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

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 manifesta...

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Bibliographic Details
Published in:The journal of clinical endocrinology and metabolism 1997-04, Vol.82 (4), p.1274-1278
Main Authors: Sarlis, Nicholas J, Chrousos, George P, Doppman, John L, Carney, J. Aidan, Stratakis, Constantine A
Format: Article
Language:English
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Summary: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).
ISSN:0021-972X
1945-7197
DOI:10.1210/jcem.82.4.3857