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

Role of radiosurgery in management of pituitary adenoma-The BNI experience

Posted on May 30, 2016 by MaryO

Screenshot 2016-05-27 13.12.55

 

S Meah, E Youssef, W White

Summary: Researchers conducted this study to determine the efficacy of stereotactic radiosurgery with CyberKnife for the treatment of recurrent pituitary adenoma. They concluded that stereotactic radiosurgery for recurrent/residual pituitary adenomas using CyberKnife appears to be relatively safe and effective when compared to conventional radiotherapy.

Methods:

  • Included in this retrospective study were patients who underwent cyberKnife radiosurgery for recurrent or residual pituitary adenoma at Barrow Neurological Institute (n=48).
  • Patients were followed for an average of 44 months.
  • Thirty-three patients had non-functioning adenomas, 10 had acromegaly, and 5 had Cushing’s disease.
  • Researchers analyzed the change in tumor size, hormonal function, and complication of therapy for each patient.

Results:

  • Tumor resection operation was performed through either the transsphenoidal or transcranial approach before CyberkKnife treatment.
  • All patients had either recurrence or residual mass in the cavernous sinus before CyberKnife treatment.
  • The total irradiation dose ranged from 2100-4000 cGy in an average of 3-5 fractions.
  • Slightly more than half of the patients (n=26, 54.2%) had smaller tumors at follow-up, while 22 (45.8%) had stable tumors.
  • Visual acuity remained unchanged post-treatment.
  • One patient developed radiation-induced temporal lobe necrosis.
  • Four patients (8.3%) required hormonal replacement due to panhypopituitarism.
  • Of the 15 patients with functioning adenoma, hormonal function improved in 12.
  • Treatment failed in 1 patient with acromegaly, 2 patients with Cushing’s disease, and 1 patient with non-functioning adenoma.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZFEBE5A85394340E188330278A399E6CF/57978/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-AACE2016&nonus=0

Filed under: 25th Annual Congress of the American Association of Clinical Endocrinologists, Cushing's, Meetings and Conferences, Treatments | Tagged: , , , , , | Leave a comment »

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

Filed under: 25th Annual Congress of the American Association of Clinical Endocrinologists, adrenal, Cushing's, Meetings and Conferences, Rare Diseases, Treatments | Tagged: , , , , , , , , , , , , , , , , | Leave a comment »

Cushing’s disease recurrence predictive factors: Outcome analysis of patients in VANCOUVER over 30 years

Posted on May 27, 2016 by MaryO

Screenshot 2016-05-27 13.12.55

 

Pituitary Disorders/Neuroendocrinology

R Chen, J Levi, M Almalki, S Yi, M Johnson, E Ur

Summary: The objective of this study was to describe the management and outcomes of patients with Cushing’s disease (CD) in the Vancouver region over a 30-year period and to investigate the predictive factors of CD recurrence. Researchers found CD recurrence in 45.8% of patients who received initial transsphenoidal surgery (TSS), and that a post-operative serum cortisol level > 140nmol/L may be a positive predictor of recurrence in these patients.

Methods:

  • Researchers retrospectively reviewed the clinical charts from endocrinologists in Vancouver who provided consent to participate in this study.
  • Included in this study were 48 patients diagnosed with CD since 1985.

Results:

  • All 48 patients received initial TSS; the mean follow-up time was 11.73 (±6.98) years.
  • More than half of the patients (n=26, 54.2%) remained in remission, and 22 patients (45.8%) received subsequent interventions due to CD recurrence.
  • Second-line therapies included repeat TSS (40.9%), stereotactic radiotherapy (18.2%), and bilateral adrenalectomy (36.4%).
  • Among patients with disease recurrence, the average post-operative serum cortisol level was significantly higher (489.0 nmol/L vs 114.7nmol/L; P=0.003).
  • The positive predictive value for recurrence with post-operative serum cortisol > 140nmol/L was 76.5% (P=0.049), while serum cortisol < 140nmol/L had an 80% predictive value for non-recurrence (P=0.035).

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ37C4C5D3BF1A4FAE9C479A696660535B/57894/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-AACE2016&nonus=0

Filed under: 25th Annual Congress of the American Association of Clinical Endocrinologists, Cushing's, Meetings and Conferences, Treatments | Tagged: , , , , , , , , , , | Leave a comment »

Rally for Medical Research Hill Day

Posted on May 26, 2016 by MaryO

Held every September, this Capitol Hill Day event continues the momentum established in 2013, and includes nearly 300 national organizations coming together in support of the Rally for Medical Research.

The purpose of the Rally is to call on our nation’s policymakers to make funding for National Institutes of Health (NIH) a national priority and raise awareness about the importance of continued investment in medical research that leads to MORE PROGRESS, MORE HOPE and MORE LIVES SAVED.

The next Rally for Medical Research Hill Day is Sept. 22, 2016.

Sign up to receive updates, including a link to register once it becomes available.

Filed under: Clinical trials, General Health, Health Care, Meetings and Conferences, Rare Diseases | Tagged: , , , | Leave a comment »

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