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

Nicholas J. Sarlis | Zendy; George P. Chrousos | Zendy; John L. Doppman | Zendy; J. Aidan Carney | Zendy; Constantine A. Stratakis | Zendy

Open Access

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

Author(s) -

Nicholas J. Sarlis,

George P. Chrousos,

John L. Doppman,

J. Aidan Carney,

Constantine A. Stratakis

Publication year - 1997

Publication title -

the journal of clinical endocrinology and metabolism

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.206

H-Index - 353

eISSN - 1945-7197

pISSN - 0021-972X

DOI - 10.1210/jcem.82.4.3857

Subject(s) - medicine , adrenalectomy , endocrinology , short stature , adrenocortical carcinoma , carney complex , circadian rhythm , adrenocortical adenoma , urinary system , cushing syndrome , adenoma , biology , biochemistry , gene

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.

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