Sunday, June 30, 2013

Cushing Disease: A Multidisciplinary Treatment Update

This activity is intended for endocrinologists, primary care physicians, nurses, nurse practitioners, and pharmacists.

The goal of this activity is to review the diagnosis and treatment of Cushing disease from a multidisciplinary perspective.

Upon completion of this activity, participants will be able to:
  1. Outline the rationale for a multidisciplinary approach to the diagnosis and treatment of patients with Cushing disease
  2. Review the safety and efficacy of current management strategies for patients with Cushing disease
  3. Describe the diagnostic workup for Cushing disease and the reasons why timely diagnosis and treatment are important

Faculty and Disclosures

As an organization accredited by the ACCME, Medscape, LLC, requires everyone who is in a position to control the content of an education activity to disclose all relevant financial relationships with any commercial interest. The ACCME defines "relevant financial relationships" as financial relationships in any amount, occurring within the past 12 months, including financial relationships of a spouse or life partner, that could create a conflict of interest.
Medscape, LLC, encourages Authors to identify investigational products or off-label uses of products regulated by the US Food and Drug Administration, at first mention and where appropriate in the content.

Laurence Katznelson, MD

Professor of Medicine and Neurosurgery, Stanford University; Medical Director, Pituitary Center, Stanford Hospital and Clinics, Stanford, California
Disclosure: Laurence Katznelson, MD, has disclosed the following relevant financial relationships:
Received grants for clinical research from: Corcept Therapeutics Inc.; Novartis Pharmaceuticals Corporation

Dr Katznelson does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Katznelson does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Brooke Swearingen, MD

Associate Professor of Neurosurgery, Harvard Medical School; Associate Visiting Neurosurgeon, Massachusetts General Hospital, Boston, Massachusetts
Disclosure: Brooke Swearingen, MD, has disclosed the following relevant financial relationships: Served as an advisor or consultant for: Novartis Pharmaceuticals Corporation
Owns stock, stock options or bonds from: Novartis Pharmaceuticals Corporation; Pfizer Inc; Amgen Inc; Roche

Dr Swearingen does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Swearingen does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Nicholas Tritos, MD

Assistant Professor of Medicine, Harvard Medical School; Staff, Neuroendocrine Unit, Massachusetts General Hospital, Boston, Massachusetts
Disclosure: Nicholas Tritos, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Corcept Therapeutics Inc; Pfizer Inc
Received grants for clinical research from: Pfizer Inc; Ipsen

Dr Tritos does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Tritos does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Susan Cornell, PharmD, CDE

Associate Professor, Pharmacy Practice, Midwestern University-Chicago, Downers Grove, Illinois; Clinical Pharmacist/Certified Diabetes Educator, DuPage Community Clinic, Wheaton, Illinois
Disclosure: Susan Cornell, PharmD, CDE, has disclosed the following relevant relationships:
Served as a speaker or member of a speakers bureau for: Johnson & Johnson Diabetes Institute

Dr Cornell does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Cornell does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Rita Pach, RN, MSN

Nurse, Johns Hopkins Pituitary Center, Baltimore, Maryland
Participation by Mrs Pach in the development of this product does not constitute or imply endorsement by the Johns Hopkins University or the Johns Hopkins Hospital and Health System.
Disclosure: Rita Pach, RN, has disclosed no relevant financial relationships.

Mrs Pach does not intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Mrs Pach does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Kristin M. Richardson

Group Scientific Director, Medscape, LLC
Disclosure: Kristin M. Richardson has disclosed no relevant financial relationships.

David Modrak, PhD

Freelance editor, Montville, New Jersey
Disclosure: David Modrak, PhD, has disclosed no relevant financial relationships.

Nafeez Zawahir, MD

CME Clinical Director, Medscape, LLC
Disclosure: Nafeez Zawahir, MD, has disclosed no relevant financial relationships.

Laurie E. Scudder, DNP, NP

Nurse Planner, Continuing Professional Education Department, Medscape, LLC; Clinical Assistant Professor, School of Nursing and Allied Health, George Washington University, Washington, DC
Disclosure: Laurie E. Scudder, DNP, NP, has disclosed no relevant financial relationships.

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This activity is designed to be completed within the time designated on the title page; physicians should claim only those credits that reflect the time actually spent in the activity. To successfully earn credit, participants must complete the activity online during the valid credit period that is noted on the title page. To receive AMA PRA Category 1 Credit™, you must receive a minimum score of 70% on the post-test.

Follow these steps to earn CME/CE credit*:
  1. Read the target audience, learning objectives, and author disclosures.
  2. Study the educational content online or printed out.
  3. Online, choose the best answer to each test question. To receive a certificate, you must receive a passing score as designated at the top of the test. We encourage you to complete the Activity Evaluation to provide feedback for future programming.
You may now view or print the certificate from your CME/CE Tracker. You may print the certificate but you cannot alter it. Credits will be tallied in your CME/CE Tracker and archived for 6 years; at any point within this time period you can print out the tally as well as the certificates from the CME/CE Tracker.

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Pregnancy and pituitary disorders

Z Karaca, F Tanriverdi, K Unluhizarci and F Kelestimur
+ Author Affiliations
Department of Endocrinology,
Erciyes University Medical School, 38039 Kayseri, Turkey
(Correspondence should be addressed to F Kelestimur; Email:

Major hormonal changes emerge during pregnancy. The pituitary gland is one of the most affected organs with altered anatomy and physiology. The pituitary gland is enlarged as a result of lactotroph hyperplasia. Due to physiological changes in the pituitary and target hormone levels, binding globulins, and placental hormones, hormonal evaluation becomes more complex in pregnant women. As a consequence of physiological hormonal changes, the evaluation of pituitary functions in pregnant women is quite different from that done in the prepregnant state. Pituitary adenomas may cause problems by their hormone secretion that affects the mother and the fetus besides causing an increased risk of tumor growth.

Furthermore, diagnosis, course, and treatment of pituitary diseases point out differences. The changes in anatomy and physiology of the pituitary gland during pregnancy are reviewed.

Pituitary disorders namely Cushing's disease; acromegaly; prolactinoma; TSH-secreting, gonadotropin-producing, and clinically nonfunctioning adenomas; craniopharyngioma; and Sheehan's syndrome, which is one of the most common causes of hypopituitarism, lymphocytic hypophysitis, and hypopituitarism, in relation to pregnancy are discussed. Being aware of all this information will prevent any serious problems which mother and child will be exposed to.

Read the entire article here:

Saturday, June 29, 2013

From Bangladesh ~ Pituitary Adenoma: When headache is a headache

“Got headache? Take a paracetamol and get relieved in a short while.”

This is common practice in our country. Almost everyone has had a headache, but rarely headache becomes a headache in our lives. Not all headaches require doctor’s attention but sometimes it represent the tip of a huge iceberg.

Mr Shafiul Islam, 38 years of age, an active male developed a gradual onset of headache, which worsened at the morning, followed by vomiting. He visited a general practitioner and took prescribed medicines, but that failed to cure the symptoms. Rather he was gradually experiencing loss of outer side vision of both eyes.

When he revisited a doctor and was advised for MRI of brain he was diagnosed with a core of “Pituitary Macrodenoma,” a tumor of a hormone producing gland of brain. Then Shafiul was referred to Neurosurgeon of Comfort Nursing home Assistant Professor Dr Moshiur Rahman, who decided to perform operation for removal of the tumor after the initial evaluation.

The pituitary gland is an endocrine gland about the size of a pea and weighing 5 grams (0.18 oz) in humans. It is a protrusion off the bottom of the hypothalamus at the base of the brain, and rests in a small, bony cavity (sella turcica). The pituitary gland secretes nine hormones. A pituitary adenoma is a slow growing and less harmful tumor arising from cells in the pituitary gland. Because they originate from cells in the pituitary gland, which is the master hormone gland, they often cause problems related to hormonal dysfunction.

Some pituitary tumors result in excessive production and over-secretion of hormones, which can result in a variety of syndromes. A large proportion of these tumors, however, do not produce any functional hormones, but instead grow to a size where they cause symptoms because they compress surrounding structures. For these reasons, larger pituitary tumors (called macroadenomas) often present with headache, visual loss and pituitary gland dysfunction.
The specific cause of pituitary adenoma development is unknown, although they are likely to be caused in part by random mutations in cells of the pituitary gland. Surgery is the first line of treatment for many symptomatic pituitary tumors in patients that are good surgical candidates, especially in patients with nonfunctioning macroadenomas.

Dr Moshiur approached the tumor by entering through nasal opening with the help of ENT specialist Associate Professor Dr Sajol Ashfaq, under general aenesthesia (fully unconscious) done by Aenesthesiologist Associate Professor Dr Shamsul Alam. After elevation of a thin membrane over the nasal partition and breaking a bone in the base of the skull they got a vision of the tumor through endoscope. After that, the tumor was removed through the nose. After three days of post-operative care, the patient was discharged. All his symptoms, headache, vomiting and poor vision improved dramatically and he got back to his normal life.

Dr Moshiur Rahman said: “The surgical approach for removing pituitary tumours is usually an endonasal (through the nostril) transsphenoidal (through the sphenoid sinus) approach. This procedure is Endoscopic Transnasal Transphenoidal Pituitary Adenomectomy, which is a safe, minimally invasive but effective, modern treatment option for Pituitary Adenoma, with few side effects and short post-operative hospital stay. This latest technology is being practiced in some centres of the capital for last few years.”

He also said, he performed three operations before successfully with no long term adverse effect. He also paid gratefulness to Associate Professor Dr Sajol Ashfaq and Associate Professor Dr Shamsul Alam for their sincere and great effort.

Once, people had to go outside of the country for this operation. Nowadays, this operation is often performed by many neurosurgeons of the capital. A few centres have also developed to provide these facilities of operation. People can take this oppurtunity confidently by choosing a competent surgical team.

Tuesday, June 25, 2013

Endoscopic transsphenoidal pituitary surgery: a good and safe primary treatment option for Cushings disease, even in case of macroadenomas or invasive adenomas.

Abstract - European Journal of Endocrinology, 06/24/2013  Clinical Article

Wagenmakers MA et al. – This study aims to gain insight in the role of endoscopic TS as a primary treatment option for CD, especially in patients with MRI negative CD and (invasive) macroadenomas. 
The data suggest that in patients with non–invasive and invasive macroadenomas the endoscopic technique of TS should be the technique of choice as remission rates seem to be higher than remission rates reported for microscopic TS, although no comparative study has been performed.

Quality of Life and Other Outcomes in Children Treated for Cushing Syndrome

Journal of Clinical Endocrinology and Metabolism, 05/16/2013  Review Article  Clinical Article

Keil MF – Cushing syndrome (CS) in children is associated with residual impairment in measures of health–related quality of life, even after successful resolution of hypercortisolemia, highlighting the need for early identification of morbidities and improvements in long–term management of these patients.

A review of the literature identifies significant morbidities associated with CS of pediatric onset, which must not be treated in isolation. CS affects children and adolescents in many ways that are different than adults. 

Post–treatment challenges for the child or adolescent treated for CS include: optimize growth and pubertal development, normalize body composition, and promote psychological health and cognitive maturation. All these factors impact health–related quality of life, which is an important outcome measure to assess the burden of disease as well as the effect of treatment.

Monday, June 24, 2013

Research on Cushing’s Disease Presented at ENDO 2013


Researchers at PHAR, in conjunction with experts from Novartis Pharmaceuticals Corporation, presented the results of several studies on Cushing’s disease at the Endocrine Society’s 95th Annual Meeting on June 19th, 2013.

Cushing’s disease, a pituitary disorder, is poorly studied because of its rarity. The PHAR research team reported on the incidence, treatment patterns, and annual economic burden associated with the disease–topics on which very little was previously known.

The three posters presented at ENDO can be viewed by clicking the links below:

Sunday, June 23, 2013

Have You Gone to the ER With an Adrenal Crisis?

This Survey is to gather information for the Adrenal Insufficiency Awareness Organization's grant project to create educational materials for ER personnel.

The survey is for those of you who have been to the ER during an impending or full blown Adrenal Crisis. (you may fill it out for a child or yourself)

 Your help is appreciated!

At the end of the survey you will have the chance to enter for a chance to win an Adrenal Insufficiency Awareness Pin.

Pasireotide for the treatment of Cushing's disease

Posted online on June 17, 2013.

Annamaria Colao Chiara Simeoli Monica De Leo Alessia Cozzolino Rosario Pivonello
Department of Clinical Medicine and Surgery, Section of Endocrinology, Federico II University, Via Sergio Pansini 5, 
80131 Naples
, Italy +39 0817462132; +39 0815465443;
Author for correspondence

Introduction: Pasireotide, a novel multireceptor targeted somatostatin analog is the first drug approved for treatment of adult patients with Cushing's disease (CD) for whom pituitary surgery is not an option or has not been curative.

Areas covered: The review describes published data on efficacy and safety of pasireotide in CD patients. In particular, the review focuses on a Phase III study (CSOM230B2305) evaluating the outcomes of treatment with pasireotide at the doses of 600 and 900 µg twice daily for 12 months in 162 CD patients. This clinical trial reported a decrease in urinary free cortisol levels in the majority of patients, with a substantial reduction in nearly half and a normalization in > 25% of patients included in the study, accompanied by an improvement in clinical picture as well as a significant reduction in pituitary tumor size. Hyperglycemia appears as the most important side effect, requiring a careful monitoring and a prompt administration of glucose-lowering medications.

Expert opinion: Pasireotide seems to have a promising role as medical option for CD patients who experienced a failure or not candidate for neurosurgery; its employment will probably induce in the near future significant changes in the therapeutic approach to CD.

Read More:

Saturday, June 22, 2013

Are You a Korlym Patient?

If so, please note - On July 1st, Korlym will not be supplied by Curascript and Accredo any more. 

Korlym will continue to be available, just through a different pharmacy called Centric Health Resources.  They are trying to reach all of you to introduce you to their program and enhanced services as well as set up your next shipment. 

If you have not yet spoken with them, please call them at 866-758-7138.

For reference, their website is:

Friday, June 21, 2013

Want to Go to an Endocrinology Conference?

73rd Scientific Sessions (2013) of American Diabetes Association CME
June 21-25, 2013 -Chicago, Illinois, United States
6th International Conference on Children s Bone Health CME
June 22-25, 2013 -Rotterdam, Netherlands
2013 Annual Meeting of the Society for Behavioral Neuroendocrinology CME
June 23-26, 2013 -Atlanta, Georgia, United States
17th Annual Conference on Hypertension - Focus on Hypertension, Diabetes and Dyslipidemia CME
June 28-30, 2013 -Charleston, South Carolina, United States
Bone Densitometry Comprehensive Exam Review CME
August 3, 2013 -Houston, Texas, United States
4th World Congress on Diabetes & Metabolism CME
August 14-16, 2013 -Skokie, Illinois, United States
Anti-Aging Medicine: Advances in Hormone Replacement CME
August 17-18, 2013 -Chicago, Illinois, United States
World Congress on Endocrinology 2013 CME
August 26-28, 2013 -Raleigh, North Carolina, United States
HBPR 2013: High Blood Pressure Research 2013 Scientific Sessions CME
September 11-14, 2013 -New Orleans, Louisiana, United States
35th Annual British Andrology Society Meeting CME
September 13-14, 2013 -Liverpool, United Kingdom
5th Florence-Utah Symposium on Genetics of Male Infertility
September 19-21, 2013 -Florence, Italy
2013 Cardiometabolic Health Congress CME
October 2-5, 2013 -Boston, Massachusetts, United States
Bone Densitometry: Principles and Procedures CME
October 5-6, 2013 -Houston, Texas, United States
11th Annual Meeting of Androgen Excess & PCOS Society CME
October 17-18, 2013 -Newport, Rhode Island, United States
AAES 2014 Annual Meeting CME
April 27-29, 2014 -Boston, Massachusetts, United States
74th Scientific Sessions of American Diabetes Association CME
June 13-17, 2014 -San Francisco, California, United States
50th EASD Annual Meeting CME
September 15-19, 2014 -Vienna, Austria
84th Annual Meeting of the American Thyroid Association CME
October 29 - November 2, 2014 -Coronado, California, United States
March 5-8, 2015 -San Diego, California, United States
AAES 2015 Annual Meeting CME
May 17-19, 2015 -Nashville, Tennessee, United States

Tuesday, June 18, 2013

Research and Markets: Pituitary ACTH Hypersecretion (Cushing's Disease) - Pipeline Review Report, H1 2013 Edition

'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).
- 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
  • Isis Pharmaceuticals, Inc.
  • Ipsen S.A.
  • Novartis AG
  • HRA Pharma, SA
  • Cortendo Invest AB
Drug Profiles: Product Description, Mechanism of Action and R&D Progress
  • LCI-699
  • mifepristone
  • Inhibitors of ACTH receptor
  • ketoconazole
  • Next Generation Cortisol Inhibitor
  • pasireotide Long Acting Release

Read more here:

Friday, June 14, 2013

The Rare Disease Search Engine That Outperforms Google

A powerful new search engine designed to help diagnose rare diseases could prove a boon for both medics and the public.
In the late 1940s, a professor at the University of Maryland School of Medicine coined an unusual phrase to describe unexpected diagnoses. “When you hear hoofbeats behind you, don’t expect to see a zebra,” he said. The phrase stuck and today, medics commonly use the term “zebra” to describe a rare disease, usually defined as one that occurs in less than 1 in 2000 of the population.
Rare diseases are inherently hard to diagnose. According to the European Organisation for Rare Disease, 25 per cent of diagnoses are delayed by between 5 and 30 years.
So it’s no surprise that medics are looking for more effective ways to do the job. An increasingly common aid in this process is the search engine, typically Google.  This forms part of an iterative process in which a medic enter symptoms into a search engine, examines lists of potential diseases and then looks for further evidence of symptoms in the patient.
The problem, of course, is that  common-or-garden search engines are not optimised for this process. Google, for example, considers pages important if they are linked to by other important pages, the basis of its famous PageRank algorithm. However, rare diseases by definition are unlikely to have a high profile on the web. What’s more, searches are likely to be plagued with returns from all sorts of irrelevant sources.
Today, Radu Dragusin at the Technical University of Denmark and a few pals unveil an alternative. These guys have set up a bespoke search engine dedicated to the diagnosis of rare diseases called FindZebra, a name based on the common medical slang for a rare disease. After comparing the results from this engine against the same searches on Google, they show that it is significantly better at returning relevant results.
The magic sauce in FindZebra is the index it uses to hunt for results. These guys have created this index by crawling a specially selected set of curated  databases on rare diseases. These include the Online Mendelian Inheritance in Man database, the Genetic and Rare Diseases Information Center and Orphanet
They then use the open source information retrieval tool Indri  to search this index via a website with a conventional search engine interface. The result is FindZebra.
Finally, they compared the results of  searches on FindZebra against the same search on Google applied to the same limited dataset, a feature that is possible with advanced Google searches.  Dragusin and co say that the Google results are significantly worse than their own.
For example, on FindZebra the search query “Boy, normal birth, deformity of both big toes (missing joint), quick development of bone tumor near spine and osteogenesis at biopsy” returns the correct diagnosis “Fibrodysplasia ossificans progressiva” as the first result. However, this diagnosis does not appear at all in the results from any type of Google search.
This indicates that the PageRank algorithm, or at least the way Google has tweaked it, is not suited to this kind of search. “Our finding, that FindZebra outperforms Google overall for this task and especially when restricted to the sites of our collection (Google Restricted), suggests that Google ranking algorithm is suboptimal for the task at hand,” they conclude.
Although still a research project, Dragusin and co have made their rare disease search engine publicly available at This could clearly become a valuable tool for the medical community.
What is less clear, however, is how this tool will be used by the general public. The site comes with the forlorn message: “Warning! FindZebra is a research project and it is to be used only by medical professionals” .
FindZebra could obviously be a hypochondriac’s charter. On the other hand, that’s true of any medical dictionary.
The informed public are increasingly visiting their doctors armed with detailed information downloaded form the internet.  Any move to improve the quality of this information must surely be of significant value.
Ref: FindZebra: A Search Engine For Rare Diseases

Tuesday, June 11, 2013

A Subtle Case of Cushing's

Synopsis: 36 year old male who presented with weight gain, proximal muscle weakness, and excessive sweating. Among multiple 24-hour urine free cortisol and midnight salivary tests, a minority were minimally elevated.

The diagnosis of Cushing's disease was questioned over an eight month evaluation period.

The challenges of diagnosing Cushing's disease are discussed.

Read more here

Monday, June 3, 2013

Researchers Find the Cause of Cushing’s Disease

A team of researchers may have zeroed in on the cause of Cushing’s disease, a condition that leads to diabetes, obesity and the risk of premature death.
More women than men get the disease, which begins usually between 20 and 50 with mostly benign tumors in the pituitary gland. It’s known that that condition results in excess production of adrenocorticotrophic hormone (ACTH). But until now, scientists haven’t been sure what drives the production of ACTH.
Now, UCLA researchers and their colleagues have zeroed in on the culprit: excessive production of testicular orphan nuclear receptor (TR4). By reducing the TR4 in lab mice, they were able to reverse tumor growth and excess ACTH production.
The findings, published in the journal Proceedings of the National Academy of Sciences, could point the way to targeted treatment of Cushing’s.