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 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.
Sarlis
N J
NJ
Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA.
Chrousos
G P
GP
Doppman
J L
JL
Carney
J A
JA
Stratakis
C A
CA
D000303:Adrenal Cortex Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D000311:Adrenal Glands / Q000000981:diagnostic imaging; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D002940:Circadian Rhythm; D003480:Cushing Syndrome / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010859:Pigmentation Disorders / Q000473:pathology*; D012871:Skin Diseases / Q000473:pathology*; D013577:Syndrome; D013997:Time Factors; D014057:Tomography, X-Ray Computed
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone