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YOU’RE INVITED! GoodHormoneHealth Webinar on Lifestyle Medicine for Patients with Endocrine Problems With Eddie Ramirez, MD


Dr. Theodore Friedman (The Wiz) will be joined by Eddie Ramirez, MD and will discuss the 6 pillars of lifestyle medicine as applied to patients with Endocrine disorders:

Nutrition


Exercise


Sleep


Stress Reduction


Good Relationships


Avoiding Toxic Substances


 

Dr. Ramirez a medical doctor, research scientist, published author and international speaker.

 



Dr. Ramirez major research interest has been how food and lifestyle has the ability to prevent and reverse physical and mental disease.



 



This has resulted in over 160 research which he has shared in 88 countries. His work has been featured on NBC's Texoma, Icelandic National TV and the most popular health program in Ecuador. He is the author of Pandemic Busters and the co-author of the 3rd edition of the textbook of Lifestyle Medicine with thought leaders of the American College of Lifestyle Medicine and the book Rethink Food together with Neal Barnard, Michael Greger, Hans Diehl, Joel Furhman and other lifestyle medicine experts. 
Sunday • January 8, 2023  • 6 PM PST



Via Zoom Click here to join the meeting or
https://us02web.zoom.us/j/4209687343?pwd=amw4UzJLRDhBRXk1cS9ITU02V1pEQT09
OR
+16699006833,,4209687343#,,,,*111116#
Slides will be available on the day of the talk here. 


There will be plenty of time for questions using the chat button. 


For more information, email us at mail@goodhormonehealth.com
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Hi, 

I will post my bla journey here in hopes it may help others. 

Cliff notes: 4 mm microadenoma in my pituitary, 3 years testing because I am cyclical, ipss, pit sx Oct 13th 2021, remission for about 8 months then recurrence. No target on mris, all my tests back high. Now I am getting a bla. 

Met my surgeon yesterday. 

To be on the safe side, since Cushing's patients are at higher risk of deep vein thrombosis and possibly throwing a clot into my lungs known as a pulmonary embolism (PE) (This would be a a really bad thing) 
If for any reason the first adrenal gland is taking more than a few hours to remove, my surgeon will remove the second a month later. Because in this lovely face Down position & being a cushie, the risks of clots are higher the longer the surgery.  My surgeon said he has every intention to remove both adrenal glands in one operation but just wanted to let me know that this was a scenario that could happen in order to reduce risks. 

Since I am getting both glands removed, I will need to be in the hospital for 2-4 days to make absolutely sure that my cortisol meds are stable so I don't go into adrenal crisis and die ☠️. 
He said they will  do IV steroids at most likely 100mg or more if needed & after surgery taper accordingly and start me on oral hydrocortisone and monitor closely.

I felt very comfortable with his operation plan.  Holy crap this is happening.  (deep breath) 

 

To see my Cushings journey, it is all posted here: 

 
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The study covered in this summary was published on Research Square as a preprint and has not yet been peer reviewed.

Key Takeaways

Adding a corticotropin-releasing hormone (CRH) stimulation test immediately following a 2-day low-dose dexamethasone suppression test (LDDST) ― what's known as a Dex-CRH test and was first introduced in 1993 ― identified Cushing disease in 5 of 65 people (7.7%) with a confirmed diagnosis but who had previously shown normal cortisol levels on a conventional LDDST.

However, the Dex-CRH test also resulted in one (2.5%) false positive case compared with an LDDST alone.

Measuring serum dexamethasone levels further improved the diagnostic accuracy of the Dex-CRH test.

Why This Matters

It can be challenging to diagnose Cushing syndrome and to differentiate Cushing disease from nonneoplastic physiologic hypercortisolism caused by conditions that can present with Cushing syndrome–like clinical features, such as diabetes and obesity.

The Dex-CRH test, first described in 1993, initially appeared superior to an LDDST alone for ruling out nonneoplastic hypercortisolism, with a report of 100% sensitivity, specificity, and diagnostic accuracy. However, subsequent studies that used different protocols and in which dexamethasone was not measured had results that called into question the accuracy, sensitivity, and specificity of the Dex-CRH test.

This study reports the accuracy, sensitivity, and specificity of the Dex-CRH test for diagnosing Cushing disease, performed as first described, in 107 patients, including 74 for whom dexamethasone was also measured.
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Abstract

Introduction

Hypertension is one of the most common clinical features of patients with overt and subclinical hypercortisolism. Although previous studies have shown the coexistence of autonomous cortisol and aldosterone secretion, it is unclear whether aldosterone plays a role in hypertension among patients with hypercortisolism. Therefore, we examined the associations of plasma aldosterone concentrations (PACs) with hypertension among patients with overt and subclinical hypercortisolism.

Methods

This single-center retrospective cohort study included patients with adrenal tumor and serum cortisol levels after 1-mg dexamethasone suppression test >1.8 µg/dL (50 nmol/L). Using multivariable regression models adjusting for baseline characteristics, we investigated the association of PACs with systolic blood pressure and postoperative improvement of hypertension after the adrenalectomy.

Results

Among 89 patients enrolled in this study (median age, 51 years), 21 showed clinical signs of Cushing syndrome (overt hypercortisolism) and 68 did not show clinical presentations (subclinical hypercortisolism). We found that higher PACs were significantly associated with elevated systolic blood pressure among patients with subclinical hypercortisolism (adjusted difference [95% CI] = +0.59 [0.19-0.99], P = 0.008) but not among those with overt hypercortisolism. Among 33 patients with subclinical hypercortisolism and hypertension who underwent adrenalectomy, the postoperative improvement of hypertension was significantly associated with higher PACs at baseline (adjusted risk difference [95% CI] = +1.45% [0.35-2.55], P = 0.01).

Conclusion

These findings indicate that aldosterone may contribute to hypertension among patients with subclinical hypercortisolism. Further multi-institutional and population-based studies are required to validate our findings and examine the clinical effectiveness of the intervention targeting aldosterone for such patients.
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Abstract

Purpose: Transsphenoidal surgery is the first-line treatment for Cushing’s disease (CD), even with negative preoperative magnetic resonance imaging (MRI) results. Some patients with persistent or recurring hypercortisolism have negative MRI findings after the initial surgery. We aimed to analyze the efficacy of repeat surgery in two groups of patients and determine if there is an association between positive MRI findings and early remission.
Patients and Methods: Clinical, imaging, and biochemical information of 42 patients who underwent repeat surgery by a single neurosurgeon between 2002 and 2021 was retrospectively analyzed. We compared the endocrinological, histopathological, and surgical outcomes before and after repeat surgery among 14 CD patients with negative MRI findings and 28 patients with positive MRI findings.

Results: Immediate remission was achieved in 29 patients (69.0%) who underwent repeat surgery. Among all patients, 28 (66.7%) had MRI findings consistent with solid lesions. There was no significant difference in remission rates between the recurrence and persistence groups (77.8% vs. 57.1%, odds ratio = 2.625, 95% confidence interval = 0.651 to 10.586). Patients in remission after repeat surgery were not associated with positive MRI findings (odds ratio = 3.667, 95% confidence interval = 0.920 to 14.622).

Conclusions: In terms of recurrence, repeat surgery in patients with either positive or negative MRI findings showed reasonable remission rates. For persistent disease with positive MRI findings, repeat surgery is still an option; however, more solid evidence is needed to determine if negative MRI findings are predictors for failed reoperations for persistent hypercortisolism.



 




Keywords: 

Cushing’s disease; MRI; persistence; recurrence; repeat surgery
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Corcept Therapeutics (NASDAQ:CORT) announced an agreement with Hikma Pharmaceuticals (OTCPK:HKMPF) USA on Thursday to resolve the ongoing patent lawsuit related to Korlym, an oral therapy indicated for patients with Cushing’s syndrome.
 


The litigation was filed in the New Jersey district court in 2021, shortly after Hikma (OTCPK:HKMPF) informed Corcept (CORT) about its submission of an Abbreviated New Drug Application (ANDA) seeking FDA approval for a generic version of Korlym.
 


Per the terms of the settlement, Corcept (CORT) has allowed Hikma (OTCPK:HKMPF) the rights to market a generic version of Korlym from Oct. 01, 2034, or earlier subject to certain conditions.
 


The companies plan to submit the agreement for the review of the U.S. Federal Trade Commission (FTC) and the United States Department of Justice (DOJ).
 


A similar patent lawsuit filed by Corcept (CORT) against the U.S. unit of Teva Pharmaceutical (TEVA) remains pending.
 


Thanks mainly to higher sales volumes of Korlym, Corcept (CORT) added $366.0M net product revenue in 2021 with ~3% YoY growth.




 



From 

https://seekingalpha.com/news/3915421-cort-stock-on-watch-after-patent-settlement-with-hikma-for-korlym
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The study covered in this summary was published on researchsquare.com as a preprint and has not yet been peer reviewed.

Key Takeaways

The hypothalamic-pituitary-adrenal (HPA) axis recovered in nearly three quarters of patients with Cushing disease (CD) within 2 years after successful trans-sphenoidal surgery (TSS), with a median recovery time of 12 months.

Preoperative total triiodothyronine (TT3) level appears to be an independent predictor of central adrenal insufficiency (CAI) in CD patients with biochemical remission post surgery.

Why This Matters

Transient CAI typically occurs after successful TSS, requiring physiologic hydrocortisone replacement until HPA recovery.

Inadequate replacement may result in glucocorticoid withdrawal symptoms, including adrenal crisis, while overreplacement could lead to glucocorticoid side effects.

Findings have been inconsistent regarding recovery time in CD patients and factors predicting HPA axis recovery.

The new findings could help clinicians predict HPA axis-function recovery time and adjust cortisone replacement treatment in postoperative CD patients.
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12 Days of Cortisol Christmas Event
"We invite you to join us for our opening 12 Days of Cortisol Christmas event, “Overcoming Fatophobia” with Dr. Karen Thames.

Dr. Karen, a licensed clinical psychologist and founder of the EPIC Foundation, has battled Cushing’s disease and is now adrenal insufficient as a result. She has faced the battle of weight stigma both professionally and personally. In this interview, she bravely shares her story and advice on how to overcome #fatphobia in #chronicillness.

Adrenal Alternative’s 12 days of Cortisol Christmas is an event where we give back to the adrenal community during the Holiday Season.

Throughout the 12 days, we will be giving away adrenal awareness merch, hosting interviews with patients and professionals, and sharing helpful resources.

Even though patients must fight the war that is #adrenalinsufficiency, we want to let you know that you are supported by an army of adrenal warriors and you are not alone."

~Adrenal Alternatives Foundation


 
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Abstract

Cushing’s syndrome (CS) is a diagnosis used to describe multiple causes of serum hypercortisolism. Cushing’s disease (CD), the most common endogenous subtype of CS, is characterized by hypercortisolism due to a pituitary tumor secreting adrenocorticotropic hormone (ACTH). A variety of tests are used to diagnose and differentiate between CD and CS. Hypercortisolism has been found to cause many metabolic abnormalities including hypertension, hyperlipidemia, impaired glucose tolerance, and central adiposity. Literature shows that many of the symptoms of hypercortisolism can improve with a low carb (LC) diet, which consists of consuming <30 g of total carbohydrates per day. Here, we describe the case of a patient with CD who presented with obesity, hypertension, striae and bruising, who initially improved some of his symptoms by implementing a LC diet. Ultimately, as his symptoms persisted, a diagnosis of CD was made. It is imperative that practitioners realize that diseases typically associated with poor lifestyle choices, like obesity and hypertension, can often have alternative causes. The goal of this case report is to provide insight on the efficacy of nutrition, specifically a LC diet, on reducing metabolic derangements associated with CD. Additionally, we will discuss the importance of maintaining a high index of suspicion for CD, especially in those with resistant hypertension, obesity and pre-diabetes/diabetes.
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Abstract

Neuroendocrine carcinomas (NEC) of the cervix are a rare disease entity and account for only 1-2% of cervical carcinomas. The small-cell variant is the most common, with a worse prognosis and a higher rate of lymphatic and hematogenous metastases when compared with other subtypes of NEC. The diagnosis is usually made when the extra-pelvic disease is already apparent. Cushing’s syndrome due to adrenocorticotropic hormone (ACTH)-secreting tumors of the cervix is exceedingly rare. To date, there have been no reported cases in the literature of Cushing’s syndrome induced by the recurrence of metastases years after the initial diagnosis. This is a case of recurrent small-cell neuroendocrine carcinoma of the cervix presenting with Cushing’s syndrome five years after her original diagnosis. We present here the workup, management, and follow-up of this patient, including multisystemic, coordinated medical care.

Case Presentation

A 39-year-old female with a history of recurrent small-cell cervical cancer presented to the emergency department (ED) of our hospital with complaints of weight gain, generalized facial edema, lightheadedness, tingling sensation of her entire face, bilateral leg edema, and abdominal distention.

Her problems started a month prior to her ED visit, when she started to complain of abdominal distention. She had a computed tomography (CT) abdomen with contrast, which revealed evidence of metastatic disease, including multiple large liver lesions (Figure 1). Subsequently, she had a positron emission tomography (PET) scan, which confirmed the presence of hypermetabolic lesions in the right peritonsillar tissue, liver, right lower quadrant of the abdomen, and bilateral pulmonary nodules with lymphadenopathy in the left hilum (Figure 2).
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Objective: This extended evaluation (EE) of the SONICS study assessed effects of levoketoconazole for an additional 6 months following open-label, 6-month maintenance treatment in endogenous Cushing’s syndrome.

Design/Methods: SONICS included dose-titration (150–600 mg BID), 6-month maintenance, and 6-month EE phases. Exploratory efficacy assessments were performed at Months 9 and 12 (relative to start of maintenance). For pituitary MRI in patients with Cushing’s disease, a threshold of ≥2 mm denoted change from baseline in largest tumor diameter.

Results: Sixty patients entered EE at Month 6; 61% (33/54 with data) exhibited normal mean urinary free cortisol (mUFC). At Months 9 and 12, respectively, 55% (27/49) and 41% (18/44) of patients with data had normal mUFC. Mean fasting glucose, total and LDL-cholesterol, body weight, body mass index, abdominal girth, hirsutism, CushingQoL, and BDI-II scores improved from study baseline at Months 9 and 12. Forty-six patients completed Month 12; 4 (6.7%) discontinued during EE due to adverse events. The most common adverse events in EE were arthralgia, headache, hypokalemia, and QT prolongation (6.7% each). No patient experienced ALT or AST >3× ULN, QTcF interval >460 msec, or adrenal insufficiency during EE. Of 31 patients with tumor measurements at baseline and Month 12 or follow-up, largest tumor diameter was stable in 27 (87%) patients, decreased in 1, and increased in 3 (largest increase 4 mm).

Conclusion: In the first long-term levoketoconazole study, continued treatment through 12-month maintenance period sustained the early clinical and biochemical benefits in most patients completing EE, without new adverse effects.

Read the whole article at https://eje.bioscientifica.com/configurable/content/journals$002feje$002faop$002feje-22-0506$002feje-22-0506.xml?t%3Aac=journals%24002feje%24002faop%24002feje-22-0506%24002feje-22-0506.xml&body=pdf-45566
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Abstract

(1) Background: Cushing’s disease (CD) is a serious endocrine disorder caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary neuroendocrine tumor (PitNET) that stimulates the adrenal glands to overproduce cortisol. Chronic exposure to excess cortisol has detrimental effects on health, including increased stroke rates, diabetes, obesity, cognitive impairment, anxiety, depression, and death. The first-line treatment for CD is pituitary surgery. Current surgical remission rates reported in only 56% of patients depending on several criteria. The lack of specificity, poor tolerability, and low efficacy of the subsequent second-line medical therapies make CD a medical therapeutic challenge. One major limitation that hinders the development of specific medical therapies is the lack of relevant human model systems that recapitulate the cellular composition of PitNET microenvironment. (2) Methods: human pituitary tumor tissue was harvested during transsphenoidal surgery from CD patients to generate organoids (hPITOs). (3) Results: hPITOs generated from corticotroph, lactotroph, gonadotroph, and somatotroph tumors exhibited morphological diversity among the organoid lines between individual patients and amongst subtypes. The similarity in cell lineages between the organoid line and the patient’s tumor was validated by comparing the neuropathology report to the expression pattern of PitNET specific markers, using spectral flow cytometry and exome sequencing. A high-throughput drug screen demonstrated patient-specific drug responses of hPITOs amongst each tumor subtype. Generation of induced pluripotent stem cells (iPSCs) from a CD patient carrying germline mutation CDH23 exhibited dysregulated cell lineage commitment. (4) Conclusions: The human pituitary neuroendocrine tumor organoids represent a novel approach in how we model complex pathologies in CD patients, which will enable effective personalized medicine for these patients.
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Objective: The first-line treatment for Cushing’s disease is transsphenoidal surgery, after which the rates of remission are 60 to 80%, with long-term recurrence of 20 to 30%, even in those with real initial remission. Drug therapies are indicated for patients without initial remission or with surgical contraindications or recurrence, and ketoconazole is one of the main available therapies. The objective of this study was to evaluate the safety profile of and the treatment response to ketoconazole in Cushing’s disease patients followed up at the endocrinology outpatient clinic of a Brazilian university hospital.

Patients and methods: This was a retrospective cohort of Cushing’s disease patients with active hypercortisolism who used ketoconazole at any stage of follow-up. Patients who were followed up for less than 7 days, who did not adhere to treatment, or who were lost to follow-up were excluded.

Results: Of the 172 Cushing’s disease patients who were followed up between 2004 and 2020, 38 received ketoconazole.
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A prolactin (PRL) test measures how much of a hormone called prolactin you have in your blood. The hormone is made in your pituitary gland, which is located just below your brain.

When women are pregnant or have just given birth, their prolactin levels increase so they can make breast milk. But it’s possible to have high prolactin levels if you’re not pregnant, and even if you’re a man.

Your doctor may order a prolactin test when you report having the following symptoms:

For women

Irregular or no periods


Infertility


Breast milk discharge when you’re not pregnant or nursing


Tenderness in your breast


Menopausal symptoms such as hot flashes and vaginal dryness


For men

Decreased sex drive


Difficulty in getting an erection


Breast tenderness or enlargement


Breast milk production (very rare)


For both

Unexplained headaches


Vision problems


Causes of Abnormal Prolactin Levels
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What Is the Role of “Growth Hormone” When You Have Stopped Growing?

Growth hormone clearly plays a key role in development during youth, but research in adults implicates it as an agent in cellular aging processes. Shlomo Melmed, MD, ChB, the first recipient of the Transatlantic Alliance Award, co-sponsored by the Endocrine Society and the European Society of Endocrinology, discusses the misconceptions of administering growth hormone in adults.

Children need growth hormone to grow into their adult height, but the hormone’s function among adults is unclear. The pituitary secretes less growth hormone as a person ages, but new research is elucidating a potentially important role for nonpituitary growth hormone generated in the periphery in regulating cellular proliferation associated with aging.

AT A GLANCE


Growth hormone levels decline with age — which may be a protective mechanism in slowing some of the effects of aging.


Nonpituitary growth hormone in the colon epithelium has been shown to inhibit the tumor suppressor gene p53, resulting in pro-proliferative effects.


Low levels of growth hormone in adulthood appear to be associated with greater longevity, whereas higher levels are associated with the adverse effects of aging.


Unraveling the effects of this mysterious hormone has been a focus of the work of Shlomo Melmed, MB ChB, dean of the faculty of medicine at Cedars-Sinai in Los Angeles. Melmed is the inaugural winner of the Transatlantic Alliance Award, an honor co-sponsored by the Endocrine Society and the European Society of Endocrinology to recognize an international leader who has made significant advancements in endocrine research on both sides of the Atlantic. As part of the award, Melmed gave a presentation at both ENDO 2022 in Atlanta in June, and at the European Congress of Endocrinology 2022 in Milan entitled, “Growth Hormone: An Adult Endocrine Misnomer?”
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Brief Summary:



This is a randomized, placebo-controlled, crossover study of SPI-62 in subjects with ACTH-dependent Cushing's syndrome. Subjects will receive each of the following 2 treatments for 12 weeks: SPI-62 and matching placebo

 


Condition or disease 


Intervention/treatment 


Phase 


Cushing's Syndrome ICushing Disease Due to Increased ACTH Secretion Cortisol ExcessCortisol; Hypersecretion Cortisol Overproduction Ectopic ACTH Secretion


Drug: SPI-62 Drug: Placebo


Phase 2


Detailed Description:



This is a multicenter, randomized, placebo-controlled, Phase 2 study to evaluate the pharmacologic effect, efficacy, and safety of SPI-62 in subjects with ACTH-dependent Cushing's syndrome. Each subject who provides consent and meets all inclusion and exclusion criteria will participate in 3 periods: a 28-day screening period (Days -35 to -8), a 7-day baseline period (Days -7 to -1), and a 24-week treatment period (Day 1 of Week 1 to Day 168 ± 3 days of Week 24). Up to 26 subjects will be enrolled with the aim that 18 subjects with Cushing's disease will complete the study. Subjects will receive each of the following 2 treatments for 12 weeks: SPI-62 and matching placebo.






Study Design



 



Go to  




 

Study Type  :


Interventional  (Clinical Trial)


Estimated Enrollment  :


26 participants


Allocation:


Randomized


Intervention Model:


Crossover Assignment


Intervention Model Description:


Staggered parallel crossover


Masking:


Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor)


Primary Purpose:


Treatment


Official Title:


SPI-62 as a Treatment for Adrenocorticotropic Hormone-dependent Cushing's Syndrome


Actual Study Start Date  :


March 1, 2022


Estimated Primary Completion Date  :


March 15, 2023


Estimated Study Completion Date  :


August 15, 2023


 

More info at https://clinicaltrials.gov/ct2/show/record/NCT05307328
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Recordati's Isturisa is expected to launch in the second or third quarter.

As part of a small 2019 deal, Italian drugmaker Recordati snagged a trio of underperforming Novartis endocrinology meds, including a late-stage candidate for Cushing's disease. Less than a year later, that drug is cleared for market after an FDA green light. 

The FDA on Friday approved Recordati's Isturisa (osilodrostat) to treat Cushing's disease—a rare disease in which patients' adrenal glands produce too much cortisol—in those who have undergone a prior pituitary gland surgery or are not eligible for one.

Isturisa, a cortisol synthesis inhibitor, will come with the FDA's orphan drug designation, providing market exclusivity for seven years, Recordati said (PDF) in a release. The drug is expected to be commercially available in the second or third quarter. 
The FDA based its review on phase 3 data showing 86% of patients treated with Isturisa showed normal cortisol levels in their urine after eight weeks, compared with 29% of patients treated with placebo, the drugmaker said. 

Recordati is "actively building its commercial, medical, and market access teams" to accommodate Isturisa's launch through its recently created U.S. endocrinology business unit, it said. The drugmaker will launch the drug with a "comprehensive distribution model" through specialty pharmacies. 

Novartis, once the owner of Isturisa, turned the asset over to Recordati in 2019 as part of a $390 million offload of some of the Swiss drugmaker's endocrinology portfolio. 

Recordati received Signifor, long-acting sister Signifor LAR and Isturisa, positioned as a successor drug to Signifor. The purchase included milestone payments tied to Isturisa.

Recordati talked up the buy of the Cushing's disease trio as a boon for its rare disease portfolio, calling it a "key and historical milestone" at the time. 

 
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Abstract

Corticotroph macroadenomas are rare but difficult to manage intracranial neoplasms. Mutations in the two Cushing’s disease mutational hotspots USP8 and USP48 are less frequent in corticotroph macroadenomas and invasive tumors. There is evidence that TP53 mutations are not as rare as previously thought in these tumors. The aim of this study was to determine the prevalence of TP53 mutations in corticotroph tumors, with emphasis on macroadenomas, and their possible association with clinical and tumor characteristics. To this end, the entire TP53 coding region was sequenced in 86 functional corticotroph tumors (61 USP8 wild type; 66 macroadenomas) and the clinical characteristics of patients with TP53 mutant tumors were compared with TP53/USP8 wild type and USP8 mutant tumors. We found pathogenic TP53 variants in 9 corticotroph tumors (all macroadenomas and USP8 wild type). TP53 mutant tumors represented 14% of all functional corticotroph macroadenomas and 24% of all invasive tumors, were significantly larger and invasive, and had higher Ki67 indices and Knosp grades compared to wild type tumors. Patients with TP53 mutant tumors had undergone more therapeutic interventions, including radiation and bilateral adrenalectomy. In conclusion, pathogenic TP53 variants are more frequent than expected, representing a relevant amount of functional corticotroph macroadenomas and invasive tumors. TP53 mutations associated with more aggressive tumor features and difficult to manage disease.
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Patients with suspected adrenocortical carcinoma (ACC) may also undergo additional tests to identify excess sex steroids and steroid precursors, mineralocorticoids and glucocorticoids.

Table 2 provides a summary of the tests recommended by the European Society of Endocrinology in collaboration with the European Network for the Study of Adrenal Tumors in patients with suspected ACC.10
 


Table 2. Diagnostic work-up in patients with suspected or proven adrenocortical carcinoma. Reproduced from Fassnacht et al., 2018,10 with permission under a Creative Commons Attribution 4.0 International License.







ACTH: adrenocorticotropic hormone; DHEA-S: dehydroepiandrosterone sulphate; CT: computed tomography; FDG-PET: 18F-fluorodeoxyglucose positron emission tomography; MRI: magnetic resonance imaging.







Click image to enlarge
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More than 80% of adults with Cushing’s disease receiving osilodrostat had normalized mean urinary free cortisol levels at 72 weeks of treatment, according to findings from the LINC 3 study extension.

“Cushing’s disease is a chronic condition, and many patients require prolonged pharmacological treatment. Therefore, evaluating long-term efficacy and safety of drug therapies in clinical trials is essential,” Maria Fleseriu, MD, FACE, professor of medicine and neurological surgery and director of the Pituitary Center at Oregon Health & Science University in Portland and a Healio | Endocrine Today co-editor, told Healio. “Our findings build on the positive results of the LINC 3 study core phase, and it was reassuring to see that continued treatment with osilodrostat for over 72 weeks provided long-term normalization of cortisol levels. Furthermore, continued treatment with osilodrostat also led to sustained improvements in clinical signs and physical manifestations of hypercortisolism, as well as health-related quality of life, which are all important factors in the management of these patients.”

Fleseriu and colleagues enrolled 106 adults with Cushing’s disease who were responders to osilodrostat (Isturisa, Recordati) at 48 weeks during the LINC 3 core study to enter the extension phase of the trial. Participants continued to receive open-label osilodrostat until 72 weeks or treatment discontinuation. Mean urinary free cortisol was collected every 12 weeks. Physical manifestations of hypercortisolism were rated at 48 and 72 weeks. Participants completed the Cushing’s Quality of Life questionnaire and Beck Depression Inventory II at 48 and 72 weeks. Adults were deemed to have completely responded to treatment if mean urinary free cortisol was less than the upper limit of normal and partially responded to treatment if mean urinary free cortisol was above the upper limit of normal but decreased more than 50% from baseline.
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Pseudo-Cushing’s syndrome is when symptoms are similar to those of Cushing’s syndrome, but further tests show that the syndrome is not present.

Common causes include consuming too much alcohol, obesity, persistently high blood glucose levels, pregnancy, and depression.

 
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Cortisol isn’t bad; you need it to help regulate your responses to life. Regulation involves a very complex interplay of feedback loops between the hypothalamus, pituitary gland, and adrenal glands, says Dr. Singh.

“In general, cortisol levels tend to peak in the late morning and gradually decline throughout the day,” he explains. “When a stressful event occurs, the increased cortisol will work alongside our ‘fight or flight’ mechanisms to either upregulate or downregulate bodily functions. [Affected systems include] the central nervous system, cardiovascular system, gastrointestinal system, or immune system.”

In addition to normal processes that trigger or suppress cortisol release, levels can also be affected by different medical conditions, Dr. Singh says. For example, if someone has abnormally high levels of cortisol, this is called Cushing’s syndrome, which is typically caused by a tumor affecting any of the glands that take part in the process of cortisol production.

When people suffer from abnormally low levels of cortisol, it’s called Addison’s disease. It generally occurs due to adrenal gland dysfunction, but could also be the result of abnormal functioning of any of the other glands in the cortisol production process.

Finally, if you use corticosteroid medications such as prednisone or dexamethasone, prolonged use will result in excessive cortisol production, Dr. Singh says.

“If the medication is not adequately tapered down when discontinued, the body’s ability to create cortisol can become permanently impaired,” he says.

From https://www.yahoo.com/lifestyle/manage-pesky-stress-hormone-cortisol-184900397.html
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Abstract

Background

Cushing’s syndrome (CS) is a rare condition of chronically elevated cortisol levels resulting in diverse comorbidities, many of which endure beyond successful treatment affecting the quality of life. Few data are available concerning patients’ experiences of diagnosis, care and persistent comorbidities.

Objective

To assess CS patients’ perspectives on the diagnostic and care journey to identify unmet therapeutic needs.

Methods

A 12-item questionnaire was circulated in 2019 by the World Association for Pituitary Organisations. A parallel, 13-item questionnaire assessing physician perceptions on CS patient experiences was performed.

Results

Three hundred twenty CS patients from 30 countries completed the questionnaire; 54% were aged 35–54 and 88% were female; 41% were in disease remission. The most burdensome symptom was obesity/weight gain (75%). For 49% of patients, time to diagnosis was over 2 years. Following treatment, 88.4% of patients reported ongoing symptoms including, fatigue (66.3%), muscle weakness (48.8%) and obesity/weight gain (41.9%). Comparisons with delay in diagnosis were significant for weight gain (P = 0.008) and decreased libido (P = 0.03). Forty physicians completed the parallel questionnaire which showed that generally, physicians poorly estimated the prevalence of comorbidities, particularly initial and persistent cognitive impairment. Only a minority of persistent comorbidities (occurrence in 1.3–66.3%; specialist treatment in 1.3–29.4%) were managed by specialists other than endocrinologists. 63% of patients were satisfied with treatment.

Conclusion

This study confirms the delay in diagnosing CS. The high prevalence of persistent comorbidities following remission and differences in perceptions of health between patients and physicians highlight a probable deficiency in effective multidisciplinary management for CS comorbidities.
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Abstract

Background. Cushing’s disease (CD) recurrence in pregnancy is thought to be associated with estradiol fluctuations during gestation. CD recurrence in the immediate postpartum period in a patient with a documented dormant disease during pregnancy has never been reported. Case Report. A 30-year-old woman with CD had improvement of her symptoms after transsphenoidal resection (TSA) of her pituitary lesion. She conceived unexpectedly 3 months postsurgery and had no symptoms or biochemical evidence of recurrence during pregnancy. After delivering a healthy boy, she developed CD 4 weeks postpartum and underwent a repeat TSA. Despite repeat TSA, she continued to have elevated cortisol levels that were not well controlled with medical management. She eventually had a bilateral adrenalectomy. Discussion. CD recurrence may be higher in the peripartum period, but the link between pregnancy and CD recurrence and/or persistence is not well studied. Potential mechanisms of CD recurrence in the postpartum period are discussed below. Conclusion. We describe the first report of recurrent CD that was quiescent during pregnancy and diagnosed in the immediate postpartum period. Understanding the risk and mechanisms of CD recurrence in pregnancy allows us to counsel these otherwise healthy, reproductive-age women in the context of additional family planning.
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Abstract: Ectopic pituitary adenoma (EPA) is a pituitary adenoma unrelated to the intrasellar component and is an extremely rare disease. EPA resembles typical pituitary adenomas in morphology, immunohistochemistry, and hormonal activity, and it may present with specific or non-specific endocrine manifestations. Here, we report a rare case of ectopic adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma in the clival region. Only three patients with ACTH-secreting pituitary adenomas occurring in the clivus have been previously reported, and the present case was diagnosed as a clivus-ectopic ACTH-secreting pituitary macroadenoma. Thus, in addition to the more common organs, such as the lung, thymus, and pancreas, in the diagnosis of ectopic ACTH syndrome, special attention should be paid to the extremely rare ectopic ACTH-secreting pituitary adenoma of the clivus region.
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