Masked renal dysfunction in patients with adrenal Cushing’s syndrome manifested by adrenalectomy

Posted on May 31, 2016 by MaryO

INTRODUCTION AND OBJECTIVES

Many patients with primary aldosteronism (PA) exhibit a decline in renal function after adrenalectomy. Excessive aldosterone secretion causes glomerular hyperfiltration, and cancellation of this excessive secretion manifests the masked renal dysfunction. Considering the mineralocorticoid effect of cortisol as with aldosterone, excessive cortisol secretion may also mask the renal dysfunction of patients with adrenal Cushing’s syndrome (CS). However, postoperative changes in renal function in patients with CS have not been evaluated. We evaluated changes in renal function after adrenalectomy in patients with functional adrenal tumor.

METHODS

A total of 164 consecutive patients underwent adrenalectomy for unilateral functional adrenal tumor at our institution between January 2004 and October 2014. Of the 164 patients, we retrospectively analyzed 118 patients (PA/CS/subclinical Cushing’s syndrome [SCS]/pheochromocytoma [PCC]: n = 51/21/13/33, respectively) who were followed up for over 6 months and whose change in renal function was evaluable. Laboratory data, including baseline hormone levels before medical treatment, were collected from medical records. Estimated glomerular filtration rate (eGFR) values were obtained using the 3-variable Japanese equation. Renal dysfunction was defined as a 20% reduction in eGFR from baseline to within 3 times average during the 6 months post-operation. The change in eGFR before and after the operation was analyzed in each adrenal tumor.

RESULTS

The mean change in eGFR before and after the operation was -14% (range, -55%-18%) in patients with PA, -9% (-48%-23%) in patients with CS, -2% in patients with SCS, and -2% in patients with PCC. The eGFR decline in patients with PA and CS was significant (p < 0.0001 and p = 0.0171, respectively), while there was no significant change in patients with SCS and PCC. After the operation, 39% (20/51) and 24% (5/21) of patients with PA and CS manifested renal dysfunction, respectively, while none of the patients with SCS and PCC manifested renal dysfunction. Preoperative renal function was not correlated with manifested renal dysfunction. Multivariate analysis identified older age and higher levels of plasma aldosterone concentration as independent predictors of renal dysfunction manifestation in patients with PA, while no clinical predictor was identified in patients with CS.

CONCLUSIONS

This is the first report that has shown a decline in eGFR after adrenalectomy in patients with CS. Possible masked renal damage should be considered in patients with CS as well as PA.

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Patients with ARMC5 mutations: The NIH clinical experience

Posted on May 27, 2016 by MaryO

Screenshot 2016-05-27 13.12.55

 

Adrenal Disorders

R Correa, M Zilbermint, A Demidowich, F Faucz, A Berthon, J Bertherat, M Lodish, C Stratakis

Summary: Researchers conducted this study to describe the different phenotypical characteristics of patients with armadillo repeat containing 5 (ARMC5) mutations, located in 16p11.2 and a likely tumor-suppressor gene. They determined that patients with bilateral adrenal enlargement, found on imaging tests, should be screened for ARMC5 mutations, which are associated with subclinical Cushing’s syndrome (CS) and primary hyperaldosteronism (PA).

Methods:

  • Researchers identified 20 patients with ARMC5 mutations (germline and/or somatic) who were enrolled in a National Institutes of Health (NIH) protocol.
  • They obtained sociodemographic, clinical, laboratory, and radiological data for all participants.

Results:

  • Three families (with a total of 8 patients) were identified with ARMC5 germline mutations; the rest of the patients (13/20) had sporadic mutations.
  • The male to female ratio was 1.2:1; mean age was 48 years and 60% of patients were African American.
  • Forty percent of patients were diagnosed with CS, 20% with subclinical CS, 30% with hyperaldosteronism, and 10% had no diagnosis.
  • The mean serum cortisol (8 am) and Urinary Free Cortisol were 13.1 mcg/dl and 77 mcg/24 hours, respectively.
  • Nearly all patients (95%) had bilateral adrenal enlargement found on CT or MRI.
  • Patients underwent the following treatments: Bilateral adrenalectomy (45%), unilateral adrenalectomy (25%), medical treatment (20%), and no treatment (10%).
  • ARMC5 mutations are associated with primary macronodular adrenal hyperplasia (PMAH) and are also seen in patients with PA, especially among African Americans.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ37C4C5D3BF1A4FAE9C479A696660535B/57884/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-AACE2016&nonus=0

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Delayed diagnosis, barriers to care increase morbidity in children with Cushing’s syndrome

Posted on May 6, 2016 by MaryO

Hispanic and black children diagnosed with Cushing’s syndrome are more likely to present with higher cortisol measurements and larger tumor size vs. white children, according to study findings presented at the annual Pediatric Academic Societies Meeting in Baltimore.

“Racial and socioeconomic disparities may contribute to the severity of disease presentation for children with Cushing’s [syndrome],” Alexandra Gkourogianni, MD, of the section on endocrinology and genetics at the Eunice Kennedy Shriver National Institute of Child Health and Human Development, and colleagues wrote. “Minority children from disadvantaged backgrounds present more frequently with comorbidities associated with longstanding [Cushing’s syndrome].”

Gkourogianni and colleagues analyzed data from 135 children treated for Cushing’s syndrome (transsphenoidal surgery) at the NIH between 1997 and 2015 (mean age, 13 years; 51% girls; 33% Hispanic or black). Researchers used a 10-point index for rating severity in pediatric Cushing’s syndrome based on predefined cutoffs; degree of hypercortisolemia, impaired glucose tolerance, and hypertension were graded on a 3-point scale (0-2); height, BMI z scores, duration of disease, and tumor invasion were graded on a 2-point scale (0-1).

Researchers found that midnight cortisol measurements were higher among Hispanic and black children vs. white children (23.3 µg/dL vs. 16 µg/dL; P = .019), as were tumor sizes (mean 6.3 mm vs. 3.3 mm; P = .016). Height standard deviation score was more severely affected in black and Hispanic children (–1.6 vs. –1.1; P = .038), and mean Cushing’s syndrome score for Hispanic and black children was higher vs. white children (4.5 vs. 3.8; P = .033).

Researchers found that median income had an independent correlation with Cushing’s syndrome score in univariate regression analysis for covariates of socioeconomic status and demographics (P = .025). Multivariable regression analysis using race, prevalence of obesity, estimated income, access to pediatric endocrinologist, age and sex confirmed that race, along with lower socioeconomic status and older age, were predictors of a higher Cushing’s syndrome score (P = .002).

“We speculate that delayed diagnosis, barriers to access to care and poorer quality health care for these underserved patients may contribute to presentation at a later age and increased morbidity,” the researchers wrote. “Additional research is needed to identify potential modifiable factors that may improve care for these patients.” – by Regina Schaffer

Reference:

Gkourogianni A, et al. Poster #445. Presented at: Pediatric Academic Societies Meeting; April 30- May 3, 2016; Baltimore.

Disclosure: Endocrine Today was unable to determine relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7Be79d7c84-d539-4a04-a548-882b9f4caadd%7D/delayed-diagnosis-barriers-to-care-increase-morbidity-in-children-with-cushings-syndrome

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Woman diagnosed with Cushing’s syndrome

Posted on May 6, 2016 by MaryO
Thursday, May 05, 2016
AsiaOne

SINGAPORE – The Health Sciences Authority (HSA) has warned the public not to purchase or consume a health product labelled in Chinese as ‘Hai Leng Hai Beh Herbal Itch Removing Capsule’ after a customer consumed it and has now been diagnosed with a metabolic disorder known as Cushing’s syndrome.

In a press release today (May 5), the HSA revealed that a woman in her 40s, bought the capsules from a retail store overseas to ease an itchy skin condition after a relative’s recommendation.

Just a day after consumption her itchy skin condition improved. However after consuming the pills for two months, she gained weight rapidly and her face became puffy.

Evidence later showed that the pills were not entirely herbal in nature and contained “potent traces of steroid and painkillers like paracetemol”, according to HSA.

Dexamethsone, a steroid which is usually prescribed for inflammatory conditions, is known to cause increased blood glucose levels that cause diabetes, high blood pressure, cataracts and muscular and bone disorders when taken over a long period of time without supervision.

These medical conditions may, in turn, cause a consumer to develop Cushing’s syndrome, said HSA.

Chlorpheniramine was also found in the capsule. HSA said the antihistamine is commonly known to treat allergic reactions and can cause drowsiness, blurred vision, vomiting and constipation.

Paracetamol is known to cause swelling of the lips or face and rashes.

The HSA has advised all sellers to stop selling and distributing the capsules immediately.

If caught selling, shop owners could be fined up to $10,000 and may be imprisoned for a period of up to two years, if convicted.

To learn more about the dangers of buying health products from dubious sources, you can visit http://www.healthdangers.sg.

Those with any information on the sale and supply of these capsules or other illegal products can contact HSA’s Enforcement Branch at 68663485 during office hours from Monday to Friday.

mldas@sph.com.sg

– See more at: http://yourhealth.asiaone.com/content/woman-diagnosed-cushings-syndrome-hsa-warns-public-avoid-itch-removing-capsule#sthash.3odVhMMe.dpuf

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Recruitment for Cushing’s Syndrome Clinical Study

Posted on May 2, 2016 by MaryO
DESCRIPTION

This trial is testing the safety and effectiveness of a new investigational drug for the treatment of Cushing’s Syndrome. Under the supervision of qualified physicians, cortisol levels and symptoms of Cushing’s Syndrome will be closely followed along with any signs of side effects.

The link below will take you to the trial website where you can review additional information and the patient screener.

http://curec.lk/1X0J6kT

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