Lim Ling Choo, Tan Leonard H C, Rajasoorya C
Department of Medicine, Alexandra Hospital, Singapore.
Ann Acad Med Singap. 2006 Dec;35(12):892-6.
We present a rare variety of adrenocorticotrophic hormone (ACTH)-independent Cushing's syndrome known as primary pigmented nodular adrenocortical disease (PPNAD).
The patient initially underwent unilateral adrenalectomy for what was thought to be a left adrenal adenoma.
Partial resolution of symptoms and demonstrable persistent hypercortisolism after surgery prompted further evaluation with findings leading to the diagnosis of Carney complex. A review of the adrenal histology was consistent with PPNAD.
This entity of PPNAD, which has rarely been reported in Asians, forms part of the Carney complex. The diagnosis may not be simple and straightforward, as illustrated in this patient.
我们报告一种罕见的促肾上腺皮质激素（ACTH）非依赖性库欣综合征，称为原发性色素沉着性结节性肾上腺皮质病（PPNAD）。
患者最初因被认为是左肾上腺腺瘤而接受了单侧肾上腺切除术。
术后症状部分缓解，但仍存在明显的持续性皮质醇增多症，促使进一步评估，最终诊断为卡尼综合征。肾上腺组织学检查结果与PPNAD一致。
PPNAD这一实体在亚洲人中鲜有报道，是卡尼综合征的一部分。如本病例所示，该疾病的诊断可能并非简单直接。
