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Endocrinologists have underlined the importance that physicians consider "a stress dose" of glucocorticoids in the event of severe COVID-19 infection in endocrine, and other, patients on long-term steroids.

 

People taking corticosteroids on a routine basis for a variety of underlying inflammatory conditions, such as asthma, allergies, and arthritis, are at elevated risk of being infected with, and adversely affected by, COVID-19. 

 

This also applies to a rarer group of patients with adrenal insufficiency and uncontrolled Cushing syndrome, as well as secondary adrenal insufficiency occurring in hypopituitarism, who also rely on glucocorticoids for day-to-day living.

 

In the event of COVID-19, all of these individuals may be unable to mount a normal stress response, and "in the case of adrenal suppression...such patients may run into severe difficulties, particularly if on intensive care units," warns Paul Stewart, MD, University of Leeds, UK, and editor-in-chief of the Journal of Clinical Endocrinology & Metabolism (JCEM).

 

As such, it is vitally important to recognize that "Injectable supplemental glucocorticoid therapy in this setting can reverse the risk of potentially fatal adrenal failure and should be considered in every case," Stewart and colleagues emphasize in a newly published editorial in JCEM.

 

They note this advice must be considered alongside World Health Organization (WHO) guidance against prescribing therapeutic glucocorticoids to treat complications of COVID-19, based on prior experience in patients with acute respiratory distress syndrome, as well as those affected by severe acute respiratory syndrome (SARS) and Middle East respiratory syndrome (MERS).
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With the novel COVID-19 virus continuing to spread, it is crucial to adhere to the advice from experts and the Centers for Disease Control and Prevention (CDC) to help reduce risk of infection for individuals and the population at large. This is particularly important for people with adrenal insufficiency and people with uncontrolled Cushing’s Syndrome.

Studies have reported that individuals with adrenal insufficiency have an increased rate of respiratory infection-related deaths, possibly due to impaired immune function. As such, people with adrenal insufficiency should observe the following recommendations:

Maintain social distancing to reduce the risk of contracting COVID-19


Continue taking medications as prescribed


Ensure appropriate supplies for oral and injectable steroids at home, ideally a 90-day preparation


In the case of hydrocortisone shortages, ask your pharmacist and physician about replacement with different strengths of hydrocortisone tablets that might be available. Hydrocortisone (or brand name Cortef) tablets have 5 mg, 10 mg or 20 mg strength




In cases of acute illness, increase the hydrocortisone dose per instructions and call the physician’s office for more details


Follow sick day rules for increasing oral glucocorticoids or injectables per your physician’s recommendations


In general, patients should double their usual glucocorticoid dose in times of acute illness


In case of inability to take oral glucocorticoids, contact your physician for alternative medicines and regimens...
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So, with the reality that social distancing is a must, how are you all handling that emotionally?

How do you stay connected and not isolated?

Do you have a plan? 

I've made one for my piano studio but I don't really have one for my family other than stocking up on food, sorta.  I already had a lot of hand sanitizer thanks to the piano students and my need to inject growth hormone every day.

If you want to chat, let me know and we can get it done.

 
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The U.S. Food and Drug Administration today approved Isturisa (osilodrostat) oral tablets for adults with Cushing's disease who either cannot undergo pituitary gland surgery or have undergone the surgery but still have the disease. Cushing's disease is a rare disease in which the adrenal glands make too much of the cortisol hormone. Isturisa is the first FDA-approved drug to directly address this cortisol overproduction by blocking the enzyme known as 11-beta-hydroxylase and preventing cortisol synthesis.

"The FDA supports the development of safe and effective treatments for rare diseases, and this new therapy can help people with Cushing's disease, a rare condition where excessive cortisol production puts them at risk for other medical issues," said Mary Thanh Hai, M.D., acting director of the Office of Drug Evaluation II in the FDA's Center for Drug Evaluation and Research. "By helping patients achieve normal cortisol levels, this medication is an important treatment option for adults with Cushing's disease."

Cushing's disease is caused by a pituitary tumor that releases too much of a hormone called adrenocorticotropin, which stimulates the adrenal gland to produce an excessive amount of cortisol. The disease is most common among adults between the ages of 30 to 50, and it affects women three times more often than men. Cushing's disease can cause significant health issues, such as high blood pressure, obesity, type 2 diabetes, blood clots in the legs and lungs, bone loss and fractures, a weakened immune system and depression. Patients may have thin arms and legs, a round red full face, increased fat around the neck, easy bruising, striae (purple stretch marks) and weak muscles. 
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Along with all of you, NADF is monitoring this outbreak by paying close attention to CDC and FDA updates. We have also asked our Medical Advisor to help answer your important questions as they come up.

We asked Medical Director Paul Margulies, MD, FACE, FACP to help us with this question:

Question: Does Adrenal Insufficiency cause us to have a weakened immune system and therefore make us more susceptible?

Response: Individuals with adrenal insufficiency on replacement doses of glucocorticoids do not have a suppressed immune system. The autoimmune mechanism that causes Addison’s disease does not cause an immune deficiency that would make one more likely to get an infection. The problem is with the individual’s ability to deal with the stress of an infection once it develops. Those with adrenal insufficiency fall into that category. When sick with a viral infection, they can have a more serious illness, and certainly require stress dose steroids to help to respond to the illness. If someone with adrenal insufficiency contracts the coronavirus, it is more likely to lead to the need for supportive care, including hospitalization.

This information from the CDC Website provides important information regarding Prevention & Treatment.  You can find this information here: https://www.cdc.gov/coronavirus/2019-ncov/about/prevention-treatment.html

 

From https://www.nadf.us/
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The Barrow Pituitary Center is dedicated to educating patients, caregivers, and loved ones by providing information which is current and non-biased. Experts at this conference will address management of the emotional and physical elements of living with pituitary disorders. We hope attendees will leave empowered to make better informed decisions about their healthcare and achieve their goals for a long and fruitful life.

Saturday, March 14, 2020
8:00 a.m. to 4:00 p.m.
$30 per person

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Presented by

Varun Kshettry, MD
Director, Advanced Endoscopic & Microscopic Neurosurgery
Cleveland Clinic Lerner College of Medicine

Register Now

After registering you will receive a confirmation email with details about joining the webinar.

 

Date: Tuesday, February 18, 2020 
Time: 10:00 AM - 11:00 AM Pacific Standard Time, 1:00 PM - 2:00 PM Eastern Standard Time

Learning Objectives:

Discuss patient expectations for pituitary surgery and recovery
Discuss best practices to minimize risk of complications
What questions to ask your medical providers
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Published: 13 January 2020

Abstract

Background

Primary aldosteronism (PA) plus subclinical Cushing’s syndrome (SCS), PASCS, has occasionally been reported. We aimed to clinically characterize patients with PASCS who are poorly profiled.

Methods

A population-based, retrospective, single-center, observational study was conducted in 71 patients (age, 58.2 ± 11.2 years; 24 males and 47 females) who developed PA (n = 45), SCS (n = 12), or PASCS (n = 14). The main outcome measures were the proportion of patients with diabetes mellitus (DM), serum potassium concentration, and maximum tumor diameter (MTD) on the computed tomography (CT) scans.

Results

The proportion of DM patients was significantly greater in the PASCS group than in the PA group (50.0% vs. 13.9%, p <  0.05), without a significant difference between the PASCS and SCS groups. Serum potassium concentration was significantly lower in the PASCS group than in the SCS group (3.2 ± 0.8 mEq/L vs. 4.0 ± 0.5 mEq/L; p <  0.01), without a significant difference between the PASCS and PA groups. Among the 3 study groups of patients who had a unilateral adrenal tumor, MTD was significantly greater in the PASCS group than in the PA group (2.7 ± 0.1 cm vs. 1.4 ± 0.1 cm; p <  0.001), without a significant difference between the PASCS and SCS groups.

Conclusions

Any reference criteria were not obtained that surely distinguish patients with PASCS from those with PA or SCS. However, clinicians should suspect the presence of concurrent SCS in patients with PA when detecting a relatively large adrenal tumor on the CT scans.

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Houston Methodist neurosurgeons and neuroscientists are looking at a new way to classify pituitary tumors that could lead to more precise and accurate diagnosing for patients in the future.

Found in up to 10% of the population, pituitary tumors, also called adenomas, are noncancerous growths on the pituitary gland and very common. Although these pituitary tumors are benign in nature, they pose a major health challenge in patients.

The new tests being investigated at Houston Methodist not only have the potential to lead to better diagnoses for patients with pituitary adenomas, but also for many other types of brain tumors in the future. The findings, which were published Jan. 28 in Scientific Reports, an online journal from Nature Publishing Group, describe a new way being looked at to study the blood of patients with pituitary tumors to determine exactly what tumor type they have and whether they might respond to medical treatment rather than surgery.

"Often called the 'master gland,' the pituitary gland controls the entire endocrine system and regulates various body functions by secreting hormones into the bloodstream to control such things as metabolism, growth and development, reproduction and sleep," said corresponding author Kumar Pichumani, Ph.D., a research physicist at the Houston Methodist Research Institute. "When pituitary adenomas occur, they may secrete too much of one or more hormones that could lead to a variety of issues, ranging from infertility and sexual dysfunction to vision problems and osteoporosis, among many other health problems."

Neurosurgeon David S. Baskin, M.D., director of the Kenneth R. Peak Center for Brain and Pituitary Tumor Treatment and Research in the Department of Neurosurgery at Houston Methodist Hospital, collaborated with Pichumani on this study. He said some pituitary tumors can be treated with medication rather than surgery, but a precise diagnosis of the type of tumor someone has and what hormone it's secreting is essential for proper treatment. This is sometimes very difficult to do based on standard endocrine hormone testing.
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Adrenococortical carcinoma (ACC) is a rare cancer, occurring at the rate of one case in two million person years. Cushing syndrome or a mixed picture of excess androgen and glucocorticoid production are the most common presentations of ACC. Other uncommon presentations include abdominal pain and adrenal incidentalomas. In the present report, a 71-year-old male presented with abdominal pain and was eventually diagnosed with ACC. He was found to have pulmonary thromboembolism following an investigation for hypoxemia, with the tumor thrombus extending upto the right atrium. This interesting case represents the unique presentation of a rare tumor, which if detected late or left untreated is associated with poor outcomes, highlighting the need for a low index of suspicion for ACC when similar presentations are encountered in clinical practice.

ACC is a rare but aggressive tumor. ACC commonly presents with rapid onset of hypercortisolism, combined hyperandrogenism and hypercortisolism, or uncommonly with compressive symptoms. Clinicians should have a low index of suspicion for ACC in patients presenting with rapid onset of symptoms related to hypercortisolism and/or hyperandrogenism. Venous thromboembolism and extension of the tumor thrombus to the right side of the heart is a very rare but serious complication of ACC that clinicans should be wary of. The increased risk of venous thromboembolism in ACC could be explained by direct tumor invasion, tumor thrombi or hypercoagulability secondary to hypercortisolism. Early diagnosis and prompt treatment can improve the long-term survival of patients with ACC.
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Sponsor:



Cedars-Sinai Medical Center



Information provided by (Responsible Party):



Shlomo Melmed, MD, Cedars-Sinai Medical Center



 




Brief Summary:




This phase 2 multicenter, open-label clinical trial will evaluate safety and efficacy of 4 weeks of oral seliciclib in patients with newly diagnosed, persistent, or recurrent Cushing disease.

Funding Source - FDA Office of Orphan Products Development (OOPD)

 




Condition or disease 


Intervention/treatment 


Phase 




Cushing Disease


Drug: Seliciclib


Phase 2



 



Detailed Description:



This phase 2 multicenter, open-label clinical trial will evaluate safety and efficacy of two of three potential doses/schedules of oral seliciclib in patients with newly diagnosed, persistent, or recurrent Cushing disease. Up to 29 subjects will be treated with up to 800 mg/day oral seliciclib for 4 days each week for 4 weeks and enrolled in sequential cohorts based on efficacy outcomes. The study will also evaluate effects of seliciclib on quality of life and clinical signs and symptoms of Cushing disease.





 



 






Ages Eligible for Study:  


18 Years and older   (Adult, Older Adult)




Sexes Eligible for Study:  


All




Accepts Healthy Volunteers:  


No




 



Criteria...





 



 
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Lacroix A, et al. Pituitary. 2019;doi:10.1007/s11102-019-01021-2.

January 7, 2020

Andre Lacroix




Most adults with persistent or recurrent Cushing’s disease treated with the somatostatin analogue pasireotide experienced a measurable decrease in MRI-detectable pituitary tumor volume at 12 months, according to findings from a post hoc analysis of a randomized controlled trial.

“Pasireotide injected twice daily during up to 12 months to control cortisol excess in patients with residual or persistent Cushing's disease was found to reduce the size of pituitary tumors in a high proportion of the 53 patients in which residual tumor was still visible at initiation of this medical therapy,” Andre Lacroix, MD, FCAHS, professor of medicine at the University of Montreal Teaching Hospital in Montreal, Canada, told Healio. “Pituitary tumors causing Cushing's syndrome which cannot be removed completely by surgery have the capacity to grow in time, and a medical therapy that can reduce tumor growth in addition to control excess cortisol production should be advantageous for the patients.”

 



Lacroix and colleagues analyzed data from 53 adults with persistent or recurrent Cushing’s disease, or those with newly diagnosed Cushing’s disease who were not surgical candidates, who had measurable tumor volume data (78% women). Researchers randomly assigned participants to 600 g or 900 g subcutaneous pasireotide (Signifor LAR, Novartis) twice daily. Tumor volume was assessed independently at 6 and 12 months by two masked radiologists and compared with baseline value and urinary free cortisol response.

 

Most adults with persistent or recurrent Cushing’s disease treated with the somatostatin analogue pasireotide experienced a measurable decrease in MRI-detectable pituitary tumor volume at 12 months.
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Sethi A, et al. Clin Endocrinol. 2019;doi:10.1111/CEN.14146.

January 5, 2020

Obesity is common at diagnosis of pituitary adenoma in childhood and may persist despite successful treatment, according to findings published in Clinical Endocrinology.

“The importance of childhood and adolescent obesity on noncommunicable disease in adult life is well recognized, and in this new cohort of patients, we report that obesity is common at presentation of pituitary adenoma in childhood and that successful treatment is not necessarily associated with weight loss,” Aashish Sethi, MD, MBBS, a pediatric endocrinologist in the department of endocrinology at Alder Hey Children’s Hospital in Liverpool, United Kingdom, and colleagues wrote. “We have reported obesity, and obesity-related morbidity in a mixed cohort of children and young adults previously, but [to] our knowledge, this is the first time this observation has been reported in a purely pediatric cohort.”

 



In a retrospective study, Sethi and colleagues analyzed clinical and radiological data from 24 white children from Alder Hey Children’s Hospital followed for a median of 3.3 years between 2000 and 2019 (17 girls; mean age at diagnosis, 15 years). Researchers assessed treatment modality (medical, surgical or radiation therapy), pituitary hormone deficiencies and BMI, as well as results of any genetic testing.

Within the cohort, 13 girls had prolactinomas (mean age, 15 years), including 10 macroadenomas between 11 mm and 35 mm in size. Children presented with menstrual disorders (91%), headache (46%), galactorrhea (46%) and obesity (38%). Nine children were treated with cabergoline alone, three also required surgery, and two were treated with the dopamine agonist cabergoline, surgery and radiotherapy.

Five children had Cushing’s disease (mean age, 14 years; two girls), including one macroadenoma. Those with Cushing’s disease presented with obesity (100%), short stature (60%) and headache (40%)....

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A diagnostic technique called bilateral inferior petrosal sinus sampling (BIPSS), which measures the levels of the adrenocorticotropic hormone (ACTH) produced by the pituitary gland, should only be used to diagnose cyclic Cushing’s syndrome patients during periods of cortisol excess, a case report shows.

When it is used during a spontaneous remission period of cycling Cushing’s syndrome, this kind of sampling can lead to false results, the researchers found.

The study, “A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome,” was published in BMC Endocrine Disorders.

Cushing’s syndrome is caused by abnormally high levels of the hormone cortisol. This is most often the result of a tumor on the pituitary gland that produces too much ACTH, which tells the adrenal glands to increase cortisol secretion.

However, the disease may also occur due to adrenal tumors or tumors elsewhere in the body that also produce excess ACTH — referred to as ectopic Cushing’s syndrome.

Because treatment strategies differ, doctors need to determine the root cause of the condition before deciding which treatment to choose.

BIPSS can be useful in this regard. It is considered a gold standard diagnostic tool to determine whether ACTH is being produced and released by the pituitary gland or by an ectopic tumor.

However, in people with cycling Cushing’s syndrome, this technique might not be foolproof.

Researchers reported the case of a 43-year-old woman who had rapidly cycling Cushing’s syndrome, meaning she had periods of excess cortisol with Cushing’s syndrome symptoms — low potassium, high blood pressure, and weight gain — followed by normal cortisol levels where symptoms resolved spontaneously.

In general, the length of each period can vary anywhere from a few hours to several months; in the case of this woman, they alternated relatively rapidly — over the course of weeks.




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Wed, Jan 8, 2020, from 4:00 PM - 5:00 PM EST

Presented by Paul Gardner, MD Associate Professor of Neurological Surgery Neurosurgical Director, Center for Cranial Base Surgery Executive Vice Chairman for Surgical Services University Pittsburgh Medical Center (UPMC)

Learning Objectives: Upon completion of this webinar, participants should be able to: Recognize the role for surgery in treating recurrent adenomas Understand the risk and role of radiosurgery for treatment of recurrent Identify treatment indications for recurrent adenomas.

Presenter Bio Paul A. Gardner, MD, is an Associate Professor in the Department of Neurological Surgery at the University of Pittsburgh School of Medicine and Neurosurgical Director of the Center for Cranial Base Surgery as well as Executive Vice Chairman for Surgical Services for the Department of Neurological Surgery at the University of Pittsburgh Medical Center (UPMC).

Dr. Gardner joined the faculty of the Department of Neurological Surgery at the University of Pittsburgh School of Medicine in 2008 after completing his residency and fellowship training at the University of Pittsburgh. He completed his undergraduate studies at Florida State University, majoring in biochemistry, and received his Medical Degree from the University of Pittsburgh School of Medicine.

Dr. Gardner completed a two-year fellowship in endoscopic endonasal pituitary and endoscopic and open skull base surgery at the University of Pittsburgh Medical Center. His research has focused on evaluating patient outcomes following these surgeries and more recently on molecular phenotyping of rare tumors. He is recognized internationally as a leader in the field of endoscopic endonasal surgery, a minimally invasive surgical approach to the skull base. His other surgical interests include pituitary tumors, open cranial base surgery, and vascular surgery.

Register here
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Approximately 20% of a cohort of adults with Cushing’s syndrome experienced at least one thrombotic event after undergoing pituitary or adrenal surgery, with the highest risk observed for those undergoing bilateral adrenalectomy, according to findings from a retrospective analysis published in the Journal of the Endocrine Society.

“We have previously showed in a recent meta-analysis that Cushing’s syndrome is associated with significantly increased venous thromboembolic events odds vs. the general population, though the risk is lower than in patients undergoing major orthopedic surgery,” Maria Fleseriu, MD, FACE, professor of neurological surgery and professor of medicine in the division of endocrinology, diabetes and clinical nutrition in the School of Medicine at Oregon Health & Science University and director of the OHSU Northwest Pituitary Center, told Healio. “However, patients undergoing many types of orthopedic surgeries have scheduled thromboprophylaxis, especially postsurgery, which is not the standard of care in patients with Cushing’s syndrome. In this study, we wanted to look in more detail at the rates of all thrombotic events, both arterial and venous, in patients at our specialized pituitary center over more than a decade.”

In a retrospective, longitudinal study, Fleseriu and colleagues analyzed data from 208 individuals with Cushing’s syndrome undergoing surgical (pituitary, unilateral and bilateral adrenalectomy) and medical treatment at a single center (79.3% women; mean age at presentation, 45 years; mean BMI, 33.9 kg/m²; 41.8% with diabetes). Individuals with severe illness and immediate mortality were excluded. Thromboembolic events (myocardial infarction, deep venous thrombosis [DVT], and pulmonary embolism or stroke) were recorded at any point up until last patient follow-up....
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Written by Kathleen Doheny with Maria Fleseriu, MD, FACE, and Vivien Herman-Bonert, MD

Cushing's disease, an uncommon but hard to treat endocrine disorder, occurs when a tumor on the pituitary gland, called an adenoma—that is almost always benign—leads to an overproduction of ACTH (adrenocorticotropic hormone), which is responsible for stimulating the release of cortisol, also known as the stress hormone.

Until now, surgery to remove the non-cancerous but problematic tumor has been the only effective treatment. Still, many patients will require medication to help control their serum cortisol levels, and others cannot have surgery or would prefer to avoid it.

Finally, a drug proves effective as added on or alternative to surgery in managing Cushing's disease. Photo; 123rf

New Drug Offers Alternative to Surgery for Cushing's Disease

Now, there is good news about long-term positive results achieved with pasireotide (Signifor)—the first medication to demonstrate effectiveness in both normalizing serum cortisol levels and either shrinking or slowing growth of tumors over the long term.1,2  These findings appear in the journal, Clinical Endocrinology, showing that patients followed for 36 months as part of an ongoing study had improved patient outcomes for Cushing’s disease.2
"What we knew before this extension study was—the drug will work in approximately half of the patients with mild Cushing's disease," says study author Maria Fleseriu, MD, FACE, director of the Northwest Pituitary Center and professor of neurological surgery and medicine in the division of endocrinology, diabetes and clinical nutrition at the Oregon Health and Sciences University School of Medicine.

“Pasireotide also offers good clinical benefits," says Dr. Fleseriu who is also the president of the Pituitary Society, “which includes improvements in blood pressure, other signs and symptoms of Cushing’s symptom], and quality of life.”2
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Please let us know if you see this endo and if he is helpful or not - thanks!

Murfreesboro Medical Clinic & SurgiCenter

272 Garrison Drive

Murfreesboro, TN 37129

615-893-4480

Murfreesboro Medical Clinic & SurgiCenter is committed to meeting the needs of Rutherford County's growing community. In addition to adding two new locations in 2019, MMC will be adding five new doctors to its team of physicians.


The physicians joining MMC this fall are: Christopher Albergo, M.D. (Endocrinology), Lauren Blackwell, D.O. (Pediatrics), C. Brad Bledsoe, M.D. (Dermatology), Britni Caplin, M.D. (ENT), and Brittany Cook, M.D. (Ophthalmology).


"With a national shortage of physicians, it is becoming more and more challenging to find quality physicians to meet the growing healthcare needs of our community," noted Joey Peay, MMC's Chief Executive Officer. "For MMC to find five quality physicians to join us in 2019 in addition to the nine that began practicing at MMC in 2018 is truly remarkable! Each of them will be a valuable member of our medical team and a wonderful member of the Murfreesboro community."


Christopher Albergo, M.D. is a board-certified Endocrinologist skilled in general endocrinology, including Hypothyroid, Parathyroid, Thyroid Cancer, Graves' Disease, Pituitary disorders, Adrenal disorders, Hypogonadism , PCOS, Obesity, Diabetes and Osteoporosis.


Read about all the new doctors here: https://www.wgnsradio.com/murfreesboro-medical-clinic-surgicenter-is-proud-to-announce-the-addition-of-five-new-physicians-to-its-practice-in-2019--cms-53464
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In patients with Cushing’s disease, removing the pituitary tumor via an endoscopic transsphenoidal surgery (TSS) leads to better remission rates than microscopic TSS, according to new research.

But regardless of surgical approach, plasma cortisol levels one day after surgery are predictive of remission, researchers found.

The study, “Management of Cushing’s disease: Changing trend from microscopic to endoscopic surgery,” was published in the journal World Neurosurgery.

Because it improves visualization and accessibility, endoscopic TSS has been gaining popularity over microscopic TSS to remove pituitary tumors in Cushing’s disease patients. Yet, although this surgery has been associated with high remission rates, whether it outperforms microscopic surgery and determining the factors affecting long-term outcomes may further ease disease recurrence after TSS.

A team with the All India Institute of Medical Sciences addressed this topic in 104 patients who underwent surgery from January 2009 to June 2017. Among these patients, 47 underwent microscopic surgery and 55 endoscopic surgery. At presentation, their ages ranged from 9 to 55 (mean age of 28). Also, patients had been experiencing Cushing’s symptoms over a mean duration of 24 months.

Eighty-seven patients showed weight gain. Hypertension (high blood pressure) and diabetes mellitus were among the most common co-morbidities, found in 76 and 33 patients, respectively. Nineteen patients had osteoporosis and 12 osteopenia, which refers to lower-than-normal bone mineral density.

As assessed with magnetic resonance imaging, 68 patients had a microadenoma (a tumor diameter smaller than one centimeter) and 27 had a macroadenoma (a tumor one centimeter or larger). Only two patients had an invasive pituitary adenoma.

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So do we need to get our bones checked too?

https://www.sciencealert.com/our-bones-provide-our-bodies-with-a-secret-weapon-that-saves-us-in-times-of-danger

Bizarre Discovery Shows Your Bones Could Be Triggering The 'Fight-or-Flight' Response

 




MIKE MCRAE



13 SEP 2019










When faced with a threat, hormones flood our bodies in preparation either for battle or a quick escape - what's commonly known as the 'fight-or-flight' response.

For decades, we've generally thought this response was driven by hormones such as adrenaline. But it now seems that one of the most important of these messengers could come from a rather unexpected place – our skeleton.

 



We usually think of chemicals like cortisol and adrenaline as the things that get the heart racing and muscles pumping. But the real star player could actually be osteocalcin, a calcium-binding protein produced by our bones.

As a response to acute stress, steroids of the glucocorticoid variety are released by the body's endocrine system, where they manage the production of a cascade of other 'get ready to rumble' chemicals throughout various tissues.

Researchers from the US, the UK, and India argue there's one tiny problem with this explanation of the fight-or-flight reaction. It isn't exactly fast.

While nobody is disputing that our bodies produce cortisol when stressed, the fact their main action is to trigger cells into transcribing specific genes – a process that takes time – makes it an unlikely candidate for a rapid physiological response.

"Although this certainly does not rule out that glucocorticoid hormones may be implicated in some capacity in the acute stress response, it suggests the possibility that other hormones, possibly peptide ones, could be involved," says geneticist Gerard Karsenty of Columbia University.

 


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Presented by

Andrew Lin, MD
Neuro-Oncologist & Neurologist
Memorial Sloak Kettering Cancer Center
 



After registering you will receive a confirmation email with details about joining the webinar.

Contact us at webinar@pituitary.org with any questions or suggestions.
 

Date: September 18, 2019 
Time: 10:00 AM - 11:00 AM. Pacific Daylight Time, 1:00 PM - 2:00 PM Eastern Daylight Time

Learning Objectives:

During the conversation I will be:
1) Defining aggressive pituitary tumors.
2) Reviewing the current treatment options for aggressive pituitary tumors.
3) Discussing experimental treatment options including a phase II trial investigating the activity of the immunotherapies nivolumab and ipilimumab.

Presenter Biography:

I am a neuro-oncologist at Memorial Sloan Kettering Cancer Center (MSK) and a member of the Multidisciplinary Pituitary & Skull Base Tumor Center. In collaboration with my colleagues in endocrine, neurosurgery, and radiation oncology, I treat patients with aggressive pituitary tumors, who are resistant to conventional treatments (i.e. surgery and radiation), with chemotherapy. With my colleagues at MSK, I have published several research articles on pituitary tumors and opened several clinical trials.
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For patients with persistent or recurring Cushing’s disease, monthly pasireotide therapy was safe and effective, leading to normal urinary free cortisol levels in 47% of patients after 2 years, according to findings published in Clinical Endocrinology.

Maria Fleseriu




“The management of Cushing’s syndrome, and particularly Cushing’s disease, remains challenging,” Maria Fleseriu, MD, FACE, professor of neurological surgery and professor of medicine in the division of endocrinology, diabetes and clinical nutrition in the School of Medicine at Oregon Health & Science University and director of the OHSU Northwest Pituitary Center, told Endocrine Today. “Long-acting pasireotide provided sustained biochemical improvements and clinical benefit in a significant proportion of patients with Cushing’s disease who elected to continue in this extension study. There were many adverse events reported overall, but no new safety signals emerging over long-term treatment.”

 



In the last decade, medical treatment for Cushing’s disease has progressed from a few steroidogenesis inhibitors to three novel drug groups: new inhibitors for steroidogenic enzymes with possibly fewer adverse effects, pituitary-directed drugs that aim to inhibit the pathophysiological pathways of Cushing’s disease, and glucocorticoid receptor antagonists that block cortisol’s action, Fleseriu, who is also an Endocrine Today Editorial Board member, said.
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Abstract

OBJECTIVE:

To report our management of bilateral adrenalectomy with autologous adrenal gland transplantation for persistent Cushing's disease, and to discuss the feasibility of autologous adrenal transplantation for the treatment of refractory Cushing's disease.

MATERIAL AND METHODS:

A retrospective analysis was performed in 4 patients (3 females, aged 14-36 years) who underwent autologous adrenal transplantation for persistent Cushing's disease after endonasal transsphenoidal resection of a pituitary tumor. The procedure was performed by implanting a vascularized adrenal graft into the left iliac fossa with direct and indirect anastomoses. Postoperative follow-up was performed in 1, 1.5, 8, and 10 years, and an over 8-year long-term follow-up was reached in 2 out of the 4 cases. Hormone replacement dosage was guided by clinical symptoms and endocrine results including serum cortisol (F), 24 h urine-free cortisol, and adrenocorticotrophic hormone levels.

RESULTS:

All 4 patients with symptomatic Cushing's disease experienced resolution of symptoms after autotransplantation without Nelson Syndrome. Functional autografts were confirmed through clinical evaluation and endocrine results. One year after transplantation, adrenal function and hormone replacement dosage remained stable without adrenal hyperplasia. After long-term follow-up, dosages of hormone replacement were reduced in all patients.

CONCLUSIONS:

In this series of 4 patients, we demonstrate the long-term efficacy of bilateral adrenalectomy with autologous adrenal transplantation and propose this procedure as a viable treatment option for refractory Cushing's disease.

© 2019 S. Karger AG, Basel.

KEYWORDS:

Cortisol; Adrenalectomy; Autologous adrenal gland transplantation ; Cushing’s disease; Nelson syndrome


PubMed http://www.ncbi.nlm.nih.gov/pubmed/31434089
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MENLO PARK, Calif., Aug. 28, 2019 (GLOBE NEWSWIRE) -- Corcept Therapeutics Incorporated (NASDAQ: CORT) announced today that the United States Patent and Trademark Office has issued a Notice of Allowance for a patent covering the administration of Korlym® with food.  The patent will expire in November 2032.

“This patent covers an important finding of our research – that for optimal effect, Korlym must be taken with food,” said Joseph K. Belanoff, MD, Corcept’s Chief Executive Officer. “Korlym’s label instructs doctors that ‘Korlym must always be taken with a meal.’”

Upon issuance, Corcept plans to list the patent, entitled “Optimizing Mifepristone Absorption” (U.S. Pat. App. 13/677,465), in the U.S. Food and Drug Administration’s Approved Drug Products with Therapeutic Equivalence Evaluations (the “Orange Book”).  Korlym is currently protected by ten patents listed in the Orange Book.

Hypercortisolism

Hypercortisolism, often referred to as Cushing’s syndrome, is caused by excessive activity of the hormone cortisol. Endogenous Cushing’s syndrome is an orphan disease that most often affects adults aged 20-50. In the United States, an estimated 20,000 patients have Cushing’s syndrome, with about 3,000 new patients diagnosed each year. Symptoms vary, but most people with Cushing’s syndrome experience one or more of the following manifestations: high blood sugar, diabetes, high blood pressure, upper-body obesity, rounded face, increased fat around the neck, thinning arms and legs, severe fatigue and weak muscles. Irritability, anxiety, cognitive disturbances and depression are also common. Hypercortisolism can affect every organ system in the body and can be lethal if not treated effectively.
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Levels of adrenocorticotropic hormone (ACTH) in circulation after pituitary surgery may help predict which Cushing’s disease patients will achieve early remission and which will eventually see the disease return, a study shows.

Also, the earlier that patients reached their lowest peak of ACTH levels, the better their long-term outcomes.

The study, “Prognostic usefulness of ACTH in the postoperative period of Cushing’s disease,” was published in the journal Endocrine Connections.

Removing the pituitary tumor through a minimally invasive surgery called transsphenoidal surgery is still the treatment of choice for Cushing’s disease patients. But not all patients enter remission, and even among those who do, a small proportion will experience disease recurrence.

While cortisol levels have been suggested as a main predictor of remission and recurrence, there is no consensus as to which cutoff point should be used after surgery, or the best time for measuring this hormone.

Because Cushing’s disease is caused by an ACTH-producing tumor in the pituitary gland, and ACTH has a short half-life (approximately 10 minutes), it is expected that ACTH levels drop markedly within a few hours after surgery.

Thus, a group of researchers in Spain aimed to determine whether blood levels of ACTH could be useful for predicting remission of Cushing’s disease both immediately after surgery (defined as less than 72 hours) and in the long term.

Researchers analyzed 65 patients with Cushing’s disease who had undergone transsphenoidal surgery (seven required a second intervention) between 2005 and 2016. Remission within three months was seen in 56 of 65 cases; late disease recurrence was seen in 18 of 58 cases.

Investigators measured the ACTH nadir concentration (defined as the lowest concentration) and the time taken to reach nadir levels after surgery, as well as the plasma ACTH concentration before hospital discharge.
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