Basal Cortisol Elevated in Patients with ACTH-Staining Pituitary Macroadenoma

Posted on May 9, 2017 by MaryO

Preoperative identification of patients with silent adrenocorticotrophic hormone-secreting tumors could potentially change the approach to management. A new study aimed to determine whether a preoperative adrenocorticotrophic hormone stimulation test for evaluation of nonfunctional pituitary macroadenoma could aid in identifying adrenocorticotrophic hormone-staining pathology yielded large variability and did not allow clinical utility.

Thus, researchers concluded that larger, multicenter research is needed to determine whether this test can be useful.

“As ACTH stimulation tests are performed routinely when evaluating macroadenoma when there is no suspicion for a state of endogenous hypercortisolism, we sought to determine if the test could reliably identify these pathologies during the preoperative evaluation. We hypothesized that patients with subclinical Cushing’s disease or silent ACTH-secreting tumors would have a higher delta cortisol on the ACTH stimulation tests vs. other types of macroadenoma pathologies,” Kevin Pantalone, DO, ECNU, FACE, staff endocrinologist and director of clinical research in the department of endocrinology at Cleveland Clinic, told Endocrine Today.

Pantalone and colleagues performed a retrospective chart review of 148 patients with pituitary macroadenoma who underwent preoperative ACTH stimulation tests, with the goal of determining whether the test can aid in the identification of ACTH-staining pathology.

Overall, 9.5% of patients showed diffuse staining, 50.6% showed other-staining (diffuse staining for anterior pituitary hormones other than ACTH) and 39.9% showed no staining (no staining for any anterior pituitary hormones).

The researchers calculated delta total cortisol at 30 and 60 minutes from baseline and reviewed preoperative ACTH stimulation tests. Additionally, Pantalone and colleagues compared the basal and maximal delta cortisol between the ACTH-staining pituitary macroadenoma and the non-ACTH staining (n = 134), other staining (n = 75) and non-staining (n = 59) tumors.

According to data reported at the American Association of Clinical Endocrinologists Annual Scientific and Clinical Congress, the ACTH-staining group had higher mean basal cortisol levels compared with the non-ACTH-staining (P = .012), other staining (P = .018) and the non-staining (P = .012) tumors. The researchers found no significant differences in maximal delta cortisol between the groups.

“While we found basal cortisol levels were higher in patients with ACTH-staining pituitary microadenoma vs. non-ACTH-staining macroadenoma, the large variability in cortisol values did not allow for clinical utility,” Pantalone told Endocrine Today.

“Unfortunately, in the end, our study was limited by the number of cases with ACTH-staining pathology. Thus, we were unable to determine if the ACTH stimulation test could reliably assist clinicians in potentially identifying ACTH-staining pathology in the preoperative setting,” he said. “A multicenter study, affording a large number of ACTH-staining tumors, is needed. This may allow for us to determine if the ACTH-stimulation test can really be clinically useful in preoperatively identifying ACTH-staining pathology.” – by Amber Cox

 From http://www.checkorphan.org/news/basal-cortisol-elevated-in-patients-with-acth-staining-pituitary-macroadenoma

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Primary Adrenal Insufficiency (PAI)

Posted on May 6, 2017 by MaryO
05.05.2017, 05:18 by SciDoc Publishers International Journal of Clinical Therapeutics and Diagnosis (IJCTD)
 Al-Jurayyan NA
Background: Primary adrenal insufficiency (PAI) in children is an uncommon, but potentially fatal. The current symptoms include weakness, fatigue, anorexia, abdominal pain, weight loss, orthostatic hypotension, salt craving and characterized by hyperpigmentation.
Material and Methods: This is a retrospective, hospital based-study, conducted at King Khalid University Hospital (KKUH), during the period January 1989 and December 2014. Review of medical record of patient diagnosed with primary adrenal insufficiency. The diagnosis was based on medical history, physical examination and low levels of glucocorticoids and raised adrenocorticotropic hormone (ACTH). Appropriate laboratory and radiological investigations were also reviewed.
Results: During the period under review, January 1989 and December 2014, a total of 125 patients with the diagnosis of primary adrenal insufficiency were seen. Inherited disorders like congenital adrenal hyperplasia and hypoplasia were common, 85.5%. However, variable autoimmune mediated etiologic diagnosis accounted for, 13%, were also seen. The appropriate various laboratory and radiological investigations should be planned.
Conclusion: Although, congenital adrenal hyperplasia was the commonest etiology, however, congenital adrenal hypoplasia should not be over looked. The diagnosis of PAI can be challenging in some patients, and therefore appropriate serological and radiological investigations should be done.

REFERENCES

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Topical Steroid Use in Psoriasis Patient Leads to Severe Adrenal Insufficiency

Posted on May 5, 2017 by MaryO

This article is written live from the American Association of Clinical Endocrinologists (AACE) 2017 Annual Meeting in Austin, TX. MPR will be reporting news on the latest findings from leading experts in endocrinology. Check back for more news from AACE 2017.

 

At the AACE 2017 Annual Meeting, lead study author Kaitlyn Steffensmeier, MS III, of the Dayton Veterans Affairs (VA) Medical Center, Dayton, OH, presented a case study describing a patient “who developed secondary adrenal insufficiency secondary to long-term topical steroid use and who with decreased topical steroid use recovered.”

The patient was a 63-year-old white male with a 23-year history of psoriasis. For 18 years, the patient had been applying Clobetasol Propionate 0.05% topically on several areas of his body every day. Upon presentation to the endocrine clinic for evaluation of his low serum cortisol, the patient complained of a 24-pound weight gain over a 2-year period, feeling fatigued, as well as facial puffiness.

Laboratory analysis found that the patient’s random serum cortisol and ACTH levels were low (0.2µg/dL and <1.1pg/mL, respectively). According to the study authors, “the labs were indicative of secondary adrenal insufficiency.” Additionally, a pituitary MRI “showed a 2mm hypoenhancing lesion within the midline of the pituitary gland consistent with Rathke’s cleft cyst versus pituitary microadenoma.”

The patient was initiated on 10mg of hydrocortisone in the morning and 5mg in the evening and was instructed to decrease the use of his topical steroid to one time per month. For the treatment of his psoriasis, the patient was started on apremilast, a phosphodiesterase-4 enzyme (PDE4) inhibitor, and phototherapy.

After 2.5 years, the patient had a subnormal response to the cosyntropin stimulation test. However, after 3 years, a normal response with an increase in serum cortisol to 18.7µg/dL at 60 minutes was obtained; the patient was then discontinued on hydrocortisone. Additionally, a stable pituitary tumor was shown via a repeat pituitary MRI.

The study authors explained that, although secondary adrenal insufficiency is not commonly reported, “one study showed 40% of patients with abnormal cortisol response to exogenous ACTH after two weeks of topical glucocorticoids usage.” Another meta-analysis of 15 studies (n=320) revealed 4.7% of patients developing adrenal insufficiency after using topical steroids. Because of this, “clinicians need to be aware of potential side effects of prolong topical steroid use,” added the study authors.

For continuous endocrine news coverage from the AACE 2017 Annual Meeting, check back to MPR’s AACE page for the latest updates.

From http://www.empr.com/aace-2017/topical-steroid-psoriasis-clobestasol-propionate/article/654335/

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8 Things You Should Know About Addison’s Disease

Posted on February 6, 2017 by MaryO

adrenal-insufficiency

 

Cortisol gets a bad rap these days. (Guilty!) Yes, this hormone surges when you’re stressed. And yes, chronic stress is bad news for your health. But while too much cortisol can lead to all sorts of stress-related side effects, too little cortisol is equally debilitating.

Just ask someone with Addison’s disease. If you suffer from this condition, your adrenal glands fail to make adequate amounts of cortisol, says Betul Hatipoglu, MD, an endocrinologist at Cleveland Clinic.

Cortisol plays a role in regulating your blood pressure, heart function, digestion, and a lot else, Hatipoglu explains. So if your adrenal glands poop out and your cortisol levels plummet, a lot can go wrong. (In as little as 30 days, you can be a whole lot slimmer, way more energetic, and so much healthier just by following the simple, groundbreaking plan in The Thyroid Cure!)

Here’s what you need to know about this condition—starting with its craziest symptom.

It can make your teeth appear whiter.

Hatipoglu once met with a patient who was suffering from fatigue, belly pain, and mild weight loss. “Her doctors thought she was depressed,” Hatipoglu recalls. Toward the end of their appointment, Hatipoglu noticed the woman’s teeth looked very white. She realized they looked white because the woman’s skin was tan. “I asked her if she’d been on vacation, and she said she hadn’t been in the sun, and that’s when I knew,” Hatipoglu says. Some Addison’s-related hormone shifts can make the skin appear darker, almost like a tan. “Addison’s is the only disease I know of that can cause darkening of the skin,” she says.

Its (other) symptoms are popular ones.

 Along with darker skin, other symptoms of Addison’s include nausea, mild-to-severe abdominal or bone pain, weight loss, a lack of energy, forgetfulness, and low blood pressure, Hatipoglu says. Of course, those same symptoms are linked to many other health issues, from thyroid disease to cancer. “It’s very easy to confuse with other disorders, so many people see a lot of doctors before finally receiving a proper diagnosis,” she says. (One exception: For young women who develop Addison’s disease, loss of body hair is a warning sign, Hatipoglu adds.)
It’s rare.
Doctors also miss or misinterpret the symptoms of Addison’s disease because it’s very uncommon. “I’m not sure if it’s quite one in a million, but it’s very rare,” Hatipoglu says. “It makes sense that many doctors don’t think of it when examining a patient with these symptoms.”
It’s often confused with adrenal insufficiency.

A lot of online resources mention Addison’s disease and adrenal insufficiency as though they were two names for the same condition. They’re not the same, Hatipoglu says. While a thyroid issue or some other hormone-related imbalance could mess with your adrenal function, Addison’s disease refers to an autoimmune disorder in which your body attacks and destroys your adrenal glands.

That destruction can happen quickly.

While it takes months or even years for some Addison’s sufferers to lose all hormone production in their adrenal glands, for others the disease can knock out those organs very rapidly—in a matter of days, Hatipoglu says. “That’s very uncommon,” she adds. But compared to other less-severe adrenal issues, the symptoms of Addison’s tend to present more dramatically, she explains. That means a sufferer is likely to experience several of the symptoms mentioned above, and those symptoms will continue to grow worse as time passes.

Anybody can get it.

Addison’s is not picky. It can strike at any age, regardless of your sex or ethnicity, Hatipoglu says. While there’s some evidence that genetics may play a role—if other people in your family have the disease or some other endocrine disorder, that may increase your risk—there’s really no way to predict who will develop the disease, she adds.

Screening for Addison’s is pretty simple.

If your doctor suspects Addison’s, he or she will conduct a blood test to check for your levels of cortisol and another hormone called ACTH. “Usually the results of that screening are very clear,” Hatipoglu says. If they’re not, some follow-up tests can determine for sure if you have the condition.

There are effective treatments.

Those treatments involve taking oral hormone supplements.  In extreme cases, if the patient’s body does not properly absorb those supplements, injections may be necessary, Hatipoglu explains. “But patients live a normal life,” she adds. “It’s a treatable disease, and the treatments are effective.”

From http://www.prevention.com/health/addisons-disease-symptoms

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Bilateral testicular tumors resulting in recurrent Cushing’s syndrome after bilateral adrenalectomy

Posted on December 21, 2016 by MaryO
Troy Puar1,2, Manon Engels3,4, Antonius E. van Herwaarden4, Fred C. G. J. Sweep4, Christina Hulsbergen-van de Kaa5, Karin Kamphuis-van Ulzen6, Vasileios Chortis7,8, Wiebke Arlt7,8, Nike Stikkelbroeck1, Hedi L Claahsen–van der Grinten3, Ad R.M.M. Hermus1
Corresponding author: Troy Puar, MRCP (UK), Department of Medicine, Div. of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands. Phone: +31 243614599, Fax: +31 243618809, e-mail: Troy_puar@cgh.com.sg
DOI: http://dx.doi.org/10.1210/jc.2016-2702
Received: July 14, 2016
Accepted: November 29, 2016
First Published Online: November 30, 2016
Abstract
Context:

Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing’s disease is extremely rare.

Patient:

We present a rare case of a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing’s disease with complete clinical resolution. Cushingoid features recurred 12 years later, along with bilateral testicular enlargement. Hormonal tests confirmed ACTH-dependent Cushing’s. Surgical resection of the testicular tumors led to clinical and biochemical remission.

Design and Results:
Gene expression analysis of the tumor tissue by qPCR showed high expression of all key steroidogenic enzymes. Adrenocortical-specific genes were 5.1 x 105 (CYP11B1), 1.8 x 102 (CYP11B2) and 6.3 x 104 (MC2R) times higher than non-steroidogenic fibroblast control. This correlated with urine steroid metabolome profiling showing 2-5 fold increases in the excretion of the metabolites of 11-deoxycortisol, 21-deoxycortisol and total glucocorticoids. Leydig-specific genes were 4.3 x 101 (LHCGR) and 9.3 x 100(HSD17B3) times higher than control and urinary steroid profiling showed 2-fold increased excretion of the major androgen metabolites androsterone and etiocholanolone. These distinctly increased steroid metabolites were suppressed by dexamethasone, but unresponsive to hCG stimulation, supporting the role of ACTH, but not LH, in regulating tumor-specific steroid excess.
Conclusion:

We report bilateral testicular tumors occurring in a patient with recurrent Cushing’s disease 12 years after bilateral adrenalectomy. Using mRNA expression analysis and steroid metabolome profiling, the tumors demonstrated both adrenocortical and gonadal steroidogenic properties, similar to testicular adrenal rest tumors found in patients with congenital adrenal hyperplasia. This suggests the presence of pluripotent cells even in patients without CAH.

Affiliations
  • 1Department of Medicine, Division of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 2Department of Endocrinology, Changi General Hospital, Singapore 529889, Singapore.
  • 3Department of Paediatrics, Division of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 4Department of Laboratory Medicine, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 5Department of Pathology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 6Department of Radiology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 7Institute of Metabolism and Systems Research, University of Birmingham, Birmingham N15 2TT, United Kingdom.
  • 8Centre for Endocrinology, Diabetes, and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, United Kingdom.

– See more at: http://press.endocrine.org/doi/abs/10.1210/jc.2016-2702#sthash.F4lfWg9j.dpuf

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