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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).

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