A Case of Cushing's syndrome due to Primary Pimary Pigmented Nodular Adrenal Dysplasia ( PPNAD ): A Case of Carney's Complex.
- Author:
Soon Jib YOO
;
Bong Yun CHA
;
Kwang Woo LEE
;
Ho Young SON
;
Sung Koo KANG
;
Youn Sik KIM
;
Jong Min LEE
;
Jong Man WON
- Publication Type:Case Report
- Keywords:
Cushing's syndrome;
Primary Pigmented Nodular Adrenal Dysplasia (PPNAD);
Carney's complex
- MeSH:
Adrenalectomy;
Adrenocorticotropic Hormone;
Cushing Syndrome*;
Dexamethasone;
Humans;
Hydrocortisone;
Infant;
Lentigo;
Lip;
Myxoma;
Neural Plate;
Pituitary Neoplasms;
Testicular Neoplasms;
Wills;
Young Adult
- From:Journal of Korean Society of Endocrinology
1997;12(1):90-98
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Primary Pigmented Nodular Adrenal Dysplasia (PPNAD) is a rare cause of Cushing's syndrome in infants and young adults. The familial occurrence, it may be variably associated with a complex of other pathologic characteristics that manifests extraadrenal disorders (includes cardiac myxomas, lentigines, mammary myxoid lesions, testicular tumors, pituitary adenomas, and neuroectodermal tumors) was considered indicative of Carneys complex. This was based on the failure of cortisol suppression by high-dose dexamethasone, either normal or suppressed basal adrenocorticotropic hormone (ACTH) levels, and normal radiographic studies of the sellar turcica, and adrenals glands is almost normal or slightlg eulaged.. Bilateral adrenalectomy has thus the only effective means of cure. The disease may be a component of a rare, but potentially dangerous complex of abnormalities that follow an autosomal-dominant mode of inheritance. Recently we experienced a case of Carney's complex composed by Cushings syndrome due to PPNAD with familial purple colored lentigines on their lips and report it with reviews of the literatures.
