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The Diagnosis and Differential Diagnosis of Cushing’s Syndrome and Pseudo-Cushing’s States

Department of Endocrinology, St. Bartholomew’s Hospital, West Smithfield, London, EC1A 7BE, United Kingdom

I. Introduction

II. Definitions and Etiology

III. Diagnostic Overview

IV. Clinical Features

V. Biochemical Diagnosis of Cushing’s Syndrome

A. Cardinal features

B. Urinary free cortisol (UFC)

C. Low-dose dexamethasone testing

D. Circadian rhythm assessment

E. Cyclical Cushing’s syndrome

VI. ACTH-Dependent vs. ACTH-Independent Cushing’s Syndrome

VII. Differential Diagnosis of ACTH-Dependent Cushing’s Syndrome

A. Overview

B. Basal testing

C. Dynamic noninvasive testing

D. Invasive testing

VIII. Other Causes of Cushing’s Syndrome

IX. Imaging

A. Pituitary

B. Adrenal

C. Ectopic secretion

X. Differentiation from Pseudo-Cushing’s States

XI. Conclusions

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				M. Korbonits, I. Bujalska, M. Shimojo, J. Nobes, S. Jordan, A. B. Grossman, and P. M. Stewart Expression of 11{beta}-Hydroxysteroid Dehydrogenase Isoenzymes in the Human Pituitary: Induction of the Type 2 Enzyme in Corticotropinomas and Other Pituitary Tumors J. Clin. Endocrinol. Metab., June 1, 2001; 86(6): 2728 - 2733. [Abstract] [Full Text] [PDF]

				A. Lacroix, N. N'Diaye, J. Tremblay, and P. Hamet Ectopic and Abnormal Hormone Receptors in Adrenal Cushing's Syndrome Endocr. Rev., February 1, 2001; 22(1): 75 - 110. [Abstract] [Full Text]

				J. Newell-Price, P. King, and A. J. L. Clark The CpG Island Promoter of the Human Proopiomelanocortin Gene Is Methylated in Nonexpressing Normal Tissue and Tumors and Represses Expression Mol. Endocrinol., February 1, 2001; 15(2): 338 - 348. [Abstract] [Full Text]

				A. White, D. W. Ray, A. Talbot, P. Abraham, A. J. Thody, and J. S. Bevan Cushing's Syndrome Due to Phaeochromocytoma Secreting the Precursors of Adrenocorticotropin J. Clin. Endocrinol. Metab., December 1, 2000; 85(12): 4771 - 4775. [Abstract] [Full Text]

				M. Moro, P. Putignano, M. Losa, C. Invitti, C. Maraschini, and F. Cavagnini The Desmopressin Test in the Differential Diagnosis between Cushing's Disease and Pseudo-Cushing States J. Clin. Endocrinol. Metab., October 1, 2000; 85(10): 3569 - 3574. [Abstract] [Full Text]

				M.-C. Lebrethon, A. B. Grossman, F. Afshar, P. N. Plowman, G. M. Besser, and M. O. Savage Linear Growth and Final Height after Treatment for Cushing's Disease in Childhood J. Clin. Endocrinol. Metab., September 1, 2000; 85(9): 3262 - 3265. [Abstract] [Full Text]

				P. Putignano, G. A. Kaltsas, M. Korbonits, P. J. Jenkins, J. P. Monson, G. M. Besser, and A. B. Grossman Alterations in Serum Protein Levels in Patients with Cushing's Syndrome before and after Successful Treatment J. Clin. Endocrinol. Metab., September 1, 2000; 85(9): 3309 - 3312. [Abstract] [Full Text]

				G. A. Kaltsas, J. D. C. Newell-Price, P. J. Trainer, G. M. Besser, and A. B. Grossman Complications of Inferior Petrosel Sinus Sampling J. Clin. Endocrinol. Metab., April 1, 2000; 85(4): 1741 - 1741. [Full Text]

				P. U. Freda and S. L. Wardlaw Diagnosis and Treatment of Pituitary Tumors J. Clin. Endocrinol. Metab., November 1, 1999; 84(11): 3859 - 3866. [Full Text]

				V. Lefournier, B. Gatta, M. Martinie, A. Vasdev, A. Tabarin, P. Bessou, J. Berge, I. Bachelot, and O. Chabre One Transient Neurological Complication (Sixth Nerve Palsy) in 166 Consecutive Inferior Petrosal Sinus Samplings for the Etiological Diagnosis of Cushing's Syndrome J. Clin. Endocrinol. Metab., September 1, 1999; 84(9): 3401 - 3402. [Full Text]

				W. W. de Herder and S. W.J. Lamberts Tumor Localization--The Ectopic ACTH Syndrome J. Clin. Endocrinol. Metab., April 1, 1999; 84(4): 1184 - 1185. [Full Text]