Sarlis N J, Chrousos G P, Doppman J L, Carney J A, Stratakis C A
Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA.
J Clin Endocrinol Metab. 1997 Apr;82(4):1274-8. doi: 10.1210/jcem.82.4.3857.
A 45-yr-old man with primary pigmented nodular adrenocortical disease (PPNAD) is described. This patient underwent unilateral adrenalectomy for ACTH-independent Cushing's syndrome (CS) in 1969. Although his daily urinary free cortisol (UFC) excretion rate normalized, and the major clinical manifestations of CS subsided, loss of a circadian cortisol rhythm persisted after surgery. Twenty-seven years later, the patient presented again with short stature, severe osteopenia, skeletal deformities, thinning of the skin, and myopathy.
本文描述了一名45岁患有原发性色素沉着性结节性肾上腺皮质疾病（PPNAD）的男性患者。该患者于1969年因促肾上腺皮质激素（ACTH）非依赖性库欣综合征（CS）接受了单侧肾上腺切除术。尽管其每日尿游离皮质醇（UFC）排泄率恢复正常，CS的主要临床表现也消退，但术后昼夜皮质醇节律仍持续缺失。27年后，该患者再次出现身材矮小、严重骨质减少、骨骼畸形、皮肤变薄和肌病。
