Choi K M, Seu J H, Kim Y H, Lee E J, Kim S J, Baik S H, Choi D S
Department of Internal Medicine, Korea University College of Medicine, Seoul.
Korean J Intern Med. 1995 Jan;10(1):68-72. doi: 10.3904/kjim.1995.10.1.68.
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.
原发性色素沉着性结节性肾上腺皮质疾病（PPNAD）是婴儿、儿童和年轻成人库欣综合征的罕见病因。其特征是增生的肾上腺皮质细胞形成多个色素沉着结节，导致非促肾上腺皮质激素依赖性皮质醇分泌过多。在生化方面，PPNAD的特征是血浆和尿皮质醇水平升高，且不受高剂量地塞米松（8mg/d，持续2天）抑制。在病理上，肾上腺含有多个深棕色或黑色结节，其间的皮质组织萎缩。尽管这种诊断罕见，但认识到这一点很重要，因为双侧肾上腺切除术是首选治疗方法。我们遇到一例因原发性色素沉着性结节性肾上腺皮质疾病导致的库欣综合征病例，并结合文献复习进行报告。
