摘要
目的 明确促肾上腺皮质激素（ACTH）非依赖性双侧肾上腺大结节样增生（AIMAH）的临床特点.方法 回顾性分析北京协和医院自2001年1月至2011年12月收治的AIMAH共30例,总结AIMAH的临床和生化特点以及治疗转归.结果 AIMAH在不同性别的发病比例差异无统计学意义,平均起病年龄（44＆#177;9）岁,中位病程是5年,最常见的临床表现是高血压（100.0％）.血皮质醇昼夜节律均消失,24h尿游离皮质醇（UFC）正常的患者仅有4例（13.3％）,所有患者小剂量地塞米松抑制试验均不被抑制,28例（93.3％）患者大剂量地塞米松抑制试验不被抑制.在异常受体表达的兴奋试验中,14例患者中有12例具有1种或1种以上兴奋试验呈阳性或部分阳性反应.25例患者采用手术治疗,行单侧肾上腺全切术的14例患者中,8例缓解,而行单侧肾上腺全切＋对侧次全切术的7例患者中,6例缓解.结论 影像学表现为双侧肾上腺大结节样增生的患者应明确有无AIMAH,并依据患者临床症状和皮质醇水平采取不同治疗方式,缓解临床症状,改善预后.
Objective To explore the clinical characteristics of adrenocorticotropic hormone （ACTH） independent macronodular adrenal hyperplasia （AIMAH）.Methods A total of 30 AIMAH patients from January 2001 to December 2011 at our hospital were reviewed retrospectively and their clinical data collected.Results AIMAH was equally distributed between genders.Their mean age was 44 ＆#177; 9 years and median course of disease 5 years.Hypertension was the most common clinical manifestation.Circadian rhythm of plasma cortisol disappeared in all patients,and the level of 24 hour urinary free cortisol （24 hUFC） was normal only in 4（13.3%） patients.Both low and high dose dexamethasone suppression tests were not suppressed in 30 （100.0%） and 28 （93.3%） patients respectively.The stimulation tests for detecting aberrant expression of hormone receptors were performed in 14 patients.At least one aberrant cortisol response was identified in 12 patients.Twenty-five patients underwent adrenalectomy.Among 7 patients of bilateral adrenalectomy,6 achieved remission while 8 patients did so among 14 patients of unilateral adrenalectomy.Conclusions AIMAH should be considered in patients with massively enlarged bilateral adrenal glands.Treatment modalities should be decided according to clinical manifestations and cortisol level so as to relieve symptoms and improve prognosis.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2014年第12期924-927,共4页
National Medical Journal of China
关键词
库欣综合征
促肾上腺皮质素
肾上腺大结节样增生
临床特点
治疗
Cushing syndrome
Adrenocorticotropic hormone
Macronodular adrenal hyperplasia
Clinical characteristics
Therapy
