Prete Alessandro, Paragliola Rosa Maria, Bottiglieri Filomena, Rota Carlo Antonio, Pontecorvi Alfredo, Salvatori Roberto, Corsello Salvatore Maria
Unit of Endocrinology, Facoltà di Medicina e Chirurgia, Università Cattolica del Sacro Cuore, Largo Francesco Vito 1, Rome, 00168, Italy.
Department of Medicine, Division of Endocrinology, Metabolism and Diabetes, and Pituitary Center Johns Hopkins University School of Medicine, 773 N Broadway, Baltimore, MD, 21205, USA.
Endocrine. 2017 Mar;55(3):969-980. doi: 10.1007/s12020-016-1007-5. Epub 2016 Jul 9.
Successful treatment of Cushing syndrome causes transient or permanent adrenal insufficiency deriving from endogenous hypercortisolism-induced hypothalamus-pituitary-adrenal-axis suppression. We analyzed pre-treatment factors potentially affecting the duration of adrenal insufficiency. We conducted a retrospective analysis on patients successfully treated for Cushing disease (15 patients) who underwent transsphenoidal surgery, and nonmalignant primary adrenal Cushing syndrome (31 patients) who underwent unilateral adrenalectomy, divided into patients with overt primary adrenal Cushing syndrome (14 patients) and subclinical primary adrenal Cushing syndrome (17 patients). Epidemiological data, medical history, and hormonal parameters depending on the etiology of hypercortisolism were collected and compared to the duration of adrenal insufficiency. The median duration of follow-up after surgery for Cushing disease and primary adrenal Cushing syndrome was 70 and 48 months, respectively. In the Cushing disease group, the median duration of adrenal insufficiency after transsphenoidal surgery was 15 months: younger age at diagnosis and longer duration of signs and symptoms of hypercortisolism before diagnosis and surgery were associated with longer duration of adrenal insufficiency. The median duration of adrenal insufficiency was 6 months for subclinical primary adrenal Cushing syndrome and 18.5 months for overt primary adrenal Cushing syndrome. The biochemical severity of hypercortisolism, the grade of hypothalamus-pituitary-adrenal-axis suppression, and treatment with ketoconazole before surgery accounted for longer duration of adrenal insufficiency. In patients with Cushing disease, younger age and delayed diagnosis and treatment predict longer need for glucocorticoid replacement therapy after successful transsphenoidal surgery. In patients with primary adrenal Cushing syndrome, the severity of hypercortisolism plays a primary role in influencing the duration of adrenal insufficiency after unilateral adrenalectomy.
库欣综合征的成功治疗会导致因内源性高皮质醇血症引起的下丘脑 - 垂体 - 肾上腺轴抑制而出现短暂或永久性肾上腺功能不全。我们分析了可能影响肾上腺功能不全持续时间的治疗前因素。我们对因库欣病接受经蝶窦手术成功治疗的患者(15例)以及因非恶性原发性肾上腺库欣综合征接受单侧肾上腺切除术的患者(31例,分为显性原发性肾上腺库欣综合征患者14例和亚临床原发性肾上腺库欣综合征患者17例)进行了回顾性分析。收集了取决于高皮质醇血症病因的流行病学数据、病史和激素参数,并与肾上腺功能不全的持续时间进行比较。库欣病和原发性肾上腺库欣综合征手术后的中位随访时间分别为70个月和48个月。在库欣病组中,经蝶窦手术后肾上腺功能不全的中位持续时间为15个月:诊断时年龄较小以及诊断和手术前高皮质醇血症体征和症状持续时间较长与肾上腺功能不全持续时间较长相关。亚临床原发性肾上腺库欣综合征患者肾上腺功能不全的中位持续时间为6个月,显性原发性肾上腺库欣综合征患者为18.5个月。高皮质醇血症的生化严重程度、下丘脑 - 垂体 - 肾上腺轴抑制程度以及手术前酮康唑治疗与肾上腺功能不全持续时间较长有关。在库欣病患者中,年龄较小以及诊断和治疗延迟预示着经蝶窦手术成功后糖皮质激素替代治疗的需求时间更长。在原发性肾上腺库欣综合征患者中,高皮质醇血症的严重程度在影响单侧肾上腺切除术后肾上腺功能不全的持续时间方面起主要作用。