Export file:

Format

  • RIS(for EndNote,Reference Manager,ProCite)
  • BibTex
  • Text

Content

  • Citation Only
  • Citation and Abstract

A Simple Complex Case: Restoration of Circadian Cortisol Activity

Henry Wellcome Laboratories for Integrative Neurosciences and Endocrinology, Dorothy Hodgkin Building, University of Bristol, Bristol, UK

A 38-year-old librarian with confirmed Carney complex (PRKAR1a mutation) was referred for further evaluation of ACTH-independent Cushing's syndrome. Previously, she was known to have schwannoma (excised), adenomyoepithelioma and normal annual echocardiograms. Over three years prior to current presentation, she had become aware of coarse hair on her chin and abdomen, as well as centripetal weight gain. She had noticed subtle but definite reduction in her girdle muscle strength. She had acquired some mood changes atypical of her personality, and had developed an interrupted sleep pattern. To our knowledge, this is the first published report of circadian RU486 therapy for PPNAD in a patient with Carney complex. It may have been possible to restore low levels surrounding the midnight hours using other agents, but the side-effect profile and lack of significantly elevated levels of cortisol made them less favorable options.
  Figure/Table
  Supplementary
  Article Metrics

Keywords carney complex; cushing's; mifepristone; PRKAR1a; RU486

Citation: Ragini C Bhake, Stafford L Lightman. A Simple Complex Case: Restoration of Circadian Cortisol Activity. AIMS Medical Science, 2015, 2(3): 182-185. doi: 10.3934/medsci.2015.3.182

References

  • 1. Milner MR, Semmes L, Silverman A, et al. (1990) Familial Cushing's Syndrome (“Carney Complex”). New Eng J Med 322 (20): 1469-1470
  • 2. Carney JA, Young Jr, WF (1992) Primary pigmented nodular adrenocortical disease and its associated conditions. The Endocrinologist 2(1): 6-21
  • 3. Stratakis CA (1998) Carney Complex: Diagnosis and management of the complex of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas. Am J Med Genet 80: 183-185    
  • 4. Stratakis CA, Kirschner LS, Carney JA (2001) Clinical and molecular features of the Carney complex: Diagnostic criteria and recommendations for patient evaluation. J Clin Endocr Metab 86(9): 4041-4046
  • 5. Anselmo J, Medeiros S, Carneiro V, et al. (2012) A large family with Carney complex caused byS147G PRKAR1A mutation shows a unique spectrum of disease including adrenocortical cancer. J Clin Endocr Metab 97: 351-359    
  • 6. Bertherat J (2006) Carney complex. Orphanet J Rare Dis 1:21 doi:10.1186/1750-1172-1-21    
  • 7. Stratakis CA, Sarlis N, Kirschner LS, et al. (1999) Paradoxical response to dexamethasone in the diagnosis of primary pigmented nodular adrenocortical disease. Ann Int Med 131: 585-591
  • 8. Sarlis NJ, Chrousos GP, Doppman JL, et al. (1997) Primary pigmented nodular adrenocortical disease: Reevaluation of a patient with Carney complex 27 years after unilateral adrenalectomy. J Clin Endocr Metab 82: 1274-1278    
  • 9. Schteingart DE (2009) Drugs in the medical treatment of Cushing's syndrome. Expert Opinion Emer Drug 14(4): 661-671
  • 10. Porpiglia F, Fiori C, Bovio S, et al. (2004) Bilateral adrenalectomy for Cushing's syndrome: A comparison between laparoscopy and open surgery. J Endoc Invest 27: 654-658    
  • 11. Fleseriu M, Biller BMK, Findling JW, et al. (2012) Mifepristone, a glucocorticoid receptor antagonist, produces clinical and metabolic benefits in patients with Cushing's syndrome. J Clin Endoc Metab 97 (6): 2039-2049
  • 12. Castinetti F, Fassnacht M, Johanssen S, et al. (2009) Merits and pitfalls of mifepristone in Cushing's syndrome. Eur J Endoc 160: 1003-1010
  • 13. Stavreva DA, Wiench M, John S, et al. (2009) Ultradian hormone stimulation induces glucocorticoid receptor-mediated pulses of gene transcription. Nat Cell Biol 11(9): 1093-1102

 

Reader Comments

your name: *   your email: *  

Copyright Info: 2015, Ragini C Bhake, et al., licensee AIMS Press. This is an open access article distributed under the terms of the Creative Commons Attribution Licese (http://creativecommons.org/licenses/by/4.0)

Download full text in PDF

Export Citation

Copyright © AIMS Press All Rights Reserved