Resolution of the physical features of Cushing’s syndrome in a patient with a cortisol secreting adrenocortical adenoma after unilateral adrenalectomy

Posted on May 1, 2016 by MaryO

A 37-year-old woman developed clinical manifestations of Cushing’s syndrome over a span of 2 years. Physical examination revealed features that best describe Cushing’s syndrome, such as wide purple striae (>1 cm) over the abdomen, facial plethora and easy bruisability.1  Other features observed were hypertension, moon facies, acne, a dorsocervical fat pad, central obesity and dyslipidaemia.

The diagnosis of hypercortisolism was confirmed using a 1 mg overnight dexamethasone suppression test (19.7 ng/dL, N: <1.8) and 24 h urine free cortisol (185.9 μg/24 h, N: 3.5–45). A suppressed adrenocorticotropic hormone (ACTH) level (4 pg/mL, N: 5–20) and a lack of hyperpigmentation suggested ACTH-independent Cushing’s syndrome. Further work up using CT with contrast of the adrenals showed a 2.4×2.3×2.4 cm right adrenal mass. The patient then underwent laparoscopic adrenalectomy of the right adrenal gland. Steroids was started postoperatively and tapered over time. Histopathology results were consistent with an adrenocortical adenoma (2.5 cm widest dimension). Six months after surgery, there was resolution of the physical features, weight loss and improvement in blood pressure.

Figure 1 is a serial photograph of the physical features seen in Cushing’s syndrome, such as moon facies, a dorsocervical fat pad and wide purple striae, taken preoperatively, and at 3 and 6 months after surgery. With treatment, physical and biochemical changes of Cushing’s syndrome both resolve through time.2 The time course of the resolution of these changes, however, is varied.2 ,3 We observed that the physical features were ameliorated at 3 months and resolved at 6 months.

Learning points

  • Physicians as well as patients should be aware that improvement of the features of Cushing’s syndrome after treatment does not occur immediately.

  • Dramatic resolution of the physical features of Cushing’s syndrome, however, can be observed as early as 6 months after surgery.

Figure 1

View larger version:

Figure 1

Physical features of Cushing’s syndrome (top to bottom: moon facies, a dorsocervical fat pad and wide purple striae (>1 cm) over the abdomen) documented before surgery, and at 3 and 6 months after surgery.

Footnotes

  • Twitter Follow John Paul Quisumbing at @jpquisumbingmd

  • Contributors JPMQ worked up the case and wrote the case report. MASS reviewed the case report and critically appraised it. JPMQ incorporated his suggestions.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

  1. ↵
    1. Ross EJ,
    2. Linch DC

    . Cushing’s syndrome-killing disease: discriminatory value of signs and symptoms aiding early diagnosis.Lancet 1982;2:646–9. doi:10.1016/S0140-6736(82)92749-0

    [CrossRef][Medline][Web of Science]
  2. ↵
    1. Sippel RS,
    2. Elaraj DM,
    3. Kebebew E, et al

    . Waiting for change: symptom resolution after adrenalectomy for Cushing’s syndrome. Surgery2008;144:1054–61. doi:10.1016/j.surg.2008.08.024

    [CrossRef][Medline][Web of Science]
  3. ↵
    1. Mishra AK,
    2. Agarwal A,
    3. Gupta S, et al

    . Outcome of adrenalectomy for Cushing’s syndrome: experience from a tertiary care center. World J Surg2007;31:1425–32. doi:10.1007/s00268-007-9067-6

    [CrossRef][Medline]

From http://casereports.bmj.com/content/2016/bcr-2016-215693.short?rss=1

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Straight Talk Cushing’s

Posted on April 8, 2016 by MaryO

awareness

 

On April 8th, people from around the world unite in support of Cushing’s Disease Awareness Day.

In this animated video, learn more about Cushing’s disease, including the mechanism of disease, clinical manifestations, symptoms and diagnosis challenges.

Filed under: adrenal, Cushing, Cushing's, Diagnostic Testing, pituitary, Treatments | Tagged: , , , | Leave a comment »

Morning Cortisol Rules Out Adrenal Insufficiency

Posted on April 7, 2016 by MaryO

endo2016

 

Key clinical point: Skip ACTH stimulation if morning serum cortisol is above 11.1 mcg/dL.

Major finding: A morning serum cortisol above 11.1 mcg/dL is a test of adrenal function with 99% sensitivity.

Data source: Review of 3,300 adrenal insufficiency work-ups.

Disclosures: There was no outside funding for the work, and the investigators had no disclosures.

BOSTON – A random morning serum cortisol above 11.1 mcg/dL safely rules out adrenal insufficiency in both inpatients and outpatients, according to a review of 3,300 adrenal insufficiency work-ups at the Edinburgh Centre for Endocrinology and Diabetes.

The finding could help eliminate the cost and hassle of unnecessary adrenocorticotropic hormone (ACTH) stimulation tests; the investigators estimated that the cut point would eliminate almost half of them without any ill effects. “You can be very confident that patients aren’t insufficient if they are above that line,” with more than 99% sensitivity. If they are below it, “they may be normal, and they may be abnormal.” Below 1.8 mcg/dL, adrenal insufficiency is almost certain, but between the cutoffs, ACTH stimulation is necessary, said lead investigator Dr. Scott Mackenzie, a trainee at the center.

In short, “basal serum cortisol as a screening test … offers a convenient and accessible means of identifying patients who require further assessment,” he said at the annual meeting of the Endocrine Society.

Similar cut points have been suggested by previous studies, but the Scottish investigation is the first to validate its findings both inside and outside of the hospital.

The team arrived at the 11.1 mcg/dL morning cortisol cut point by comparing basal cortisol levels and synacthen results in 1,628 outpatients. They predefined a sensitivity of more than 99% for adrenal sufficiency to avoid missing anyone with true disease. The cut point’s predictive power was then validated in 875 outpatients and 797 inpatients. Morning basal cortisol levels proved superior to afternoon levels.

The investigators were thinking about cost-effectiveness, but they also wanted to increase screening. “We may be able to reduce the number of adrenal insufficiency cases we are missing because [primary care is] reluctant to send people to the clinic for synacthen tests” due to the cost and inconvenience. As with many locations in the United States, “our practice is to do [ACTH on] everyone.” If there was “a quick and easy 9 a.m. blood test” instead, it would help, Dr. Mackenzie said.

Adrenal insufficiency was on the differential for a wide variety of reasons, including hypogonadism, pituitary issues, prolactinemia, fatigue, hypoglycemia, postural hypotension, and hyponatremia. Most of the patients were middle aged, and they were about evenly split between men and women.

There was no outside funding for the work, and the investigators had no disclosures.

aotto@frontlinemedcom.com

From http://www.clinicalendocrinologynews.com/specialty-focus/pituitary-thyroid-adrenal-disorders/single-article-page/morning-cortisol-rules-out-adrenal-insufficiency/af59bab2bb014ca9d352c792f9d41653.html

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Cushing’s Syndrome and Primary Adrenal Disorders

Posted on April 5, 2016 by MaryO

endo2016

 

April 03, 2016

Oral Session: Cushing’s Syndrome and Primary Adrenal Disorders

Patients with diabetes mellitus diagnosed with Addison’s disease have a markedly increased additional risk of death

D Chantzichristos, A Persson, B Eliasson, M Miftaraj, S Franzén, R Bergthorsdottir, S Gudbjörnsdottir, A-M Svensson, G Johannsson

Summary: Researchers sought to determine if patients with diabetes (DM) who are diagnosed with Addison’s disease (AD) have an increased risk of mortality (DM+AD). They concluded that patients diagnosed with DM+AD had a nearly 4-fold increased risk of mortality compared to controls.

Methods:

  • Researchers identified patients who were first diagnosed with DM (type 1 or 2) and then AD using both the Swedish National Diabetes Register (NDR) and the National Inpatient Register between January 1st, 1996 and December 31th, 2012.
  • Each patients was matched with 5 controls based on sex, year of birth, type of DM, year when DM was diagnosed, and period of time in NDR were selected in NDR.
  • Researchers obtained causes of death data for both groups during the same time period from the Swedish Register for Cause-Specific Mortality.

Results:

  • A total of 1,355 patients were identified: 226 patients had DM (type 1 or 2) and AD and 1,129 matched DM controls.
  • At baseline, patients with DM+AD and patients with DM had a mean (±SD) age of 52.3 (±20.1) and 54.1 (±18.9) years, respectively.
  • In both groups, 47% were women and 65% had type 1 DM.
  • Mean (±SD) HbA1c at baseline was 7.8% (±3.5%) or 62.0 (±14.7) mmol/mol for the DM+AD group and 7.6% (±3.5%) or 59.6 (±14.7) mmol/mol for the DM controls.
  •  More than one-quarter of patients with DM+AD (64/226, 28%) died vs 112 of 1,129 controls (10%).
  • The estimated relative risk increase (hazard ratio) in overall mortality in the DM+AD group was 3.83 (95% confidence interval, 2.80 to 5.24) compared with the DM controls.
  • There was no significant association between type of DM and gender on relative mortality risk.
  • The most common cause of death in both groups was cardiovascular diseases (33% and 34%, respectively).
  • The second most common cause of death in DM+AD patients was DM and its related complications (23%) and cancer in the DM group (29%).
  • Fourteen percent of DM+AD patients died from cancer.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ6AA1CEC190F5428EA690616DAA054518/56981/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-ENDO2016&nonus=0

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Action For Adrenal Disease

Posted on April 3, 2016 by MaryO
Watch online Saturday April 16 at 1:00 PM eastern at https://plus.google.com/events/cpjbd8celcbfgngp8und662s198?hl=en
Secondary Adrenal Insufficiency and Addison’s Disease can be deadly, mostly because of the lack of education and awareness. We have lost too many and need to be proactive in preventing unnecessary deaths! Join us as we educate on what these diseases are, how easily they can become deadly and preventive measures we can all take to help this community. Brought to you by the National Adrenal Disease Foundation, with speakers who have personal experience with these diseases.

Our program will include:

Senior Administrator Nichole Klute Rushton••• of the Addison’s Disease Support Group (https://www.facebook.com/groups/addisons.support/) on Facebook, will speak in detail about the unfortunate adrenal insufficient patients who have tragically passed, reminding us that the danger of loss of life is a reality for every person with adrenal insufficiency who doesn’t receive the vital hormones they need

Administrator Debby Hunter ••• of the Living With Addison’s Disease Support on Facebook (https://www.facebook.com/groups/LivingWithAddisonsDisease/) who will give us tips on how we can approach our local emergency facilities and hospitals with information about adrenal insufficiency and its care in a crisis situation. She will also share her own personal experience with going through an adrenal crisis.

Deputy Sheriff Chris Spires••• who will speak on life as the husband of an Addison’s disease patient, and share with us how the law enforcement community views adrenal insufficient patients

Melanie Wong ••• National Adrenal Disease Foundation (http://www.NADF.us) Executive Director, who will speak about the recent tragic losses, and the vital importance of reminding the medical community about adrenal insufficiency, as well as NADF’s latest project to get NADF Adrenal Crisis Care posters displayed in every emergency room facility in the United States.

Filed under: adrenal, adrenal crisis, Meetings and Conferences, Rare Diseases | Tagged: , , , | 1 Comment »

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