CAJ | 학술논문

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±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.
목적 명학촉신상선피질격소(ACTH)비의뢰성쌍측신상선대결절양증생(AIMAH)적림상특점.방법 회고성분석북경협화의원자2001년1월지2011년12월수치적AIMAH공30례,총결AIMAH적림상화생화특점이급치료전귀.결과 AIMAH재불동성별적발병비례차이무통계학의의,평균기병년령(44±9)세,중위병정시5년,최상견적림상표현시고혈압(100.0％).혈피질순주야절률균소실,24h뇨유리피질순(UFC)정상적환자부유4례(13.3％),소유환자소제량지새미송억제시험균불피억제,28례(93.3％)환자대제량지새미송억제시험불피억제.재이상수체표체적흥강시험중,14례환자중유12례구유1충혹1충이상흥강시험정양성혹부분양성반응.25례환자채용수술치료,행단측신상선전절술적14례환자중,8례완해,이행단측신상선전절+대측차전절술적7례환자중,6례완해.결론 영상학표현위쌍측신상선대결절양증생적환자응명학유무AIMAH,병의거환자림상증상화피질순수평채취불동치료방식,완해림상증상,개선예후.
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 ± 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.