pages http://dx.doi.org/10.1155/2013/205208
1Department of Internal Medicine, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk NA10, Cleveland, OH 44195, USA
2Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, OH 44195, USA
Received 3 February 2013; Accepted 22 February 2013
Academic Editors: C. Capella, T. Cheetham, M. Demura, and K. Iida
Copyright © 2013 Aldo Schenone Giugni et al. This is an open access article distributed under theCreative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Exophthalmos is typically associated with Graves’ ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing’s syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia.
A 38-year-old female noticed progressively worsening bilateral proptosis for a period of two years, to the point causing episodes of ocular dislocation from her sockets. She also noted irregular menstrual cycles during this time and was amenorrheic for 6 months prior to referral. She underwent extensive workup by her primary care physician including thyroid tests which were normal. She then underwent orbital decompression surgery in June 2011 with transient improvement of symptoms. However in the next 12 months she gained 60 lbs and developed proximal muscle weakness, purplish abdominal striae, facial hirsutism, and easy bruisability. She was also diagnosed with new onset diabetes and hypertension during this time and was treated with Metformin and Lisinopril, respectively. Physical examination revealed an obese female with a BMI of 43 and BP . She had frank stigmata of Cushings syndrome (CS). She had bilateral proptosis with Hertel’s exophthalmometry readings of 26 mm (right) and 27 mm (left) (Figure 1). Visual acuity was bilaterally. There was no corneal/conjunctival congestion or lid retraction/lag. Fundus exam was normal. Extraocular movements were intact and visual fields were normal on confrontation. Tonometry was not performed. Labs done prior to referral indicated midnight salivary cortisol of 654 ng/dL (normal 112 ng/dL) and post 1 mg dexamethasone cortisol of 16.9 mcg/dL. Random ACTH level was 50 (8–42 pg/mL). MRI of pituitary gland revealed 1.6 cm macroadenoma with deviation of the stalk to the right (Figure 2). MRI also indicated bilateral exophthalmos with increased retrorbital fat (Figure 2). Prolactin was 40 (2–17.4 ng/mL) consistent with stalk effect, gonadotropins were low, and IGF-1, free T4 were normal. Patient underwent trans-sphenoidal removal of the tumor which stained diffusely with ACTH (Figure 3). Patient is being treated with hydrocortisone and followed closely by her ophthalmologist. Although the exophthalmos persisted after the pituitary surgery, episodes of ocular dislocation had not occurred at 3 months followup.
Exophthalmos or proptosis refers to forward displacement of the eyeball. It has to be differentiated from retraction of the eyelids, which can cause an illusion of exophthalmos. Conventionally, exophthalmos refers to ocular proptosis secondary to endocrinopathies. Graves’ disease is the most common endocrine cause of exophthalmos. Although described in 1932 by Harvey Cushing in 4 of his 12 patients with Cushings disease, this is an often forgotten clinical sign [1] in patients with CS. We have presented a case highlighting the importance of exophthalmos and its association with hypercortisolemia.
Exophthalmos is seen in about 30–45% of patients with Cushings syndrome (CS) [1–3]. Kelly reported that exophthalmos (exceeding 16 mm) occurred in 45% of active CS, 21% of iatrogenic CS, and 20% of treated CS in comparison to 2% in controls [3]. Cases of severe exophthalmos preceding the evolution of CS have been reported in the literature [4, 5].
The cause of exophthalmos in CS is still unknown. Multiple theories have been proposed including fat redistribution and increased retro-orbital fat, associated thyroid disease, and an exophthalmos causative factor. It has been proposed that retro-orbital fat deposition is also part of the fat re-distribution seen in CS, resulting in increase in volume of the retro-orbital tissues and a consequent rise in intra-orbital pressure [3, 6]. Orbital fat volume was increased in patients with CS and orbital muscles are relatively spared [7, 8]. In contrast to patients with Graves’ disease the retrorbital fat in CS is devoid of inflammatory cell infiltration. Whether differential fat deposition in the orbits is due to increased glucocorticoid receptor density, defective lipolysis or increased lipoprotein lipase activity is not known.
The authors report no conflict of interests.
Filed under: Cushing's, pituitary | Tagged: Cushing's, endocrinology, exophtalmos | Leave a comment »
November 7, 2012 The US Food and Drug Administration’s (FDA) Endocrinologic and Metabolic Drugs Advisory Committee (EMDAC) voted unanimously in support of the use of Signifor® (pasireotide) for the treatment of patients with Cushing’s disease who require medical therapeutic intervention. The recommendation was based on data from clinical trials of pasireotide, including PASPORT-CUSHINGS (PASireotide clinical trial PORTfolio – CUSHING’S disease), the largest randomized Phase III study to evaluate a medical therapy in patients with Cushing’s disease.
Patient Assistance for SIGNIFOR support for patients includes:
– Therapy-specific support programs for out-of-pocket costs
– Alternative assistance searches and referrals to Federal and State assistance programs
– Referrals to Independent Charitable Foundations for assistance with co-pay costs
– Patient assistance for low-income and uninsured patients
For more information, or to speak to a Patient Assistance NOW Endocrinology representative, please call 1-877-503-3377 (select option #3 for SIGNIFOR) Monday to Friday 8 am – 8 pm ET.
Filed under: Clinical trials, Cushing's, pituitary | Tagged: Cushing's, FDA, pasireotide, patient assistance, Signifor, therapy, treatment | 1 Comment »
Even after successful treatment, patients with Cushing’s disease who were older when diagnosed or had prolonged exposure to excess cortisol face a greater risk of dying or developing cardiovascular disease, according to a recent study accepted for publication in The Endocrine Society’s Journal of Clinical Endocrinology & Metabolism (JCEM).
Cushing’s disease is a rare condition where the body is exposed to excess cortisol – a stress hormone produced in the adrenal gland – for long periods of time.
Researchers have long known that patients who have Cushing’s disease are at greater risk of developing and dying from cardiovascular disease than the average person. This study examined whether the risk could be eliminated or reduced when the disease is controlled. Researchers found that these risk factors remained long after patients were exposed to excess cortisol.
“The longer patients with Cushing’s disease are exposed to excess cortisol and the older they are when diagnosed, the more likely they are to experience these challenges,” said Eliza B. Geer, MD, of Mount Sinai Medical Center and lead author of the study. “The findings demonstrate just how critical it is for Cushing’s disease to be diagnosed and treated quickly. Patients also need long-term follow-up care to help them achieve good outcomes.”
The study found cured Cushing’s disease patients who had depression when they started to experience symptoms of the disease had an elevated risk of mortality and cardiovascular disease. Men were more at risk than women, a trend that may be explained by a lack of follow-up care, according to the study. In addition, patients who had both Cushing’s syndrome and diabetes were more likely to develop cardiovascular disease.
The study examined one of the largest cohorts of Cushing’s disease patients operated on by a single surgeon. The researchers retrospectively reviewed charts for 346 Cushing’s disease patients who were treated between 1980 and 2011. Researchers estimated the duration of exposure to excess cortisol by calculating how long symptoms lasted before the patient went into remission. The patients who were studied had an average exposure period of 40 months.
The findings may have implications for people who take steroid medications, Geer said. People treated with high doses of steroid medications such as prednisone, hydrocortisone or dexamethasone are exposed to high levels of cortisol and may experience similar conditions as Cushing’s disease patients.
“While steroid medications are useful for treating patients with a variety of conditions, the data suggests health care providers need to be aware that older patients or those who take steroid medications for long periods could be facing higher risk,” Geer said. “These patients should be monitored carefully while more study is done in this area.”
From http://www.medicalnewstoday.com/releases/256284.php
Filed under: Cushing's, Health Care, pituitary | Tagged: adrenal, cardiovascular, cortisol, Cushing's, depression, dexamethasone, diabetes, hydrocortisone, Mount Sinai Medical Center, prednisone | 2 Comments »
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