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Abstract：
Cushing\'s disease is a rare condition that occurs due to an adrenocorticotrophin-producing corticotrophinoma arising from the pituitary gland. The consequent hypercortisolaemia results in multisystem morbidity and mortality. This study aims to report incidence, clinicopathological characteristics, remission outcomes and mortality in a regional pituitary neurosurgical cohort of patients diagnosed with Cushing\'s disease in Northern Ireland (NI) from 2000 to 2019. Clinical, biochemical and radiological data from a cohort of patients operated for Cushing\'s disease were retrospectively collected and analysed. Fifty-three patients were identified, resulting in an estimated annual incidence of Cushing\'s disease of 1.39-1.57 per million population per year. Females accounted for 72% (38/53) of the cohort. The majority (74%, 39/53) of corticotrophinomas were microadenomas and in 44% (17/39) of these no tumour was identified on preoperative magnetic resonance imaging. Histopathological characterisation was similarly difficult, with no tumour being identified in the histopathological specimen in 40% (21/53) of cases. Immediate postoperative remission rates were 53% and 66% when considering serum morning cortisol cut-offs of ≤ 50 nmol/L (1.8 µg/dL) and ≤ 138 nmol/L (5 µg/dL), respectively, in the week following pituitary surgery. Approximately 70% (37/53) of patients achieved longer-term remission with a single pituitary surgery. Three patients had recurrent disease. Patients with Cushing\'s disease had a significantly higher mortality rate compared to the NI general population (standardised mortality ratio 8.10, 95% CI 3.3-16.7, P < 0.001). Annual incidence of Cushing\'s disease in NI is consistent with other Northern European cohorts. Functioning corticotrophinomas are a clinically, radiologically and histopathologically elusive disease with increased mortality compared to the general population.
摘要：
库欣病是一种罕见的疾病，由于垂体产生的促肾上腺皮质激素产生的促肾上腺皮质激素瘤。随之而来的高皮质醇血症导致多系统发病率和死亡率。这项研究旨在报告发病率，临床病理特征，北爱尔兰2000-2019年诊断为库欣病的区域垂体神经外科队列患者的缓解结局和死亡率。临床,我们回顾性收集并分析了一组库欣氏病患者的生化和放射学数据.确认了53名患者,导致库欣病的年发病率估计为每百万人口每年1.39-1.57。女性占队列的72％（38/53）。大多数（74%，39/53）的促肾上腺皮质激素瘤是微腺瘤，在术前磁共振成像中没有发现其中44％（17/39）的肿瘤。组织病理学表征同样困难，在40％（21/53）的病例中，组织病理学标本中未发现肿瘤。当考虑垂体手术后一周的血清早晨皮质醇临界值≤50nmol/L（1.8µg/dL）和≤138nmol/L（5µg/dL）时，术后即刻缓解率分别为53％和66％。大约70％（37/53）的患者通过一次垂体手术获得了长期缓解。3例患者疾病复发。与北爱尔兰普通人群相比，库欣病患者的死亡率明显更高（标准化死亡率8.10，95％置信区间3.3-16.7，p<0.001）。北爱尔兰库欣病的年发病率与其他北欧人群一致。功能性促肾上腺皮质激素瘤是一种临床上，与普通人群相比，放射学和组织病理学上难以捉摸的疾病死亡率增加。
