Stacpoole P W, Interlandi J W, Nicholson W E, Rabin D
Medicine (Baltimore). 1982 Jan;61(1):13-24.
Isolated adrenocorticotropin (ACTH) deficiency is a rare cause of secondary adrenocortical insufficiency. This review summarizes the clinical and laboratory features of 39 previously reported cases plus 4 new patients. The clinical manifestations of isolated ACTH deficiency are variable, nonspecific and similar to those seen in adrenocortical insufficiency of any cause. The diagnosis of isolated ACTH deficiency due to intrinsic pituitary disease is made unequivocally when all the following criteria are met: 1) low basal urinary 17-hydroxycorticosteroid (17-OHCS) levels with or without low basal plasma cortisol, 2) low or normal basal plasma ACTH, 3) stimulation of cortisol, 17-OHCS or both during prolonged ACTH administration, 4) lack of 17-OHCS elevation in response to metyrapone and 5) normal secretory indices of other pituitary hormones. Isolated ACTH deficiency secondary to suprapituitary (e.g., hypothalamic) dysfunction is also based upon the above criteria, but, in addition, is associated with stimulation of cortisol and ACTH secretion following vasopressin administration.
孤立性促肾上腺皮质激素（ACTH）缺乏是继发性肾上腺皮质功能不全的罕见原因。本综述总结了39例既往报道病例以及4例新患者的临床和实验室特征。孤立性ACTH缺乏的临床表现多样、无特异性，与任何原因引起的肾上腺皮质功能不全所见相似。当满足以下所有标准时，可明确诊断为因垂体自身疾病导致的孤立性ACTH缺乏：1）基础尿17-羟皮质类固醇（17-OHCS）水平低，无论基础血浆皮质醇水平是否低；2）基础血浆ACTH水平低或正常；3）在长时间给予ACTH期间，皮质醇、17-OHCS或两者均有升高；4）对甲吡酮无反应，17-OHCS不升高；5）其他垂体激素的分泌指标正常。继发于垂体上（如下丘脑）功能障碍的孤立性ACTH缺乏也基于上述标准，但此外，还与给予血管加压素后皮质醇和ACTH分泌增加有关。
