Clinical analysis of 8 cases of primary pigmented nodular adrenocortical disease
- VernacularTitle:8例原发性色素性结节状肾上腺皮质病的临床分析
- Author:
Wei LI
;
Kai FENG
;
Ou WANG
;
Quanzong MAO
;
Mingming HU
;
Xin YUE
;
Zhaolin LU
- Publication Type:Journal Article
- Keywords:
Cushing's syndrome;
primary pigmented nodular adrenocortical disease;
Carney complex;
diagnosis;
surgery
- From:
Basic & Clinical Medicine
2010;30(5):538-541
- CountryChina
- Language:Chinese
-
Abstract:
Objective To summarize the clinical characteristics of primary pigmented nodular adrenocortical disease (PPNAD).Methods Eight patients with PPNAD from 2001 to 2009 in PUMC Hospital were reviewed,their clinical data were collected.Results PPNAD often occurred in adolescents.62.5% of patients with PPNAD were complicated with Carney complex(CNC).In addition to general features of Cushing's syndrome,amenorrhea and growth retardation in stature were very frequent in clinical manifestations of PPNAD.Plasma ACTH was undetectable,circadian rhythm of plasma cortisol was disappeared,glucocorticoid excretion was increased paradoxically during the dexamethasone suppression test in 50% patients with PPNAD.Adrenal imaging from 75% patients revealed normal-sized adrenal glands or suspectable micronodular changes.Adrenal pathologic analysis revealed numerous brown cortical nodules containing lipofuscin pigmentation.Unilateral adrenalectomy may relieve symptoms of Cushing's syndrome,but plasma ACTH and circadian rhythm of plasma cortisol were difficult to recovere.Hypercorticoidism might re-occure after unilateral adrenalectomy.Conclusion PPNAD should be bewared in ACTH independent Cushing's syndrome patients without apparent adrenal mass,and CNC should be screened and followed up.
