Literature DB >> 19289537

The prevalence and characteristic features of cyclicity and variability in Cushing's disease.

Krystallenia I Alexandraki1, Gregory A Kaltsas, Andrea M Isidori, Scott A Akker, William M Drake, Shern L Chew, John P Monson, G Michael Besser, Ashley B Grossman.   

Abstract

OBJECTIVE: Cyclical Cushing's syndrome may render the diagnosis and management of Cushing's disease difficult. The aim of the present study was to investigate the prevalence of cyclicity and variability in patients with Cushing's disease, and to identify putative distinctive features.
DESIGN: Retrospective case-note study.
METHODS: We analysed the case records of 201 patients with Cushing's disease in a retrospective case-note study. Cyclicity was considered as the presence of at least one cycle, defined as a clinical and/or biochemical hypercortisolaemic peak followed by clinical and biochemical remission, followed by a new clinical and/or biochemical hypercortisolaemic peak. The fluctuations of mean serum cortisol levels, as assessed by a 5-point cortisol day curve, defined the variability.
RESULTS: Thirty (14.9%; 26 females) patients had evidence of cyclicity/variability. 'Cycling' patients were older but no difference in sex or paediatric distribution was revealed between 'cycling' and 'non-cycling' patients. The median number of cycles was two for each patient, and 4 years was the median intercyclic period. A trend to lower cure rate post-neurosurgery and lower adenoma identification was observed in 'cycling' compared with 'non-cycling' patients. In multivariate analysis, older patients, longer follow-up, female sex and no histological identification of the adenoma were associated with an increased risk of cyclic disease.
CONCLUSIONS: This large population study reveals that cyclicity/variability is not an infrequent phenomenon in patients with Cushing's disease, with a minimum prevalence of 15%. Physicians should be alert since it can lead to frequent problems in diagnosis and management, and no specific features can be used as markers.

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Year:  2009        PMID: 19289537     DOI: 10.1530/EJE-09-0046

Source DB:  PubMed          Journal:  Eur J Endocrinol        ISSN: 0804-4643            Impact factor:   6.664


  24 in total

Review 1.  Cushing's syndrome: why is diagnosis so difficult?

Authors:  David C Aron
Journal:  Rev Endocr Metab Disord       Date:  2010-06       Impact factor: 6.514

2.  Cyclical Cushing's: how best to catch the ups and downs.

Authors:  Malik Asif Humayun; Tanya Hart; Tristan Richardson
Journal:  BMJ Case Rep       Date:  2017-07-13

3.  The changing faces of corticotroph cell adenomas: the role of prohormone convertase 1/3.

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Journal:  Endocrine       Date:  2016-08-04       Impact factor: 3.633

Review 4.  The ectopic ACTH syndrome.

Authors:  Krystallenia I Alexandraki; Ashley B Grossman
Journal:  Rev Endocr Metab Disord       Date:  2010-06       Impact factor: 6.514

5.  Prolonged remission after long-term treatment with steroidogenesis inhibitors in Cushing's syndrome caused by ectopic ACTH secretion.

Authors:  S T Sharma; L K Nieman
Journal:  Eur J Endocrinol       Date:  2011-12-21       Impact factor: 6.664

Review 6.  Physiological basis for the etiology, diagnosis, and treatment of adrenal disorders: Cushing's syndrome, adrenal insufficiency, and congenital adrenal hyperplasia.

Authors:  Hershel Raff; Susmeeta T Sharma; Lynnette K Nieman
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7.  Delayed remission after transsphenoidal surgery in patients with Cushing's disease.

Authors:  Elena Valassi; Beverly M K Biller; Brooke Swearingen; Francesca Pecori Giraldi; Marco Losa; Pietro Mortini; Douglas Hayden; Francesco Cavagnini; Anne Klibanski
Journal:  J Clin Endocrinol Metab       Date:  2010-01-15       Impact factor: 5.958

Review 8.  Psychosomatic aspects of Cushing's syndrome.

Authors:  Nicoletta Sonino; Francesco Fallo; Giovanni A Fava
Journal:  Rev Endocr Metab Disord       Date:  2010-06       Impact factor: 6.514

9.  Hypercortisolism is associated with increased coronary arterial atherosclerosis: analysis of noninvasive coronary angiography using multidetector computerized tomography.

Authors:  Nicola M Neary; O Julian Booker; Brent S Abel; Jatin R Matta; Nancy Muldoon; Ninet Sinaii; Roderic I Pettigrew; Lynnette K Nieman; Ahmed M Gharib
Journal:  J Clin Endocrinol Metab       Date:  2013-04-04       Impact factor: 5.958

10.  Paradoxical response to dexamethasone and spontaneous hypocortisolism in Cushing's disease.

Authors:  Anurag R Lila; Vijaya Sarathi; Tushar R Bandgar; Nalini S Shah
Journal:  BMJ Case Rep       Date:  2013-01-29
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