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皮质醇增多症分为垂体性、肾上腺性和异源性三类。垂体性皮质醇增多症又称库欣病,是因垂体促肾上腺皮质激素（adrenocorticotrophic hormone,ACTH）腺瘤或腺体增生所致。儿童库欣病少见。我们回顾性总结了北京协和医院1990年1月～1999年7月手术时年龄小于14岁的12例儿童库欣病的诊断、经蝶窦手术与疗效,现报道如下。 一、对象与方法 1.一般情况:本组男6例,女6例。年龄8～14岁,平均12.3岁。病程8个月～6年,平均病程2.5年。首发症状向心性肥胖11例,发育迟缓1例。临床症状:11例表现为向心性肥胖、满月脸、水牛背、锁骨上脂肪垫、皮肤红润菲薄。合并高血压6例,发育迟缓5例,紫纹3例,低血钾2例,骨质疏松1例。 2.临床检查:血浆ACTH增高5例,尿游离皮质醇和血浆皮质醇增高12例,血浆皮质醇昼夜节律消失8例,符合尿游离皮质醇地塞米松抑制试验（小剂量不能抑制、大剂量能抑制）9例。影像学检查:蝶鞍X线侧位体层摄片示蝶窦呈甲壳型4例、鞍前型3例、全鞍型5例。2例行CT冠状扫描和矢状重建,1例显示鞍隔膨隆、垂体柄偏移,1例未见异常。10例行蝶鞍MRI检查,7例显示鞍隔饱满和膨隆,其中5例鞍内有T1低信号、增强后病变无强化,3例未见异常。
Cushing’s syndrome is divided into pituitary, adrenal and heterogenous three categories. Pituitary Cushing’s disease also known as Cushing’s disease, is due to pituitary adrenocorticotrophic hormone (ACTH) adenoma or gland hyperplasia. Cushing disease rare in children. We retrospectively reviewed the diagnosis of Cushing’s disease in 12 children younger than 14 years of age from January 1990 to July 1999 in Peking Union Medical College Hospital and surgery and curative effect of transsphenoidal surgery, are reported below. First, the object and method 1. General situation: The group of 6 males and 6 females. Age 8 to 14 years old, an average of 12.3 years old. Course of 8 months to 6 years, the average duration of 2.5 years. The first symptom to heart obesity in 11 cases, 1 case of stunting. Clinical symptoms: 11 cases showed centripetal obesity, full moon face, buffalo back, supraclavicular fat pad, skin rosy meager. 6 cases of hypertension, 5 cases of stunting, purple pattern in 3 cases, hypokalemia in 2 cases, 1 case of osteoporosis. Clinical examination: 5 cases of plasma ACTH increased urinary free cortisol and plasma cortisol increased in 12 cases, plasma cortisol disappeared in 8 cases of circadian rhythm, in line with urine free cortisol dexamethasone inhibition test (small dose can not be suppressed, high dose of energy Inhibition) in 9 cases. Imaging examination: Sella X ray lateral body showed sphenoid sinus shunt in 4 cases, 3 cases of saddle type, 5 cases of full saddle. 2 cases underwent CT coronary angiography and sagittal reconstruction, 1 case showed segmental septal bulges, pituitary stalk shift, 1 case no abnormalities. MRI was performed on 10 cases of sellae, 7 cases showed full sellar valgus and bulging septum, 5 cases had low signal of T1 in the saddle, no enhancement was found in the lesion and 3 cases showed no abnormality.