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The occurrence of different subtypes of endogenous Cushing’s syndrome (CS) in single individuals is extremely rare. We here present the case of a female patient who was successfully cured from adrenal CS 4 years before being diagnosed with Cushing’s disease (CD). The patient was diagnosed at the age of 50 with ACTH-independent CS and a left-sided adrenal adenoma, in January 2015. After adrenalectomy and histopathological confirmation of a cortisol-producing adrenocortical adenoma, biochemical hypercortisolism and clinical symptoms significantly improved. However, starting from 2018, the patient again developed signs and symptoms of recurrent CS. Subsequent biochemical and radiological workup suggested the presence of ACTH-dependent CS along with a pituitary microadenoma. The patient underwent successful transsphenoidal adenomectomy, and both postoperative adrenal insufficiency and histopathological workup confirmed the diagnosis of CD. Exome sequencing excluded a causative germline mutation but showed somatic mutations of the β-catenin protein gene (CTNNB1) in the adrenal adenoma, and of both the ubiquitin specific peptidase 8 (USP8) and the glucocorticoid receptor (NR3C1) genes in the pituitary adenoma. In conclusion, our case illustrates that both ACTH-independent and ACTH-dependent CS may develop in a single individual even without evidence for a common genetic background. Introduction Endogenous Cushing´s syndrome (CS) is a rare disorder with an incidence of 0.2–5.0 per million people per year (1, 2). The predominant subtype (accounting for about 80%) is adrenocorticotropic hormone (ACTH)-dependent CS. The vast majority of this subtype is due to an ACTH-secreting pituitary adenoma [so called Cushing´s disease (CD)], whereas ectopic ACTH-secretion (e.g. through pulmonary carcinoids) is much less common. In contrast, ACTH-independent CS can mainly be attributed to cortisol-producing adrenal adenomas. Adrenocortical carcinomas, uni-/bilateral adrenal hyperplasia, and primary pigmented nodular adrenocortical disease (PPNAD) may account for some of these cases as well (3, 4). Coexistence of different subtypes of endogenous CS in single individuals is even rarer but has been described in few reports. These cases were usually observed in the context of prolonged ACTH stimulation on the adrenal glands, resulting in micronodular or macronodular hyperplasia (5–9). A sequence of CD and PPNAD was also described in presence of Carney complex, a genetic syndrome characterized by the loss of function of the gene encoding for the regulatory subunit type 1α of protein kinase A (PRKAR1A) (10). Moreover, another group reported the case of a patient with Cushing's disease followed by ectopic Cushing's syndrome more than 30 years later (8). To our knowledge, however, we here describe the first case report on a single patient with a cortisol-producing adrenocortical adenoma and subsequent CD. Read the rest of the article at https://www.frontiersin.org/articles/10.3389/fendo.2021.731579/full -
This article was originally published here J Clin Endocrinol Metab. 2021 Jul 29:dgab557. doi: 10.1210/clinem/dgab557. Online ahead of print. ABSTRACT CONTEXT: Coronavirus disease 2019 (COVID-19) is a proinflammatory and prothrombotic condition, but its impact on adrenal function has not been adequately evaluated. CASE REPORT: A 46-year-old woman presented with abdominal pain, hypotension, and skin hyperpigmentation after COVID-19 infection. The patient had hyponatremia, serum cortisol <1.0 µg/dL, adrenocorticotropin (ACTH) of 807 pg/mL, and aldosterone ❤️ ng/dL. Computed tomography (CT) findings of adrenal enlargement with no parenchymal and minimal peripheral capsular enhancement after contrast were consistent with bilateral adrenal infarction. The patient had autoimmune hepatitis and positive antiphospholipid antibodies, but no previous thrombotic events. The patient was treated with intravenous hydrocortisone, followed by oral hydrocortisone and fludrocortisone. DISCUSSION: We identified 9 articles, including case reports, of new-onset adrenal insufficiency and/or adrenal hemorrhage/infarction on CT in COVID-19. Adrenal insufficiency was hormonally diagnosed in 5 cases, but ACTH levels were measured in only 3 cases (high in 1 case and normal/low in other 2 cases). Bilateral adrenal nonhemorrhagic or hemorrhagic infarction was identified in 5 reports (2 had adrenal insufficiency, 2 had normal cortisol levels, and 1 case had no data). Interestingly, the only case with well-characterized new-onset acute primary adrenal insufficiency after COVID-19 had a previous diagnosis of antiphospholipid syndrome. In our case, antiphospholipid syndrome diagnosis was established only after the adrenal infarction triggered by COVID-19. CONCLUSION: Our findings support the association between bilateral adrenal infarction and antiphospholipid syndrome triggered by COVID-19. Therefore, patients with positive antiphospholipid antibodies should be closely monitored for symptoms or signs of acute adrenal insufficiency during COVID-19. PMID:34463766 | DOI:10.1210/clinem/dgab557
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With the goal of reducing false positives for adrenal insufficiency (AI), scientists are recommending a new, more precise diagnostic cutoff of 14-15 μg/dL of serum cortisol, rather than the current 18 μg/dL. The new data were published in the Journal of the Endocrine Society. Among the 110 patients evaluated in the retrospective analysis, new cortisol cutoffs after adrenocorticotropic hormone (ACTH) stimulation were identified when using several of the newer, more widely used diagnostic assays currently available, including Elecsys II (14.6 μg/dL), Access (14.8 μg/dL), and liquid chromatography-tandem mass spectrometry (LC-MS/MS) (14.5 μg/dL). Bradley Javorsky, MD, an endocrinologist and researcher at the Medical College of Wisconsin, served as the study's first author. He recently discussed the findings with MedPage Today. The exchange has been edited for length and clarity. What was the key knowledge gap your study was designed to address? Javorsky: It is safe to say that most clinicians, including many endocrinologists -- not to mention practice guidelines and clinical information resources -- still regard 18 μg/dL as the cutoff for making the biochemical diagnosis of AI after ACTH stimulation testing. However, this cutoff was derived from older polyclonal immunoassays that are no longer being used in many institutions. Newer, more specific monoclonal immunoassays and LC-MS/MS are being used instead. With these more specific assays, one might expect the cutoffs to be lower. What was your finding? Javorsky: After ACTH stimulation, the cutoff values for the newer, more specific cortisol assays were indeed lower at 14-15 μg/dL. Although there was excellent correlation between the new and older assays, the results from the new assays were 22-39% lower than those found by the older and less-specific Elecsys I assay, hence the lowered threshold. Did anything surprise you about the study results? Javorsky: Baseline cortisol had to be very low (approximately <2 μg/dL) in order to be predictive of subnormal cortisol values. This underscores the observation that ACTH stimulation testing is not perfectly sensitive. What are the clinical takeaways from these results? Javorsky: To avoid false-positive ACTH stimulation testing results -- and by extension avoid over-treating patients with glucocorticoids -- clinicians should be aware of the cortisol assay used in their institution and the new cortisol cutoff when evaluating patients for adrenal insufficiency. It should also be reinforced that careful interpretation in the context of clinical history is still essential to making the correct diagnosis. Discordant results among different assays underscore the importance of clinical judgment from an experienced physician when diagnosing AI. What are the takeaways? Javorsky: I think it is important that laboratories make the type of cortisol assay used in their institution easily accessible to clinicians and strongly consider posting the new cortisol cutoff after ACTH stimulation testing when reporting results. Read the study here and expert commentary on the clinical implications here. Disclosures Javorsky reported being a consultant for Clarus Therapeutics and a research investigator for Novartis Pharmaceuticals. Primary Source Journal of the Endocrine Society Source Reference: Javorsky BR, et al "New cutoffs for the biochemical diagnosis of adrenal insufficiency after ACTH stimulation using specific cortisol assays" J Endocrine Soc 2021; 5(4): bvab022. From https://www.medpagetoday.com/endocrine-society/adrenal-disorders/93188
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DEER PARK, Ill., June 15, 2021 (GLOBE NEWSWIRE) -- Eton Pharmaceuticals, Inc (Nasdaq: ETON), the U.S. marketer of ALKINDI SPRINKLE®, a treatment for adrenocortical insufficiency in pediatric patients, today announced that it has acquired U.S. and Canadian rights to Crossject’s ZENEO® hydrocortisone needleless autoinjector, which is under development as a rescue treatment for adrenal crisis. “The ZENEO autoinjector is a revolutionary delivery system, and this product is a terrific strategic fit with our current adrenal insufficiency business. Patients, advocacy groups, and physicians in the adrenal insufficiency community have repeatedly expressed to us the need for a hydrocortisone autoinjector, so we are excited to be partnering with Crossject to bring this product to patients in need,” said Sean Brynjelsen, CEO of Eton Pharmaceuticals. Patrick Alexandre, CEO of Crossject, added: ‘‘We are proud to announce a sound commercial agreement for ZENEO® Hydrocortisone in the US and Canada with an American leader in adrenal insufficiency. ETON has successfully established strong relations with the patient communities and medical specialists that are its core focus. ZENEO® Hydrocortisone answers a medical need. This strong partnership will contribute to saving lives by bringing to patients and their families a modern autoinjection possibility.’’ “We are delighted about Eton Pharmaceuticals' plans to partner with Crossject to bring this incredibly needed product to patients in the U.S.”, said Dina Matos, Executive Director of CARES Foundation, a leading North American advocacy foundation for patients with congenital adrenal hyperplasia, the most common presentation of adrenal insufficiencies in children. “The challenge for patients and caregivers facing an adrenal crisis is serious; an easy-to-use needleless autoinjector of hydrocortisone will be a game changer for our patients. We welcome this advancement.” ZENEO® is a proprietary needleless device developed and manufactured by Crossject. The pre-filled, single-use device propels medication through the skin in less than a tenth of a second. The device’s compact form factor, simple two-step administration, and needle-free technology make it an ideal delivery system for emergency medications that need to be administered in stressful situations by non-healthcare professionals. Crossject holds more than 400 global patents on the device, including 24 issued in the United States that extend as far as 2037, and has successfully completed bioequivalence and human factor studies with the ZENEO device using various medications. Crossject has developed a proprietary, room-temperature stable liquid formulation of hydrocortisone to be delivered via the ZENEO device. ZENEO hydrocortisone is expected to be the first and only hydrocortisone autoinjector available for patients that require a rescue dose of hydrocortisone. Currently, injectable hydrocortisone is only available in the United States in a lyophilized powder formulation that must be reconstituted and manually delivered via a traditional syringe. Eton expects to submit a New Drug Application for the product to the U.S. Food and Drug Administration in 2023 and plans to request Orphan Drug Designation. In the United States, it is estimated that approximately 100,000 patients currently suffer from adrenocortical insufficiency and are at risk for adrenal crisis. Under the terms of the agreement, Crossject will receive development and regulatory milestone payments from Eton of up to $5.0 million, commercial milestones of up to $6.0 million, and a 10% royalty on net sales of the product. Crossject will be responsible for the management and expense of development, clinical, and manufacturing activities. Eton will be responsible for all regulatory and commercial activities. About Adrenal Crisis Patients with adrenal insufficiency can go into adrenal crisis if their cortisol levels are too low. Adrenal crisis is typically caused by missed doses of maintenance hydrocortisone, trauma, surgery, illness, fever, or major psychological distress. Signs of adrenal crisis include hyperpigmentation, severe weakness, nausea, abdominal pain, and confusion. It is estimated that approximately 8% of adrenal insufficiency patients will report an adrenal crisis in any given year and more than 6% of cases result in death. About Crossject Crossject (ISIN: FR0011716265; Ticker: ALCJ; LEI: 969500W1VTFNL2D85A65) is developing and is soon to market a portfolio of drugs dedicated to emergency situations: epilepsy, overdose, allergic shock, severe migraine and asthma attack. The company’s portfolio currently contains eight products in advanced stages of development, including 7 emergency treatments, 5 of which are intended for life-threatening situations. Thanks to its patented needle-free self-injection system, Crossject aims to become the world leader in self-administered emergency drugs. The company has been listed on the Euronext Growth market in Paris since 2014, and benefits from Bpifrance funding. About Eton Pharmaceuticals Eton Pharmaceuticals, Inc. is an innovative pharmaceutical company focused on developing and commercializing treatments for rare diseases. The company currently owns or receives royalties from three FDA-approved products, including ALKINDI® SPRINKLE, Biorphen®, and Alaway Preservative Free®, and has six additional products that have been submitted to the FDA. Company Contact: David Krempa dkrempa@etonpharma.com 612-387-3740 From https://www.globenewswire.com/news-release/2021/06/15/2247745/0/en/Eton-Pharmaceuticals-Acquires-U-S-and-Canadian-Rights-to-ZENEO-Hydrocortisone-Autoinjector.html
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— Gradual dose escalation had fewer adverse events, same therapeutic benefit, as quicker increases by Kristen Monaco, Staff Writer, MedPage Today May 27, 2021 A more gradual increase in oral osilodrostat (Isturisa) dosing was better tolerated among patients with Cushing's disease, compared with those who had more accelerated increases, a researcher reported. Looking at outcomes from two phase III trials assessing osilodrostat, only 27% of patients had hypocortisolism-related adverse events if dosing was gradually increased every 3 weeks, said Maria Fleseriu, MD, of Oregon Health & Science University in Portland, in a presentation at the virtual meeting of the American Association of Clinical Endocrinology (AACE). On the other hand, 51% of patients experienced a hypocortisolism-related adverse event if osilodrostat dose was increased to once every 2 weeks. Acting as a potent oral 11-beta-hydroxylase inhibitor, osilodrostat was first approved by the FDA in March 2020 for adults with Cushing's disease who either cannot undergo pituitary gland surgery or have undergone the surgery but still have the disease. The drug is currently available in 1 mg, 5 mg, and 10 mg film-coated tablets. The approval came based off of the positive findings from the complementary LINC3 and LINC4 trials. The LINC3 trial included 137 adults with Cushing's disease with a mean 24-hour urinary free cortisol concentration (mUFC) over 1.5 times the upper limit of normal (50 μg/24 hours), along with morning plasma adrenocorticotropic hormone above the lower limit of normal (9 pg/mL). During the open-label, dose-escalation period, all the participants were given 2 mg of osilodrostat twice per day, 12 hours apart. Over this 12-week titration phase, dose escalations were allowed once every 2 weeks if there were no tolerability issues to achieve a maximum dose of 30 mg twice a day. After this 12-week dose-escalation schedule, additional bumps up in dose were permitted every 4 weeks. The median daily osilodrostat dose was 7.1 mg. The LINC4 trial included 73 patients with Cushing's disease with an mUFC over 1.3 times the upper limit of normal. The 48 patients randomized to receive treatment were likewise started on 2 mg bid of osilodrostat. However, this trial had a more gradual dose-escalation schedule, as doses were increased only every 3 weeks to achieve a 20 mg bid dose. After the 12-week dose-escalation phase, patients on a dose over 2 mg bid were restarted on 2 mg bid at week 12, where dose escalations were permitted once every 3 weeks thereafter to achieve a maximum 30 mg bid dose during this additional 36-week extension phase. Patients in this trial achieved a median daily osilodrostat dose of 5.0 mg. In both studies, patients' median age was about 40 years, the majority of patients were female, and about 88% had undergone a previous pituitary surgery. When comparing the adverse event profiles of both trials, Fleseriu and colleagues found that more than half of patients on the 2-week dose-escalation schedule experienced any grade of hypercortisolism-related adverse events. About 10.2% of these events were considered grade 3. About 28% of these patients had adrenal insufficiency -- the most common hypercortisolism-related adverse event reported. This was a catch-all term that include events like glucocorticoid deficiency, adrenocortical insufficiency, steroid withdrawal syndrome, and decreased cortisol, Fleseriu explained. Conversely, only 27.4% of patients on a 3-week dose escalation schedule experienced a hypercortisolism-related adverse event, and only 2.7% of these were grade 3. No grade 4 events occurred in either trial, and most events were considered mild or moderate in severity. "These adverse events were not associated with any specific osilodrostat dose of mean UFC level," Fleseriu said, adding that most of these events occurred during the initial dose-escalation periods. About 60% and 58% of all hypocortisolism-related adverse events occurred during the dose titration period in the 2-week and 3-week dose-escalation schedules, respectively. These events were managed via dose reduction, a temporary interruption in medication, and/or a concomitant medication. Very few patients in either trial permanently discontinued treatment due to these adverse events, Fleseriu noted. "Despite differences in the frequency of dose escalation, the time to first mUFC normalization was similar in the LINC3 and LINC4 studies," she said, adding that "gradual increases in osilodrostat dose from a starting dose of 2 mg bid can mitigate hypocortisolism-related adverse events without affecting mUFC control." "For patients with Cushing's disease, osilodrostat should be initiated at the recommended starting dose with incremental dose increases, based on individual response and tolerability aimed at normalizing cortisol levels," Fleseriu concluded. Kristen Monaco is a staff writer, focusing on endocrinology, psychiatry, and dermatology news. Based out of the New York City office, she’s worked at the company for nearly five years. Disclosures The LINC3 and LINC4 trials were funded by Novartis. Fleseriu reported relationships with Novartis, Recordati, and Strongbridge Biopharma. Primary Source American Association of Clinical Endocrinology Source Reference: Fleseriu M, et al "Effect of dosing and titration of osilodrostat on efficacy and safety in patients with Cushing's disease (CD): Results from two phase III trials (LINC3 and LINC4)" AACE 2021. From https://www.medpagetoday.com/meetingcoverage/aace/92824?xid=nl_mpt_DHE_2021-05-28&eun=g1406328d0r&utm_source=Sailthru&utm_medium=email&utm_campaign=Daily Headlines Top Cat HeC 2021-05-28&utm_term=NL_Daily_DHE_dual-gmail-definition
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Some of the latest research advancements in the field of endocrinology presented at the Endocrine Society's virtual ENDO 2021 meeting included quantifying diabetic ketoacidosis readmission rates, hyperglycemia as a severe COVID-19 predictor, and semaglutide as a weight loss therapy. Below are a few more research highlights: More Safety Data on Jatenzo In a study of 81 men with hypogonadism -- defined as a serum testosterone level below 300 ng/dL -- oral testosterone replacement therapy (Jatenzo) was both safe and effective in a manufacturer-sponsored study. After 24 months of oral therapy, testosterone concentration increased from an average baseline of 208.3 ng/dL to 470.1 ng/dL, with 84% of patients achieving a number in the eugonadal range. And importantly, the treatment also demonstrated liver safety, as there were no significant changes in liver function tests throughout the 2-year study -- including alanine aminotransferase (28.0 ± 12.3 to 26.6 ± 12.8 U/L), aspartate transaminase (21.8 ± 6.8 to 22.0 ± 8.2 U/L), and bilirubin levels (0.58 ± 0.22 to 0.52 ± 0.19 mg/dL). Throughout the trial, only one participant had elevation of liver function tests. "Our study finds testosterone undecanoate is an effective oral therapy for men with low testosterone levels and has a safety profile consistent with other approved testosterone products, without the drawbacks of non-oral modes of administration," said lead study author Ronald Swerdloff, MD, of the Lundquist Research Institute in Torrance, California, in a statement. In addition, for many men with hypogonadism, "an oral option is preferred to avoid issues associated with other modes of administration, such as injection site pain or transference to partners and children," he said. "Before [testosterone undecanoate] was approved, the only orally approved testosterone supplemental therapy in the United States was methyltestosterone, which was known to be associated with significant chemical-driven liver damage." Oral testosterone undecanoate received FDA approval in March 2019 following a rocky review history. COVID-19 Risk With Adrenal Insufficiency Alarming new data suggested that children with adrenal insufficiency were more than 23 times more likely to die from COVID-19 than kids without this condition (relative risk 23.68, P<0.0001). This equated to 11 deaths out of 1,328 children with adrenal insufficiency compared with 215 deaths out of 609,788 children without this condition (0.828% vs 0.035%). These young patients with adrenal insufficiency also saw a much higher rate of sepsis (RR 21.68, P<0.0001) and endotracheal intubation with COVID-19 infection (RR 25.45, P<0.00001). Data for the analysis were drawn from the international TriNetX database, which included patient records of children ages 18 and younger diagnosed with COVID-19 from 60 healthcare organizations in 31 different countries. "It's really important that you take your hydrocortisone medications and start stress dosing as soon as you're sick," study author Manish Raisingani, MD, of the University of Arkansas for Medical Sciences and Arkansas Children's in Little Rock, explained during a press conference. "This will help prevent significant complications due to COVID-19 or any other infections. A lot of the complications that we see in kids with adrenal insufficiency are due to inadequate stress dosing of steroids." And with kids starting to return back to in-person schooling, "parents should also be reeducated about using the emergency injections of hydrocortisone," Raisingani added. He noted that the COVID-19 complication rates were likely so high in this patient population because many had secondary adrenal insufficiency due to being on long-term, chronic steroids. Many also had comorbid respiratory illnesses, as well. Cushing's Death Risk In a systematic review and meta-analysis of 87 studies -- including data on 17,276 patients with endogenous Cushing's syndrome -- researchers found that these patients face a much higher death rate than those without this condition. Overall, patients with endogenous Cushing's syndrome faced a nearly three times higher mortality ratio (standardized mortality ratio 2.91, 95% CI 2.41-3.68, I2=40.3%), with those with Cushing's disease found to have an even higher mortality risk (SMR 3.27, 95% CI 2.33-4.21, I2=55.6%). And those with adrenal Cushing's syndrome also saw an elevated death risk, although not as high as patients with the disease (SMR 1.62, 95% CI 0.08-3.16, I2=0.0%). The most common causes of mortality among these patients included cardiac conditions (25%), infection (14%), and cerebrovascular disease (9%). "The causes of death highlight the need for aggressive management of cardiovascular risk, prevention of thromboembolism, and good infection control, and emphasize the need to achieve disease remission, normalizing cortisol levels," said lead study author Padiporn Limumpornpetch, MD, of the University of Leeds in England, in a statement. From https://www.medpagetoday.com/meetingcoverage/endo/91808
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Adrenal insufficiency increases the risk for severe outcomes, including death, 23-fold for children who contract COVID-19, according to a data analysis presented at the ENDO annual meeting. “Adrenal insufficiency in pediatrics does increase risk of complications with COVID-19 infections,” Manish Gope Raisingani, MD, assistant professor in the department of pediatrics in the division of pediatric endocrinology at Arkansas Children's Hospital, University of Arkansas for Medical Sciences, told Healio. “The relative risk of complications is over 20 for sepsis, intubation and mortality, which is very significant.” Source: Adobe Stock Using the TriNetX tool and information on COVID-19 from 54 health care organizations, Raisingani and colleagues analyzed data from children (aged 0-18 years) with COVID-19; 846 had adrenal insufficiency and 252,211 did not. The mortality rate among children with adrenal insufficiency was 2.25% compared with 0.097% for those without, for a relative risk for death of 23.2 (P < .0001) for children with adrenal insufficiency and COVID-19. RRs for these children were 21.68 for endotracheal intubation and 25.45 for sepsis. “Children with adrenal insufficiency should be very careful during the pandemic,” Raisingani said. “They should take their steroid medication properly. They should also be appropriately trained on stress steroids for infection, other significant events.” From https://www.healio.com/news/endocrinology/20210321/severe-covid19-risks-greatly-increased-for-children-with-adrenal-insufficiency
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Patients with adrenal insufficiency may have higher rates of cardiovascular events due to the presence of cardiovascular comorbidities, shows a study published in The Journal of Clinical Endocrinology and Metabolism. Led by Kanchana Ngaosuwan, MD, PhD, of Imperial College London, UK, the authors of this population-based matched cohort study also found that cerebrovascular events were independently increased in patients with secondary adrenal insufficiency, particularly in those treated with irradiation therapy. Cardiovascular mortality, specifically from ischemic heart disease, was higher regardless of having secondary adrenal insufficiency or primary adrenal insufficiency (Addison’s disease). Adrenal insufficiency occurs when the adrenal glands fail to produce adequate glucocorticoids. In Addison’s disease, it arises from the adrenal glands, but in secondary adrenal insufficiency, it occurs as a result of a pituitary or hypothalamic condition. Glucocorticoid replacement therapy is usually the first line of defense, but the treatment is associated with a number of adverse events, such as cardiovascular disease. Ischemic heart disease is the leading cause of death for patients with Addison’s disease. Data from this study was sourced from the Clinical Practice Research Datalink which collected information from 15,354,125 individuals living in the United Kingdom between 1987 and 2017. Data from patients prescribed glucocorticoid prescriptions for adrenal insufficiency (primary: n=2,052; secondary: n=3,948) and random age and gender matched controls (primary: n=20,366; secondary: n=39,134) were assessed for comorbidities and clinical outcomes. Patients and controls had previous cardiovascular disease (17.5% vs 11.2%), diabetes (10.4% vs 4.8%), hypertension (22.1% vs 13.6%), dyslipidemia (20.5% vs 5.0%), and 19.6% and 4.9% of patients and controls were taking statins, respectively. Composite cardiovascular events occurred at a rate of 31.4 (95% CI, 29.6-33.3) per 1,000 person-years among the patients and 24.4 (95% CI, 23.9-24.9; P <.0001) per 1,000 person years among the controls. Stratified by adrenal insufficiency subtype, after correcting for cofounders, patients with primary (adjusted hazard ratio [aHR], 1.08; 95% CI, 0.96-1.22) and secondary (aHR, 1.10; 95% CI, 1.01-1.19) adrenal insufficiency were at marginally increased risk for composite cardiovascular events. Cerebrovascular disease occurred at a rate of 10.4 (95% CI, 9.5-11.5) per 1.000 person years among the patients and 7.2 (95% CI, 7.0-7.5; P <.0001) per 1,000 person years among the controls. Only patients with secondary adrenal insufficiency were at increased risk for cerebrovascular disease (aHR, 1.53; 95% CI, 1.34-1.74). All patients had increased risk for hospitalization due to cardiovascular diseases (aHR, 1.41; 95% CI, 1.28-1.55) and only the patients with secondary adrenal insufficiency were more likely to be hospitalized with cerebrovascular disease (aHR, 1.63; 95% CI, 1.28-2.08). Patients had increased rates of cardiovascular mortality compared with controls (9.9 vs 6.4 per 1,000 person years; P <.0001). Both patients with primary (aHR, 1.58; 95% CI, 1.19-2.10) and secondary (aHR, 1.23; 95% CI, 0.99-1.52) insufficiency were at increased risk for cardiovascular mortality. Risk for cerebrovascular mortality was elevated for patients with secondary insufficiency (aHR, 1.14; 95% CI, 0.78-1.67). Stratified by secondary insufficiency, age, and sex, women (aHR, 1.18; 95% CI, 1.04-1.31; P =.016) and patients who were less than 50 years old (aHR, 1.58; 95% CI, 1.22-2.03; P <.0001) were at increased risk for composite cardiovascular events. Similarly, patients 50 years old or younger were at increased risk for cerebrovascular disease (aHR, 3.67; 95% CI, 2.60-5.17; P <.0001). These data may be limited by the cohort imbalance of disease risk factors, although the investigators corrected for these features, some residual biases may remain. While further study is needed to assess changes in treatment approaches, the authors suggested that “these findings support further optimization of glucocorticoid replacement in conjunction with cardio protective interventions in patients with adrenal insufficiency.” Reference Ngaosuwan K, Johnston D G, Godsland I F, et al. Cardiovascular disease in patients with primary and secondary adrenal insufficiency and the role of comorbidities. J Clin Endocrinol Metab. 2021;dgab063. doi:10.1210/clinem/dgab063. From https://www.endocrinologyadvisor.com/home/topics/cardiovascular-and-metabolic-disorders/adrenal-insufficiency-associated-with-increased-cvd-and-cerebrovascular-disease/
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A retrospective cohort study was performed to compare mortality risk and causes of death in adrenal insufficiency with an individually-matched reference population. Researchers examined 6,821 patients with adrenal insufficiency (primary, 2052; secondary, 3948) and 6,7564 individually-matched controls (primary, 20366; secondary, 39134). It was shown that in adrenal insufficiency, mortality was elevated, particularly primary, even with individual matching, and was found early in the disease course. The data demonstrated that cardiovascular disease was the major cause but mortality from infection was also high. The adrenal crisis was a common contributor. The outcomes suggested that early education for prompt treatment of infections and avoidance of adrenal crisis hold the potential to decrease mortality. The Journal of Clinical Endocrinology & Metabolism, dgab096, https://doi.org/10.1210/clinem/dgab096 Abstract Context Mortality data in patients with adrenal insufficiency are inconsistent, possibly due to temporal and geographical differences between patients and their reference populations. Objective To compare mortality risk and causes of death in adrenal insufficiency with an individually-matched reference population. Design Retrospective cohort study. Setting UK general practitioner database (CPRD). Participants 6821 patients with adrenal insufficiency (primary, 2052; secondary, 3948) and 67564 individually-matched controls (primary, 20366; secondary, 39134). Main outcome measures All-cause and cause-specific mortality; hospital admission from adrenal crisis. Results With follow-up of 40799 and 406899 person-years for patients and controls respectively, the hazard ratio (HR; [95%CI]) for all-cause mortality was 1.68 [1.58 - 1.77]. HRs were greater in primary (1.83 [1.66 - 2.02]) than in secondary (1.52 [1.40 - 1.64]) disease; (HR; primary versus secondary disease, 1.16 [1.03 - 1.30]). The leading cause of death was cardiovascular disease (HR 1.54 [1.32-1.80]), along with malignant neoplasms and respiratory disease. Deaths from infection were also relatively high (HR 4.00 [2.15 - 7.46]). Adrenal crisis contributed to 10% of all deaths. In the first two years following diagnosis, the patients’ mortality rate and hospitalisation from adrenal crisis were higher than in later years. Conclusion Mortality was increased in adrenal insufficiency, especially primary, even with individual matching and was observed early in the disease course. Cardiovascular disease was the major cause but mortality from infection was also high. Adrenal crisis was a common contributor. Early education for prompt treatment of infections and avoidance of adrenal crisis hold potential to reduce mortality. PDF available at https://academic.oup.com/jcem/advance-article-abstract/doi/10.1210/clinem/dgab096/6141434?redirectedFrom=fulltext
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The treatment of adrenal insufficiency with hydrocortisone granules in children with congenital adrenal hyperplasia (CAH) was associated with an absence of adrenal crises and normal growth patterns over a 2-year period, according to study findings published in The Journal of Clinical Endocrinology and Metabolism. The study included a total of 17 children with CAH and 1 child with hypopituitarism. All included participants were <6 years old who were receiving current adrenocortical replacement therapy, including hydrocortisone with or without fludrocortisone. Hydrocortisone medications used in this population were converted from pharmacy compounded capsules to hydrocortisone granules without changing the dose. These study participants were followed by study investigators for 2 years. Glucocorticoid replacement therapy was given three times a day for a median treatment duration of 795 days. Treatment was adjusted by 3 monthly 17-hydroxyprogesterone (17-OHP) profiles in children with CAH. There were a 150 follow-up visits throughout the study. At each visit, participants underwent assessments that measured hydrocortisone dose, height, weight, pubertal status, adverse events, and incidence of adrenal crisis. A total of 40 follow-up visits had changes in hydrocortisone doses based on salivary measurements (n=32) and serum 17-OHP levels (n=8). At time of study entry, the median daily doses of hydrocortisone were 11.9 mg/m2 for children between the ages of 2 to 8 years, 9.9 mg/m2 for children between 1 month and 2 years, and 12.0 mg/m2 for children <28 days of age. At the end of the study, the respective doses for the 3 age groups were 10.2, 9.8, and 8.6. The investigators observed no trends in either accelerated growth or reduced growth; however, 1 patient with congenital renal hypoplasia and CAH did show reduced growth. While 193 treatment-emergent adverse events, including pyrexia, gastroenteritis, and viral upper respiratory tract infection, were reported in 14 patients, there were no observed adrenal crises. Limitations of this study included the small sample size as well as the relatively high drop-out rate of the initial sample. The researchers concluded that “hydrocortisone granules are an effective treatment for childhood adrenal insufficiency providing the ability to accurately prescribe pediatric appropriate doses.” Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures. Reference Neumann U, Braune K, Whitaker MJ, et al. A prospective study of children 0-7 years with CAH and adrenal insufficiency treated with hydrocortisone granules. Published online September 4, 2020. J Clin Endocrinol Metab. doi:10.1210/clinem/dgaa626
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Adults with adrenal insufficiency who are adequately treated and trained display the same incidence of COVID-19-suggestive symptoms and disease severity as controls, according to a presenter. “Adrenal insufficiency is supposed to be associated with an increased risk for infections and complications,” Giulia Carosi, a doctoral student in the department of experimental medicine at Sapienza University of Rome, said during a presentation at the virtual European Congress of Endocrinology Annual Meeting. “Our aim was to evaluate the incidence of COVID symptoms and related complications in this group.” In a retrospective, case-control study, Carosi and colleagues evaluated the incidence of COVID-19 symptoms and complications among 279 adults with primary or secondary adrenal insufficiency (mean age, 57 years; 49.8% women) and 112 adults with benign pituitary nonfunctioning lesions without hormonal alterations, who served as controls (mean age, 58 years; 52.7% women). All participants lived in the Lombardy region of northern Italy. Participants completed a standardized questionnaire by phone on COVID-19-suggestive symptoms, such as fever, cough, myalgia, fatigue, dyspnea, gastrointestinal symptoms, conjunctivitis, loss of smell, loss of taste, upper respiratory tract symptoms, thoracic pain, headaches and ear pain. Patients with primary or secondary adrenal insufficiency were previously trained to modify their glucocorticoid replacement therapy when appropriate. From February through April, the prevalence of participants reporting at least one symptom of viral infection was similar between the adrenal insufficiency group and controls (24% vs. 22.3%; P = .788). Researchers observed “highly suggestive” symptoms among 12.5% of participants in both groups. No participant required hospitalization and no adrenal crisis was reported. Replacement therapy was correctly increased for about 30% of symptomatic participants with adrenal insufficiency. Carosi noted that few nasopharyngeal swabs were performed (n = 12), limiting conclusions on the exact infection rate (positive result in 0.7% among participants with adrenal insufficiency and 0% of controls; P = .515). “We can conclude that hypoadrenal patients who have regular follow-up and trained about risks for infection and sick day rules seem to present the same incidence of COVID-19 symptoms and the same disease severity as controls,” Carosi said. As Healio previously reported, there is no evidence that COVID-19 has a more severe course among individuals with primary and secondary adrenal insufficiency; however, those with adrenal insufficiency are at increased risk for respiratory and viral infections, and patients experiencing major inflammation and fever are at risk for life-threatening adrenal crisis. In a position statement issued by the American Association of Clinical Endocrinologists in March, researchers wrote that people with adrenal insufficiency or uncontrolled Cushing’s syndrome should continue to take their medications as prescribed and ensure they have appropriate supplies for oral and injectable steroids at home, with a 90-day preparation recommended. In the event of acute illness, those with adrenal insufficiency are instructed to increase their hydrocortisone dose per instructions and call their health care provider for more details. Standard “sick day” rules for increasing oral glucocorticoids or injectables would also apply, according to the statement. From https://www.healio.com/news/endocrinology/20200910/no-increased-covid19-risk-with-adequately-treated-adrenal-insufficiency
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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 If experiencing fever, cough, shortness of breath or other symptoms, call both the COVID-19 hotline (check your state government website for contact information) and your primary care physician or endocrinologist Monitor symptoms and contact your physician immediately following signs of illness Acquire a medical alert bracelet/necklace in case of an emergency Individuals with uncontrolled Cushing’s Syndrome of any origin are at higher risk of infection in general. Although information on people with Cushing’s Syndrome and COVID-19 is scarce, given the rarity of the condition, those with Cushing’s Syndrome should strictly adhere to CDC recommendations: Maintain social distancing to reduce the risk of contracting COVID-19 If experiencing fever, cough, shortness of breath or other symptoms, call both the COVID-19 hotline (check your state government website for contact information) and your primary care physician or endocrinologist In addition, people with either condition should continue to follow the general guidelines at these times: Stay home as much as possible to reduce your risk of being exposed When you do go out in public, avoid crowds and limit close contact with others Avoid non-essential travel Wash your hands with soap and water regularly, for at least 20 seconds, especially before eating or drinking and after using the restroom and blowing your nose, coughing or sneezing If soap and water are not readily available, use an alcohol-based sanitizer with at least 60% alcohol Cover your nose and mouth when coughing or sneezing with a tissue or a flexed elbow, then throw the tissue in the trash Avoid touching your eyes, mouth or nose when possible From https://www.aace.com/recent-news-and-updates/aace-position-statement-coronavirus-covid-19-and-people-adrenal
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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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On Dec 12th, I am speaking at a sold-out event and telling half of a funny story, then posting it on YouTube, To hear the rest of the story people have to go to my website which is all about Cushing's disease. Every day I see people who I am certain have Cushing's but don't know it. I want to reach these people and the general public. What title can I use for my video? I need your help with this. The story is much like Abbott and Costello's Who's on second, what's on third routine. But there has to be a connection to cushing's. So far, I have: Is it obesity or Cushing's disease? When I get the title and post the video, I need the support of everyone here to view it and go to my website. If you could share and get family and friends to do the same that would be greatly appreciated. Wouldn't it be great if the video went viral and so many people would learn about Cushing's? We can make this happen if I get your support. Thanks everyone. Keep working on a better tite for me. Can't wait to see your suggestions. Thanks again. jan
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I am looking for some place like The Mayo clinic or Endocrinologists that would be interested in setting up a dietary study with their Cushing's patients, I am having great success with my specialized diet in lessening the symptoms of cushing's and want to help others get a better quality of life while living with this disease. The first picture is me with Cushing's in 2013 before surgery. the next two pictures are me now with a cushing's recurrence while on my specialized diet. For 3 years I used my body as a science experiment with foods. I don't have a moon face, I have not gained any weight, my girth is much less and my energy and strength are much better than the first time I had Cushing's. The only difference is my diet. For 2 years my endo refused to test me for cushing's again because I did not look the way I should. I had to get other doctors to do the first and secong level tests then I brought those results to my endo and asked him to do the dex suppression test. All tests confirmed Cushing's recurrence. He still won't believe me that my diet has anything to do with the way I look or feel. I am the proof, but he still wont beieve me. What will it take for people to listen to us and believe us????
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untilPresented by Irina Bancos, MD Assistant Professor of Medicine Endocrinology Department Mayo Clinic, Rochester, MN Space is limited. Reserve your webinar seat. After registering you will receive a confirmation email containing information about joining the webinar. Contact us at webinar@pituitary.org if you have any questions. Date: Tuesday, May 28, 2019 Time: 10:00 AM - 11:00 AM Pacific Daylight Time, 1:00 PM - 2:00 PM Eastern Daylight Time Webinar Description Learning Objectives: To distinguish between primary and secondary adrenal insufficiency To understand the pitfalls of current diagnostic tests to diagnose adrenal insufficiency. To describe physiological replacement therapy for adrenal insufficiency To distinguish between adrenal insufficiency and glucocorticoid withdrawal syndrome. Presenter Bio Dr. Irina Bancos is the Assistant Professor of Medicine and works in the Pituitary-Adrenal-Gonadal subdivision of Endocrinology division at Mayo Clinic, Rochester. She also serves as Director of the Endocrine testing center. Dr. Bancos received her M.D. from the Iuliu Hatieganu Medical University in Cluj-Napoca, Romania. She has completed her Internal Medicine Residency at Danbury Hospital in CT and Endocrinology Fellowship at Mayo Clinic, Rochester. In addition, Dr. Bancos completed a two year research fellowship (Mayo Foundation Scholarship) at the University of Birmingham, United Kingdom where she received training in steroid profiling and adrenal disorders. In 2015 she returned to Mayo Clinic, where her clinical and research interests include adrenal and pituitary tumors, adrenal insufficiency, congenital adrenal hyperplasia, Cushing syndrome, and mechanisms of steroid regulation of metabolism. Between 2015 and 2018, Dr. Bancos was the principal investigator and leader of the Transform the Adrenal Practice team at Mayo Clinic. Dr. Bancos has published 77 scientific articles. In addition to clinical practice in the pituitary-adrenal-gonadal clinic, Dr. Bancos enjoys teaching fellows, residents and medical students. She is the principal investigator of several ongoing prospective studies in Cushing syndrome, adrenal insufficiency, prolactinoma, and adrenal tumors. Dr. Bancos currently holds several grants in the field of steroid regulation of aging, metabolism and body composition.
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- webinar
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Presented by Irina Bancos, MD Assistant Professor of Medicine Endocrinology Department Mayo Clinic, Rochester, MN Space is limited. Reserve your webinar seat. After registering you will receive a confirmation email containing information about joining the webinar. Contact us at webinar@pituitary.org if you have any questions. Date: Tuesday, May 28, 2019 Time: 10:00 AM - 11:00 AM Pacific Daylight Time, 1:00 PM - 2:00 PM Eastern Daylight Time Webinar Description Learning Objectives: To distinguish between primary and secondary adrenal insufficiency To understand the pitfalls of current diagnostic tests to diagnose adrenal insufficiency. To describe physiological replacement therapy for adrenal insufficiency To distinguish between adrenal insufficiency and glucocorticoid withdrawal syndrome. Presenter Bio Dr. Irina Bancos is the Assistant Professor of Medicine and works in the Pituitary-Adrenal-Gonadal subdivision of Endocrinology division at Mayo Clinic, Rochester. She also serves as Director of the Endocrine testing center. Dr. Bancos received her M.D. from the Iuliu Hatieganu Medical University in Cluj-Napoca, Romania. She has completed her Internal Medicine Residency at Danbury Hospital in CT and Endocrinology Fellowship at Mayo Clinic, Rochester. In addition, Dr. Bancos completed a two year research fellowship (Mayo Foundation Scholarship) at the University of Birmingham, United Kingdom where she received training in steroid profiling and adrenal disorders. In 2015 she returned to Mayo Clinic, where her clinical and research interests include adrenal and pituitary tumors, adrenal insufficiency, congenital adrenal hyperplasia, Cushing syndrome, and mechanisms of steroid regulation of metabolism. Between 2015 and 2018, Dr. Bancos was the principal investigator and leader of the Transform the Adrenal Practice team at Mayo Clinic. Dr. Bancos has published 77 scientific articles. In addition to clinical practice in the pituitary-adrenal-gonadal clinic, Dr. Bancos enjoys teaching fellows, residents and medical students. She is the principal investigator of several ongoing prospective studies in Cushing syndrome, adrenal insufficiency, prolactinoma, and adrenal tumors. Dr. Bancos currently holds several grants in the field of steroid regulation of aging, metabolism and body composition.
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Metoclopramide, a gastrointestinal medicine, can increase cortisol levels after unilateral adrenalectomy — the surgical removal of one adrenal gland — and conceal adrenal insufficiency in bilateral macronodular adrenal hyperplasia (BMAH) patients, a case report suggests. The study, “Retention of aberrant cortisol secretion in a patient with bilateral macronodular adrenal hyperplasia after unilateral adrenalectomy,” was published in Therapeutics and Clinical Risk Management. BMAH is a subtype of adrenal Cushing’s syndrome, characterized by the formation of nodules and enlargement of both adrenal glands. In this condition, the production of cortisol does not depend on adrenocorticotropic hormone (ACTH) stimulation, as usually is the case. Instead, cortisol production is triggered by a variety of stimuli, such as maintaining an upright posture, eating mixed meals — those that contain fats, proteins, and carbohydrates — or exposure to certain substances. A possible treatment for this condition is unilateral adrenalectomy. However, after the procedure, some patients cannot produce adequate amounts of cortisol. That makes it important for clinicians to closely monitor the changes in cortisol levels after surgery. Metoclopramide, a medicine that alleviates gastrointestinal symptoms and is often used during the postoperative period, has been reported to increase the cortisol levels of BMAH patients. However, the effects of metoclopramide on BMAH patients who underwent unilateral adrenalectomy are not clear. Researchers in Japan described the case of a 61-year-old postmenopausal woman whose levels of cortisol remained high after surgery due to metoclopramide ingestion. The patient was first examined because she had experienced high blood pressure, abnormal lipid levels in the blood, and osteoporosis for ten years. She also was pre-obese. She was given medication to control blood pressure with no results. The lab tests showed high serum cortisol and undetectable levels of ACTH, suggesting adrenal Cushing’s syndrome. Patients who have increased cortisol levels, but low levels of ACTH, often have poor communication between the hypothalamus, the pituitary, and the adrenal glands. These three glands — together known as the HPA axis — control the levels of cortisol in healthy people. Imaging of the adrenal glands revealed they were both enlarged and presented nodules. The patient’s cortisol levels peaked after taking metoclopramide, and her serum cortisol varied significantly during the day while ACTH remained undetectable. These results led to the BMAH diagnosis. The doctors performed unilateral adrenalectomy to control cortisol levels. The surgery was successful, and the doctors reduced the dose of glucocorticoid replacement therapy on day 6. Eight days after the surgery, however, the patient showed decreased levels of fasting serum cortisol, which indicated adrenal insufficiency — when the adrenal glands are unable to produce enough cortisol. The doctors noticed that metoclopramide was causing an increase in serum cortisol levels, which made them appear normal and masked the adrenal insufficiency. They stopped metoclopramide treatment and started replacement therapy (hydrocortisone) to control the adrenal insufficiency. The patient was discharged 10 days after the surgery. The serum cortisol levels were monitored on days 72 and 109 after surgery, and they remained lower than average. Therefore she could not stop hydrocortisone treatment. The levels of ACTH remained undetectable, suggesting that the communication between the HPA axis had not been restored. “Habitual use of metoclopramide might suppress the hypothalamus and pituitary via negative feedback due to cortisol excess, and lead to a delayed recovery of the HPA axis,” the researchers said. Meanwhile, the patient’s weight decreased, and high blood pressure was controlled. “Detailed surveillance of aberrant cortisol secretion responses on a challenge with exogenous stimuli […] is clinically important in BMAH patients,” the study concluded. “Caution is thus required for assessing the actual status of the HPA axis.” From https://cushingsdiseasenews.com/2019/05/07/metoclopramide-conceals-adrenal-insufficiency-after-gland-removal-bmah-patients-case-report/
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The use of an insulin pump to deliver continuous pulsatile cortisol may be a viable treatment option in patients with severe adrenal insufficiency who are unresponsive to oral corticosteroids, according to study results presented at the 28th Annual Congress of the American Association of Clinical Endocrinologists, held April 24 to 28, 2019, in Los Angeles, California. According to the investigators, increasing oral steroid doses may be required to prevent adrenal crisis in patients with adrenal insufficiency. However, in light of the associated side effects of long-term use of steroids, an alternative treatment method is needed. Insulin pumps, typically used to treat patients with diabetes, can be used to deliver steroids and may provide symptom control, prevent adrenal crisis, and lower required corticosteroid dose. The current study enrolled patients with adrenal insufficiency who could not absorb oral corticosteroid treatment or were not responding to treatment. Of 118 patients with adrenal insufficiency, 6 patients were switched to pump treatment. The results indicated that the use of cortisol pumps was associated with a 78.5% risk reduction for adrenal crisis compared with oral corticosteroids. As hydrocortisone dose was gradually tapered using the cortisol pump, there was a mean dose reduction of 62.77 mg compared with oral corticosteroid therapy. The researchers noted that in addition to reducing the number of adrenal crises, use of a cortisol pump was found to be associated with better symptom control and quality of life. “Continuous pulsatile cortisol replacement via pump is an option for management of severe adrenal insufficiency in patients unresponsive to oral therapy,” concluded the researchers. Reference Khalil A, Ahmed F, Alzohaili O. Insulin pump for adrenal insufficiency, a novel approach to the use of insulin pumps to deliver corticosteroids in patients with poor cortisol absorption. Presented at: American Association of Clinical Endocrinologists 28th Annual Scientific & Clinical Congress; April 24-28, 2019; Los Angeles, CA. From https://www.endocrinologyadvisor.com/home/conference-highlights/aace-2019/cortisol-pumps-may-be-viable-option-to-reduce-adrenal-crisis-in-severe-adrenal-insufficiency/
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13th Annual Conference for Adults with Endocrine Disorder
MaryO posted a calendar event in Cushie Calendar
until13th Annual Conference for Adults with Endocrine Disorders in Partnership with Barrow Neurological Institute Pituitary Center February 28th, 2019 - March 3rd, 2019 Phoenix, Arizona Schedule of Events Thursday 5:00 pm - 7:00 pm Welcome Reception, Wyndham Garden Phoenix Midtown Friday 9:00 am - 4:00 pm Exhibitors, Barrow Pituitary Center 10:00 am - 12:00 pm Educational Segments, Barrow Pituitary Center 12:00 am - 1:00 pm Lunch (included) 1:00 pm - 3:00 pm Educational Segments, Barrow Pituitary Center 5:00 pm - 8:00 pm Group outing to Scottsdale Waterfront Saturday 10:00 am - 12:00 pm Educational Segments, Barrow Pituitary Center 12:00 am - 1:00 pm Lunch (included) 1:00 pm - 3:30 pm Educational Segments, Barrow Pituitary Center Sunday 9:00 am - 1:30 pm Educational Segments, Wyndham Garden Phoenix Midtown ********************************************************** Friday Educational Segments at Barrow Pituitary Center 10:00 am Managing Cushings: Navigating Through the Maze, Yuen or 10:00 am Managing AGHD: Daily and Beyond, Knecht 11:00 am Hypothalamic Obesity: Not Just Calories In, Calories Out, Connor 12:00 pm LUNCH (included) 1:00 pm Me, Myself and My Adrenal Insufficiency, Yuen 2:00 pm Navigating the Medical Maze, Herring Saturday Educational Segments at Barrow Pituitary Center 10:00 am Beyond AGHD and Cushings: Familial and Genetic Factors, Stratakis 11:00 am Q&A, Stratakis 12:00 pm LUNCH (included) 1:00 pm Tools for Coping with my Endocrine Disorder, Jonas 2:00 pm Finnigan and Friends: A Year in AI Training, Palmer 2:30 pm Quality of Life Study, Cushings, Edgar & Keil or 2:30 pm Life is What You Make Of It, Jones Sunday Educational Segments at Wyndham Garden Phoenix Midtown 9:00 am Preventing Muscle Wasting and Nutrition, Fine 10:00 am Nuances of Treating Hypothyroidism, Friedman 11:00 am Macrilen Stimulation Test for Growth Hormone Deficiency, Friedman 11:45 am The New and The Old for Diagnosing Cushing's Syndrome, Friedman 12:30 pm Ask the Wiz, Friedman Location Barrow Neurological Institute at St. Joseph's Hospital and Medical Center Goldman Auditorium and Sonntag Pavilion 350 W. Thomas Rd. Phoenix, AZ 85013 Transportation will be provided on Friday and Saturday between the Wyndham Hotel to Barrow for an hour prior to the segments and an hour after close of the segments. The hotel is approximately 1/2 mile away from Barrow Pituitary Center if you choose to walk or travel there on your own. Hotel Room Rates and Reservations Wyndham Garden Phoenix Midtown 3600 N. 2nd Ave. Phoenix, AZ 85013 $109 per night + tax. Includes free wifi, parking and buffet breakfast To make hotel reservations call 602-604-4900 and ask for The MAGIC Foundation guest room block. Refrigerators are first come so be sure to request one when making your reservation. Airport Transportation Transportation is not provided to/from the hotel from the airport. The Wyndham is approximately 9 miles from the airport. Preferred airport is Phoenix, AZ - PHX - Sky Harbor Intl. Deadline to Register and book your hotel is January 28, 2019 View the entire PDF Program-
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13th Annual Conference for Adults with Endocrine Disorders in Partnership with Barrow Neurological Institute Pituitary Center February 28th, 2019 - March 3rd, 2019 Phoenix, Arizona Schedule of Events Thursday 5:00 pm - 7:00 pm Welcome Reception, Wyndham Garden Phoenix Midtown Friday 9:00 am - 4:00 pm Exhibitors, Barrow Pituitary Center 10:00 am - 12:00 pm Educational Segments, Barrow Pituitary Center 12:00 am - 1:00 pm Lunch (included) 1:00 pm - 3:00 pm Educational Segments, Barrow Pituitary Center 5:00 pm - 8:00 pm Group outing to Scottsdale Waterfront Saturday 10:00 am - 12:00 pm Educational Segments, Barrow Pituitary Center 12:00 am - 1:00 pm Lunch (included) 1:00 pm - 3:30 pm Educational Segments, Barrow Pituitary Center Sunday 9:00 am - 1:30 pm Educational Segments, Wyndham Garden Phoenix Midtown ********************************************************** Friday Educational Segments at Barrow Pituitary Center 10:00 am Managing Cushings: Navigating Through the Maze, Yuen or 10:00 am Managing AGHD: Daily and Beyond, Knecht 11:00 am Hypothalamic Obesity: Not Just Calories In, Calories Out, Connor 12:00 pm LUNCH (included) 1:00 pm Me, Myself and My Adrenal Insuffiency, Yuen 2:00 pm Navigating the Medical Maze, Herring Saturday Educational Segments at Barrow Pituitary Center 10:00 am Beyond AGHD and Cushings: Familial and Genetic Factors, Stratakis 11:00 am Q&A, Stratakis 12:00 pm LUNCH (included) 1:00 pm Tools for Coping with my Endocrine Disorder, Jonas 2:00 pm Finnigan and Friends: A Year in AI Training, Palmer 2:30 pm Quality of Life Study, Cushings, Edgar & Keil or 2:30 pm Life is What You Make Of It, Jones Sunday Educational Segments at Wyndham Garden Phoenix Midtown 9:00 am Preventing Muscle Wasting and Nutrition, Fine 10:00 am Nuances of Treating Hypothyroidism, Friedman 11:00 am Macrilen Stimulation Test for Growth Hormone Defiency, Friedman 11:45 am The New and The Old for Diagnosing Cushing's Syndrome, Friedman 12:30 pm Ask the Wiz, Friedman Location Barrow Neurological Institute at St. Joseph's Hospital and Medical Center Goldman Auditorium and Sonntag Pavilion 350 W. Thomas Rd. Phoenix, AZ 85013 Transportation will be provided on Friday and Saturday between the Wyndham Hotel to Barrow for an hour prior to the segments and an hour after close of the segments. The hotel is approximately 1/2 mile away from Barrow Pituitary Center if you choose to walk or travel there on your own. Hotel Room Rates and Reservations Wyndham Garden Phoenix Midtown 3600 N. 2nd Ave. Phoenix, AZ 85013 $109 per night + tax. Includes free wifi, parking and buffet breakfast To make hotel reservations call 602-604-4900 and ask for The MAGIC Foundation guest room block. Refrigerators are first come so be sure to request one when making your reservation. Airport Transportation Transportation is not provided to/from the hotel from the airport. The Wyndham is approximately 9 miles from the airport. Preferred airport is Phoenix, AZ - PHX - Sky Harbor Intl. Deadline to Register and book your hotel is January 28, 2019 View the entire PDF Program
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Patients with different subtypes of Cushing’s syndrome (CS) have distinct plasma steroid profiles. This could be used as a test for diagnosis and classification, a German study says. The study, “Plasma Steroid Metabolome for Diagnosis and Subtyping Patients with Cushing Syndrome,” appeared in the journal Clinical Chemistry. A quick diagnosis of CS is crucial so that doctors can promptly give therapy. However, diagnosing CS is often complicated by the multiple tests necessary not just to diagnose the disease but also to determine its particular subtype. Cortisol, which leads to CS when produced at high levels, is a steroid hormone. But while earlier studies were conducted to determine whether patients with different subtypes of CS had distinct steroid profiles, the methods researchers used were cumbersome and have been discontinued for routine use. Recently, a technique called LC-MS/MS has emerged for multi-steroid profiling in patients with adrenocortical dysfunction such as congenital adrenal hyperplasia, adrenal insufficiency and primary aldosteronism. Researchers at Germany’s Technische Universität in Dresden used that method to determine whether patients with the three main subtypes of CS (pituitary, ectopic and adrenal) showed differences in plasma steroid profiles. They measured levels of 15 steroids produced by the adrenal glands in single plasma samples collected from 84 patients with confirmed CS and 227 age-matched controls. They found that CS patients saw huge increases in the plasma steroid levels of 11-deoxycortisol (289%), 21-deoxycortisol (150%), 11-deoxycorticosterone (133%), corticosterone (124%) and cortisol (122%), compared to patients without the disease. Patients with the ectopic subtype had the biggest jumps in levels of these steroids. However, plasma 18-oxocortisol levels were particularly low in ectopic disease. Other steroids demonstrated considerable variation. Patients with the adrenal subtype had the lowest concentration of dehydroepiandrosterone (DHEA) and DHEA-SO4, which are androgens. Patients with the ectopic and pituitary subtype had the lowest concentration of aldosterone. Through the use of 10 selected steroids, patients with different subtypes of CS could be identified almost as closely as with other tests, including the salivary and urinary free cortisol test, the dexamethasone-suppressed cortisol test, and plasma adrenocorticotropin levels. The misclassification rate using steroid levels was 9.5 percent, compared to 5.8 percent in other tests. “This study using simultaneous LC-MS/MS measurements of 15 adrenal steroids in plasma establishes distinct steroid metabolome profiles that might be useful as a test for CS,” the team concluded, adding that using LC-MS/MS is advantageous, as specimen preparation is simple and the entire panel takes 12 minutes to run. This means it could be offered as a single test for both identification and subtype classification. From https://cushingsdiseasenews.com/2018/01/02/plasma-steroid-levels-used-screen-diagnosis-subtyping-patients-cushing-syndrome/
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The New Jersey Department of Health passed a waiver in October of last year that allows ambulances to carry Solu-Cortef, for the purposes of treating an adrenal crisis. As a result, New Jersey ambulances can be better prepared to treat adrenal insufficiency. This news was brought to NADF by Karen Fountain of the CARES Foundation, who has been helping push state health directors to accept protocols to help treat adrenal insufficient patients during an emergency. Adrenal insufficient people in New Jersey should contact their local EMS to make them aware of the waiver, and encourage them to carry Solu-Cortef in their ambulances. The hope is that other states, and eventually the entire coun- try and beyond, will start having their ambulances carry the needed medication to treat adrenal crisis. http://www.nadf.us
