病理为垂体促肾上腺皮质激素细胞增生的库欣病

Cushing's disease due to corticotrope hyperplasia

目的回顾性分析手术后病理证实为垂体促肾上腺皮质激素(ACTH)细胞增生的22例库欣病患者的临床资料。方法男性3例,女性19例,平均年龄28.7岁,平均病程2年。22例临床均表现为典型的库欣综合征,均有血浆皮质醇和尿24h游离皮质醇(UFC)水平增高,血浆ACTH水平增高5例,小剂量地塞米松抑制试验不能抑制、大剂量地塞米松抑制试验能抑制17例。12例行蝶鞍CT冠状扫描和矢状重建,7例正常,5例有异常发现;15例行蝶鞍MRI,3例正常,12例有异常发现。22例均行经口鼻蝶窦显微外科垂体探查术。结果术后病理均为垂体ACTH细胞增生。随访6个月至15年,治愈13例,缓解3例,进步2例,无效4例,3例复发。结论垂体ACTH细胞增生可引起库欣病,对这些患者,可考虑手术探查,行垂体部分、大部分或次全切除,多数可获得较好的疗效。

Objective To discuss the Cushing's disease due to corticotrope hyperplasia.Methods Among the22patients with corticotrope hyperplasia,3were male and19were female.Mean age was28.7yrs old and mean history was2years.They all had the clinical manifestation of typical Cushing's syndrome,accompanied by hypertension in14patients,mense disorder in10patients,osteoporosis in9patients,headache in6patients,diabetes in4patients,hypokalemia in2patients,vision distrubance in2patients and psychiatric symptom in1patient.They all had high level of plasma cortisol and urine free cortisol(UFC)of24hrs.5had high level of plasma ACTH.The small dose dexamethansone suppression test showed positive and large dose dexamethansone suppression test showed negtive in17patients.Coronal CT showed normal in7patients,abnormal in5patients.MRI showed normal in3patients,abnormal in12patients.All had trans-spheniod hypophysectomy.Results Pathology showed corticotrope hyperplasia in all22patients.Postoperative follow-up ranged from6months to15years.13were cured,3were remission,2were improved,4were inefficent and3recurred.Conclusion Corticotrope hyperplasia could result in Cushing's disease.To these patients,surgical explortation and partial?subtotal hypophysectomy could be considered.Most of them had good effect.[