Incidence and late prognosis of cushing's syndrome: a population-based study.
Incidence and late prognosis of cushing's syndrome: a population-based study.
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库欣综合征的发病率和晚期预后：一项基于人群的研究。
DOI：
10.1210/jcem.86.1.7093
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发表时间：
2001
期刊：
影响因子：
--
通讯作者：
J. Weeke
中科院分区：
文献类型：
--
作者：
J. Lindholm;S. Juul;J. Jørgensen;J. Astrup;P. Bjerre;U. Feldt;Claus Hagen;J. Jørgensen;M. Kosteljanetz;L. Kristensen;P. Laurberg;K. Schmidt;J. Weeke
主要目的是评估库欣综合征，特别是库欣病的发病率和晚期结局。检索了丹麦11年期间诊断为库欣综合征的所有患者的信息。库欣病的发病率为1.2-1.7/百万年，肾上腺腺瘤为0.6/百万年，肾上腺癌为0.2/百万年。其他类型的库欣综合征是罕见的。在139例非恶性疾病患者中，11.1%的患者在随访期间死亡（中位数，8.1年;范围，3.1-14.0），标准死亡率比（SMR）为3.68 [95%置信区间（CI），2.34-5.33]。SMR部分归因于诊断后第一年内死亡率的增加。8名患者在接受治疗前死亡。恶性肿瘤患者预后极差。对初次手术后超过5年的患者进行了单独研究，包括对其健康质量的问卷调查。在45例经蝶窦神经外科手术治愈的库欣病患者中，只有1例死亡（SMR，0.31; CI，0.01-1.72），而20例初次神经外科手术后持续性皮质醇增多症患者中有6例死亡（SMR，5.06; CI，1.86-11.0）。肾上腺腺瘤患者的SMR为3.95（CI，0.81-11.5）。感知的健康质量显着受损，只有在患者与库欣病，并出现独立的疾病控制或存在垂体功能减退。结论是：1）库欣综合征是罕见的，并与死亡率增加，在没有并发恶性肿瘤的患者也; 2）死亡率过高主要是在第一年的疾病;和3）受损的健康质量在长期生存的库欣病是没有得到充分解释。
The main purpose was to assess the incidence and late outcome of Cushing's syndrome, particularly in Cushing's disease. Information for all patients diagnosed with Cushing's syndrome during an 11-yr period in Denmark was retrieved. The incidence was 1.2-1.7/million.yr (Cushing's disease), 0.6/million.yr (adrenal adenoma) and 0.2/million.yr (adrenal carcinoma). Other types of Cushing's syndrome were rare. In 139 patients with nonmalignant disease, 11.1% had died during follow-up (median, 8.1 yr; range, 3.1-14.0), yielding a standard mortality ratio (SMR) of 3.68 [95% confidence interval (CI), 2.34-5.33]. The SMR was partly attributable to an increased mortality within the first year after diagnosis. Eight patients died before treatment could be undertaken. The prognosis in patients with malignant disease was very poor. Patients in whom more than 5 yr had elapsed since initial surgery were studied separately, including a questionnaire on their perceived quality of health. In 45 patients with Cushing's disease who had been cured through transsphenoidal neurosurgery, only 1 had died (SMR, 0.31; CI, 0.01-1.72) compared with 6 of 20 patients with persistent hypercortisolism after initial neurosurgery (SMR, 5.06; CI, 1.86-11.0). In patients with adrenal adenoma, SMR was 3.95 (CI, 0.81-11.5). The perceived quality of health was significantly impaired only in patients with Cushing's disease and appeared independent of disease control or presence of hypopituitarism. It is concluded that 1) Cushing's syndrome is rare and is associated with increased mortality, in patients with no concurrent malignancy also; 2) the excess mortality was mainly observed during the first year of disease; and 3) the impaired quality of health in long-term survivors of Cushing's disease is not fully explained.
儿童和青少年经蝶手术治疗库欣病后的长期结果。
DOI：
10.1210/jcem.82.10.4290
发表时间：
1997
期刊：
The Journal of clinical endocrinology and metabolism.
影响因子：
--
作者：
Devoe,DJ;Miller,WL;Conte,FA;Kaplan,SL;Grumbach,MM;Rosenthal,SM;Wilson,CB;Gitelman,SE
通讯作者：
Gitelman,SE