Endocrine Society Releases Guidelines on Treatment of Cushing’s Syndrome

Posted on August 26, 2015 by MaryO

To lessen the risk for comorbidity and death, the Endocrine Society’s newly published guidelines on the treatment of Cushing’s syndrome focus on surgical resection of the causal tumor with the goal of normalizing cortisol levels. Furthermore, there is increased emphasis on individualizing treatment options when choosing a second-line treatment.

In July 2015, the Endocrine Society published treatment guidelines to assist endocrinologists in appropriately initiating treatment or referring patients with Cushing’s syndrome to treatment. A task force of experts compiled evidence from systematic reviews and graded the strength of the recommendations.

“We hope that it will lead to improved treatment of comorbidities both before and after definitive treatment of the syndrome, and to increased individualization of patient treatment,” said chair of the task force Lynnette Nieman, MD, who is chief of the Endocrinology Consultation Service at the National Institutes of Health Clinical Center.

“There are two new drugs that were approved in 2012, and so I think that is what prompted the review. Still, medications are not the first line of treatment, but we have some new therapeutic options, and I think the idea was to help people understand where to use them,” Julie Sharpless, MD, assistant professor and director of the UNC Multidisciplinary Pituitary Adenoma Program, told Endocrinology Advisor.

“The primary treatment is surgical resection of the causal tumor(s). If that cannot be done (because the tumor is occult or metastatic) or is not successful, then the choice of secondary treatment should be individualized to the patient. The comorbidities of Cushing’s syndrome, for example hypertension and diabetes, should be treated separately as well,” Nieman said.

For example, the guidelines recommend surgical removal of the causative lesion, with the exception of cases which are unlikely to cause a drop in glucocorticoids or in patients who are not surgical candidates.

Likewise, in patients with benign unilateral adrenal adenoma, adrenalectomy by an experienced surgeon has a high rate of cure in children and adults. Because of the poor prognosis associated with adrenal carcinoma, the guidelines highlight the need for complete resection and possibly medical treatment to stabilize cortisol levels.

Other first-line treatment options include recommending surgical resection of ectopic ACTH-secreting tumors; referring to an experienced pituitary surgeon for transsphenoidal selective adenomectomy; treatments to block hormone receptors in bilateral micronodular adrenal hyperplasia; and surgical removal in bilateral adrenal disorders.

The elevated mortality rate seen in patients with Cushing’s syndrome is due to infection, venous thrombosis and cardiovascular disease (CVD). Appropriately lowering cortisol levels improves hypertension, insulin resistance, dyslipidemia and obesity in patients with Cushing’s syndrome. Therefore, the guidelines highlight the need for restoring cortisol levels and treating the associated comorbidities.

Nevertheless, the task force specifically recommends against treatment without an established diagnosis or when there are no signs of Cushing’s syndrome and hypothalamic-pituitary-adrenal laboratory studies are borderline.

In patients who are not surgical candidates or in cases of noncurative resection, the decision on whether to consider second-line treatment options such as medical therapy, radiation, bilateral adrenalectomy or repeat transsphenoidal surgery should be based on several factors. For instance, the guidelines recommend taking into consideration location and size of the tumor, patient desires, goals of treatment and level of biochemical control.

The guidelines note medical therapy should be based on cost, efficacy and individualization of treatment. Endocrinologists can approach medical therapy with a goal of establishing normal cortisol levels or reducing cortisol levels to very low levels and replacing to achieve desired levels.

Remission in Cushing’s syndrome is associated with notable improvement; however, long-term follow-up is recommended for osteoporosis, CVD and psychiatric conditions.

After treatment, patients may experience reductions in weight, blood pressure, lipids and glucose levels that may allow reduction or discontinuation of medications. Even so, patients with a history of Cushing’s syndrome tend to have higher rates of hypertension, hyperlipidemia and diabetes. Likewise, rates of myocardial infarction are higher in this population, further emphasizing the need for treatment and management of diabetes and hypertension.

Sharpless highlighted that Cushing’s syndrome is rare.

“There are multiple studies that have shown that patients do better when they are treated in a specialty center where people see a lot of cases of this. So in that sense, treatment is not usually going to fall to the general practitioner,” she said.

She continued that the guidelines are helpful and provide guidance to endocrinologist who “can’t readily refer their patient to a pituitary center.”

Sharpless went on to describe the multidisciplinary care involved in Cushing’s syndrome including endocrinologists, neurosurgeons, radiologists, counselors and radiation oncologist.

“When the care is complicated, you want to ensure all of your providers have reviewed your case together and figured out the best plan.”

The guidelines were co-sponsored by the European Society of Endocrinology. Nieman received salary support for her work on the manuscript from the Intramural Research Program of the Eunice Kennedy Shiver Institute of Child Health and Human Development. Members of the task force reported multiple disclosures.

Reference

  1. Nieman LK et al. J Clin Endocrinol Metab. 2015;100(8):2807-2831.

From http://www.endocrinologyadvisor.com/adrenal/cushings-syndrome-endocrine-society-guidelines/article/434307/

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Given Adrenal Symptoms, Blood Test Recommended

Posted on July 28, 2015 by MaryO

adrenal-glands

 

Q: My husband’s recent CT scan of his stomach and digestive system revealed that he has nodules on both adrenal glands. It was suggested that he undergo a blood test to determine whether the nodules are producing hormones.

For 21 months, he has experienced high blood pressure, nausea, diarrhea, anxiety and abdominal pain. Could this be the source of his problems? If so, what course of action would you recommend?

A: The adrenal gland is responsible for the production of several essential hormones.

Tumors, or nodules, of the adrenal glands are common. They can be categorized into those that make hormones and those that don’t, and also by whether the tumors are benign or malignant.

The most common, by far, are benign, nonfunctioning tumors. These are usually discovered on an ultrasound or a CT scan obtained for some other reason.

More than 4 percent of people have an adrenal mass, and 85 percent are nonfunctional.

The symptoms that your husband has, however, raise a concern that he might have a hormone-producing tumor.

Four types of hormones are commonly produced by adrenal tumors: cortisone, aldosterone, sex hormones (estrogen or androgens) and catecholamines (epinephrine and norepinephrine).

A cortisone-producing adrenal tumor causes Cushing’s syndrome. It usually causes weight gain, especially in the abdomen; skin changes, including striae, or “stretch marks”; high blood pressure; and a predisposition to diabetes. Anxiety and abdominal pain are uncommon.

Aldosterone raises blood pressure, so a person with a functioning adrenal tumor making aldosterone usually has high blood pressure, but the other symptoms you mention for your husband aren’t common for this type of tumor.

Adrenal tumors that make epinephrine and the related norepinephrine are called pheochromocytomas. Hypertension is almost universal with this condition, and anxiety is frequently reported.

Tumors that produce sex hormones are rare, and they are present in men with androgen excess or feminization, in the case of estrogen-secreting tumors.

Although your husband’s symptoms aren’t specific for any one condition, the combination of his symptoms and adrenal nodules concerns me.

I agree with the recommendation to look for excess amounts of hormones in the blood. This can often be achieved with a simple blood test; however, a catheter is occasionally placed in the adrenal vein to sample blood coming from the gland (and its nodule) directly.

By comparing one side against the other, doctors can determine which side might be producing excess hormones.

An endocrinologist is the expert most likely to be familiar with these conditions.

Dr. Roach answers letters only in his North America Syndicate column but provides an order form of available health newsletters at http://www.rbmamail.com. Write him at 628 Virginia Dr., Orlando, FL 32853-6475; or ToYour GoodHealth@med. cornell.edu.

From http://www.dispatch.com/content/stories/life_and_entertainment/2015/07/27/given-adrenal-symptoms-blood-test-recommended.html

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HSA issues alert on 2 illegal pain relief products

Posted on June 4, 2015 by MaryO

SINGAPORE: The Health Sciences Authority (HSA) on Wednesday (Jun 3) issued an alert on two illegal health products sold in Singapore, one of which caused a consumer to be hospitalised for adverse reactions.

HSA added that investigations are ongoing and laboratory tests have found undeclared potent medicinal ingredients in the products.

A woman in her 40s has been hospitalised for “delirium (confusion), high blood sugar, electrolyte imbalance in her blood and suspected Cushing’s syndrome (characterised by a round face or ‘moon face’ and upper body obesity within thin limbs)” after consuming an illegal pain relief product, said HSA.

The woman had been consuming the “powder packed in unlabelled pink sachets” for pain relief over a prolonged period of time because she believed it had “miraculous powers”, HSA added. The powder was found to contain the illegal ingredients Chlorpheniramine and Dexamethasone.

The patient had bought the powder from “an auntie who sells powdered medicine” – a woman in her 60s who would personally deliver the products to the addresses provided by the customers, said HSA.

HSA raided the peddler’s home and discovered another illegal health product – black pills labelled “special effect rheumatism pill”. They were found to contain Dexamethasone and Chlorpheniramine, as well as Bromhexine.

Dexamethasone could cause diabetes, high blood pressure, cataracts and Cushing’s syndrome, among other adverse reactions. Chlorpheniramine and Bromhexine could cause symptoms including diarrhoea, nausea and vomiting.

The authority advised members of the public who have purchased or are consuming the products to:

Consult a doctor as soon as possible before stopping usage as sudden discontinuation of steroids without proper medical supervision can cause serious withdrawal symptoms

Avoid buying health products from street peddlers, unknown websites or sellers, or even from well-meaning friends or relatives

Be wary of health products not properly labelled with ingredient information or make exaggerated claims like “instant pain relief”

Seek appropriate medical treatment from a doctor for the management of acute and chronic medical conditions such as arthritis and rheumatism

via HSA issues alert on 2 illegal pain relief products – Channel NewsAsia.

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Enzyme that triggers muscle wasting could be key to REVERSING signs of ageing | Daily Mail Online

Posted on May 21, 2015 by MaryO

Drawing on expertise from both the University of Birmingham and Queen Elizabeth Hospitals Birmingham, they applied their knowledge of Cushing’s syndrome to the new problem (sarcopenia).

Cushing’s is hormonal disorder caused by high levels of cortisol. Patients suffer from the syndrome see marked changes in their body composition.

The effects can be devastating for patients who can develop features such as muscle wasting and weakness, weight gain, thinning of the bones, diabetes, high blood pressure and heart disease.

Dr Hassan-Smith said: ‘Looking at this particular enzyme seemed like an intriguing way forward.

‘We knew how it works in relation to Cushing’s Syndrome, which is characterised by similar symptoms, and thought it would be worthwhile applying what we knew to the ageing population.’

Currently there are no treatments for sarcopenia, the team explained.

But pharmaceutical companies are developing and testing ways to block or switch off the enzyme, with a focus on treatments for conditions including diabetes.

The team is excited about taking the results of their study forward into future research, with one eye on adapting the inhibitors already in development to combat muscle ageing.

Dr Hassan Smith added: ‘The next stage is a “proof of concept” study to look at the effects of these inhibitive pharmaceuticals on muscle function, before opening it up into a clinical trial.

‘It’s an as yet unexplored area that could yield beneficial results for a problem that is becoming more prevalent as our lifespans increase.’

The study was published in the journal of Clinical Endocrinology and Metabolism.

 

via Enzyme that triggers muscle wasting could be key to REVERSING signs of ageing | Daily Mail Online.

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If One Partner Has Cushing’s Syndrome, Can The Couple Still Get Pregnant?

Posted on May 12, 2015 by MaryO

Cushing’s syndrome can affect fertility in both men and women.

Women

The high levels of cortisol in Cushing’s syndrome disrupt a woman’s ovaries. Her menstrual periods may stop completely or become irregular. As a result, women with Cushing’s syndrome almost always have difficulty becoming pregnant.5,6,7 For those who do become pregnant, the risk of miscarriage is high.5,6,7

In rare cases, usually when a woman’s Cushing’s syndrome is caused by a benign adrenal tumor, pregnancy can occur, but it brings high risk for the mother and fetus.5,6,7

After a woman is treated for Cushing’s syndrome, her ovaries often recover from the effects of too much cortisol. Her regular menstrual cycles will return, and she can become pregnant.8

In some women, regular periods do not return after they are treated for Cushing’s syndrome. This occurs if surgery removes the part of the pituitary gland involved in reproduction.4 An infertility specialist can prescribe hormone therapy to bring back regular periods, ovulation, and fertility.8

Men

A man diagnosed with Cushing’s syndrome may have a decline in sperm production and could have reduced fertility.9 He also might experience a lowered sex drive as well as impotence (pronounced IM-puh-tuhns). In addition, some medications used to treat Cushing’s syndrome can reduce fertility.10 However, fertility usually recovers after Cushing’s syndrome is cured and treatment has stopped.9

Does Cushing’s syndrome affect pregnancy?

Cushing’s syndrome can cause serious and potentially life-threatening effects for the mother and the fetus during pregnancy.11,12 For example, Cushing’s syndrome raises a woman’s risk of developing pregnancy-related high blood pressure (called preeclampsia, pronounced pree-i-KLAMP-see-uh, or eclampsia) and/or pregnancy diabetes, which also is called gestational (pronounced je-STEY-shuhn-ul) diabetes). Infection and slow healing of any wounds are more likely, as is heart failure. When the syndrome is caused by a tumor, it will be surgically removed as early as possible to reduce any threat.13

  1. Margulies, P. (n.d.). Adrenal diseases—Cushing’s syndrome: The facts you need to know. Retrieved May 21, 2012, from National Adrenal Diseases Foundation website http://www.nadf.us/adrenal-diseases/cushings-syndrome/ External Web Site Policy
  2. Nieman, L. K., & Ilias, I. (2005). Evaluation and treatment of Cushing’s syndrome. Journal of American Medicine, 118(12), 1340-1346. PMID 16378774.
  3. American Cancer Society. (n.d.). Fact sheet on pituitary tumors. Retrieved May 19, 2012, fromhttp://documents.cancer.org/acs/groups/cid/documents/webcontent/003133-pdf.pdf (PDF – 171 KB). External Web Site Policy
  4. Biddie, S. C., Conway-Campbell, B. L, & Lightman, S. L. (2012). Dynamic regulation of glucocorticoid signalling in health and disease. Rheumatology, 51(3), 4034-4112. Retrieved May 19, 2012, from PMID: 3281495.
  5. Abraham, M. R., & Smith, C. V. (n.d.). Adrenal disease and pregnancy.Retrieved April 8, 2012, fromhttp://emedicine.medscape.com/article/127772-overview – aw2aab6b6. External Web Site Policy
  6. Pickard, J., Jochen, A. L., Sadur, C. N., & Hofeldt, F. D. (1990). Cushing’s syndrome in pregnancy. Obstetrical & Gynecological Survey, 45(2), 87-93.PMID 2405312.
  7. Lindsay, J. R., Jonklaas, J., Oldfield, E. H., & Nieman, L. K. (2005). Cushing’s syndrome during pregnancy: Personal experience and review of the literature. Journal of Clinical Endocrinology and Metabolism, 90(5), 3077.PMID 15705919.
  8. Klibansky, A. (n.d.). Pregnancy after cure of Cushing’s disease. Retrieved April 27, 2012, fromhttp://03342db.netsolhost.com/page/pregnancy_after_cure_of_cushings_disease.php. External Web Site Policy
  9. Jequier, A.M. Endocrine infertility. In Male infertility: A clinical guide (2nd ed.). Cambridge University Press, 2011: chap 20, pages 187-188. Retrieved May 19, 2012, from http://books.google.com/books?id=DQL0YC79uCMC&pg=PA188&lpg=PA188&dq=male+infertility+causes+and+treatment+Cushing&source=bl&ots=k1Ah5tVJC7&sig=WJR4N0wUawlh0Rant31QMPq6ufs&hl=en&sa=X&ei=hGe5T-LrHYSX6AHgrvmzCw&ved=0CGoQ6AEwAQ#v=onepage&q=male%20infertility%20causes%20and%20treatment%20Cushing&f=false. External Web Site Policy
  10. Stewart, P. M., & Krone, N. P. (2011). The adrenal cortex. In Kronenberg, H. M., Shlomo, M., Polonsky, K. S., Larsen P. R. (Eds.). Williams textbook of endocrinology (12th ed.). (chap. 15). Philadelphia, PA: Saunders Elsevier.
  11. Abraham, M. R., & Smith, C. V. Adrenal disease and pregnancy. Retrieved April 8, 2012, from http://emedicine.medscape.com/article/127772-overview – aw2aab6b6. External Web Site Policy
  12. Buescher, M. A. (1996). Cushing’s syndrome in pregnancy. Endocrinologist, 6, 357-361.
  13. Ezzat, S., Asa, S. L., Couldwell, W. T., Barr, C. E., Dodge, W. E., Vance M. L., et al. (2004). The prevalence of pituitary adenomas: A systematic review.Cancer, 101(3), 613-619. PMID 15274075.

From https://www.nichd.nih.gov/health/topics/cushing/conditioninfo/pages/faqs.aspx

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