This Article Full Text Full Text (PDF) Submit a related Letter to the Editor Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Sarlis, N. J. Articles by Stratakis, C. A. Search for Related Content PubMed PubMed Citation Articles by Sarlis, N. J. Articles by Stratakis, C. A.

Primary Pigmented Nodular Adrenocortical Disease: Reevaluation of a Patient with Carney Complex 27 Years after Unilateral Adrenalectomy

Developmental Endocrinology Branch, National Institute of Child Health and Human Development (G.P.C., C.A.S.); Laboratory of Molecular and Cellular Biology, National Institute of Diabetes and Digestive and Kidney Diseases (N.J.S.); and the Department of Radiology, Warren G. Magnuson Clinical Center (J.L.D.), National Institutes of Health, Bethesda, Maryland 20892; and the Department of Laboratory Medicine and Pathology, Mayo Clinic (J.A.C.), Rochester, Minnesota 55905

Address all correspondence and requests for reprints to: Constantine A. Stratakis, M.D., D.Sc., Unit on Genetics and Endocrinology, Section on Pediatric Endocrinology/Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N262, 10 Center Drive, MSC 1862, Bethesda, Maryland 20892-1862. E-mail: stratakc{at}cc1.nichd.nih.gov

A 45-yr-old man with primary pigmented nodular adrenocortical disease (PPNAD) is described. This patient underwent unilateral adrenalectomy for ACTH-independent Cushing’s syndrome (CS) in 1969. Although his daily urinary free cortisol (UFC) excretion rate normalized, and the major clinical manifestations of CS subsided, loss of a circadian cortisol rhythm persisted after surgery. Twenty-seven years later, the patient presented again with short stature, severe osteopenia, skeletal deformities, thinning of the skin, and myopathy(Cline Endocrinol Metab 82: 1274–1278, 1997).

This article has been cited by other articles:

				J R Meinardi, B H R Wolffenbuttel, and R P F Dullaart Cyclic Cushing's syndrome: a clinical challenge Eur. J. Endocrinol., September 1, 2007; 157(3): 245 - 254. [Abstract] [Full Text] [PDF]

				M. Boscaro, L. Barzon, and N. Sonino The Diagnosis of Cushing's Syndrome: Atypical Presentations and Laboratory Shortcomings Arch Intern Med, November 13, 2000; 160(20): 3045 - 3053. [Abstract] [Full Text] [PDF]

				C. A. Stratakis, N. Sarlis, L. S. Kirschner, J. A. Carney, J. L. Doppman, L. K. Nieman, G. P. Chrousos, and D. A. Papanicolaou Paradoxical Response to Dexamethasone in the Diagnosis of Primary Pigmented Nodular Adrenocortical Disease Ann Intern Med, October 19, 1999; 131(8): 585 - 591. [Abstract] [Full Text] [PDF]

				R. V. Garcia-Mayor, L. F. P. Mendez, C. Paramo, and R. L. Cano Spontaneous Complete Remission of Primary Pigmented Adrenocortical Disease J. Clin. Endocrinol. Metab., October 1, 1997; 82(10): 3517a - 3518. [Full Text]