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1995 ; 10
(1
): 68-72
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Cushing s syndrome due to primary pigmented nodular adrenocortical disease--a
case report reviews of the literature
#MMPMID7626560
Choi KM
; Seu JH
; Kim YH
; Lee EJ
; Kim SJ
; Baik SH
; Choi DS
Korean J Intern Med
1995[Jan]; 10
(1
): 68-72
PMID7626560
show ga
Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of
Cushing's syndrome in infants, children and young adults. It is characterized by
non-adrenocorticotropic hormone-dependent hypersecretion of cortisol by multiple,
pigmented nodules of hyperplastic adrenocortical cells. Biochemically, PPNAD is
characterized by elevated levels of plasma and urinary cortisol that are not
suppressed by high doses of dexamethasone (8mg/d for 2 days). Pathologically, the
adrenal glands contain multiple dark brown or black nodules and the intervening
cortical tissue is atrophic. Recognition of this diagnosis, although rare, is
important, as bilateral adrenalectomy is the treatment of choice. We experienced
a case of Cushing's syndrome due to primary pigmented nodular adrenocortical
disease and report it with reviews of the literature.