肾上腺皮质增生类疾病的诊断及外科治疗(附180例报告)

目的 探讨肾上腺皮质增生类疾病的临床诊断及外科治疗方法. 方法 肾上腺皮质增生患者180例.男74例,女106例.年龄6～76岁,平均40岁.其中皮质醇增多症152例,醛固酮增多症28例.180例均行肾上腺手术治疗,术后病理检查均符合肾上腺皮质增生诊断.回顾分析患者临床表现特点、实验室检查结果、肾上腺CT影像特点及疗效. 结果 180例中库欣病(CD)107例,促肾上腺皮质激素(ACTH)非依赖性肾上腺大结节增生(AIMAH)22例,异位ACTH所致肾上腺皮质增生(EAAH)19例,原发性色素结节样肾上腺皮质增生(PPNAH)4例,特发性醛固酮增多症(IHA)28例.实验室检查前4组平均24 h尿游离皮质醇(24 h UFC)分别为287.6(95.2～535.7)、307.9(24.8～808.2)、852.5(102.5～3127.0)、564.3(243.8～1124.6)μg,皮质醇分泌节律消失比例分别为99%(102/103)、92%(11/12)、100%(17/17)、100%(4/4);AIMAH、EAAH、PPNAH组大、小剂量地塞米松抑制试验(DDST)均不被抑制,CD组大剂量DDST可被抑制.28例IHA患者中血钾降低(<3.5 mmol/L)17例,尿钾升高(>30 mmol/24 h)15例,肾素及醛固酮测定符合诊断.180例患者肾上腺CT检查均提示病变侧肾上腺体积增大.102例皮质醇增多症患者行单侧肾上腺切除,术后1周内CD、AIMAH、EAAH、PPNAH组平均24 h UFC分别为157.4(56.2～233.5)、117.9(22.5～418.5)、756.7(116.5～1137.0)、164.3(124.6～422.6)μg.21例IHA患者行单侧肾上腺病灶切除,术后血压恢复正常8例(38%),较术前下降13例(62%).57例行双侧肾上腺手术治疗(双侧全切39例,一侧全切一侧次全切16例,双侧次全切除2例),其中皮质醇增多症患者术后1周复查24 h UFC为12.8～98.5μg,平均77.6μg;IHA患者血压正常.106例(59%)随访4～158个月,平均32个月,库欣病症状及高血压症状均有改善. 结论 需要手术治疗的肾上腺皮质增生类疾病首选单侧肾上腺全切治疗,后期根据临床观察可选择对侧肾上腺次全切除或全切除,可获得较好治疗效果.

Diagnosis and surgical management of adrenocortical hyperplasia disease (report of 180 cases)

Objective To review the diagnosis and surgical therapeutic methods of adrenocorti-cal hyperplasia disease. Methods One hundred and eighty adrenocortical hyperplasia patients (74 males and 106 females with a mean age of 40 years) were retrospectively analyzed. The patients were divided into hypereortisolism (n=152) and aldosteronism (n=28) according to secretion. Data of clinical characteristic, endocrine and image examination were collected. All patients were treated by operation. Results Of these patients, 107 had Cushing disease (CD), 28 had adrenocorticotropin independent macronodular adrenal hyperplasia (AIMAH), 19 had ectopic adrenocorticotropin adrenal hyperplasia (EAAH), 4 had primary pigmented nodular adrenocortical hyperplasia (PPNAH), 28 had DOI: 10.3760/cma.j.issn. 1000-6702.2009.05.002idiopathic hyperaldosteronism (IHA). 24 h urinay free cortisol (24hUFC) excretion of CD, AIMAH, EAAH and PPNAH were 95.2-535.7 μg (mean, 287.6 μg), 24.8-808.2 μg (mean, 307.9 μg), 102.5-3127.0 μg (mean, 852.5 μg), 243.8-1124.6 μg (mean. 564.3 μg). The proportion loss of the serum cortisol circadian rhythm were 99% (102/103), 92% (11/12), 100% (17/17), 100% (4/ 4), respectively. Low- and high-dose dexamethasone suppression tests (DDST) failed to suppress cor-tisol secretion in AIMAH, PPNAH and EAAH groups, but HDDST was suppressed in CD group. Of the 28 IHA cases, 17 had hypokalemia and 15 had high urine kalium (>30 mmol/24 h). The results of plasma renin activity and serum aldosterone accorded with the diagnosis. Unilateral adrenalectomy were operated in 102 hypercortisolism cases, and 24hUFC of CD, AIMAH, EAAH and PPNAH were 56.2-233.5 μg (mean, 157.4 μg), 22.5-418.5 μg (mean, 117.9 μg), 116.5-1137.0 μg (mean, 756.7 μg), 124.6-422.6 μg (mean, 164.3 μg) 1 week after operation. The blood pressure was nor-mal in 8 paitents and droped in 13 patients for IHA after unilateral adrenalectomy. 24hUFC were nor-mal in 55 patients after bilateral adrenalectomy for hypercortisolism. One hundred and six patients were followed up for 4-158 months, the Cushing syndrome ameliorated and blood pressure dropped. Conclusions Unilateral adrenalectomy is the first choice for adrenocortical hyperplasia disease which needs operation. The operation mode of the contralateral adrenal gland is based on the hyperplasia types and clinical observation.