Zhang Yushi, Li Hanzhong
Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College Beijing 100730, China.
Int J Clin Exp Med. 2015 Oct 15;8(10):19311-7. eCollection 2015.
To review and discuss the diagnostic and surgical therapeutic methods of adrenocortical hyperplastic disease.
A retrospective analysis was done to 180 adrenocortical hyperplasia patients (74 males, 109 females, aged 6~76 (average 40.1). Studies were done to the relationship between patients' clinical characteristics, biochemical, endocrinological and imaging examination results, the therapeutic effects.
Among all 180 cases, there are 107 Cushing disease (CD), 19 ectopic adrenocorticotropin adrenal hyperplasia (EAAH), 28 adrenocorticotropin independent macronodular adrenal hyperplasia (AIMAH), 4 primary pigmented nodular adrenocortical hyperplasia (PPNAH), and 28 Idiopathic Hyperaldosteronism (IHA). Twenty-four-hour urinary free cortisol (24 h UFC) excretion of CD, EAAH, AIMAH and PPNAH patients were 95.2535.7 µg (average 287.6 µg), 24.8808.2 µg (average 307.9 µg), 102.53127.0 µg (average 852.5 µg), and 243.81124.6 µg (average 564.3 µg). Both low and high-dose dexamethasone suppression tests (DDST) were not suppressed in AIMAH, PPNAH and EAAH groups, but HDDST was suppressed in CD group. CT thin scanning results of 180 patients all showed enlargements in the affected side adrenal gland. Unilateral adrenalectomies were performed in 102 hypercortisolism cases. Local lesion excisions were done to 21 IHA patients. 57 patients had surgeries in both sides of the adrenal glands (39 bilateral total adrenalectomies, 16 total adrenalectomy in one side andsubtotal adrenalectomy in the other, 2 bilateral subtotal adrenalectomies). 106 (59%) patients were followed up for 4~158 (average 32) months.
Unilateral adrenalectomy was the first choice for operable adrenocortical hyperplasia patients. The operation mode for the other adrenal gland should be based on the type of hyperplasia and clinical observation.
回顾并探讨肾上腺皮质增生性疾病的诊断及外科治疗方法。
对180例肾上腺皮质增生患者（男74例，女109例，年龄6～76岁，平均40.1岁）进行回顾性分析。研究患者的临床特征、生化、内分泌及影像学检查结果之间的关系以及治疗效果。
180例患者中，库欣病（CD）107例，异位促肾上腺皮质激素肾上腺增生（EAAH）19例，促肾上腺皮质激素非依赖性大结节性肾上腺增生（AIMAH）28例，原发性色素沉着性结节性肾上腺皮质增生（PPNAH）4例，特发性醛固酮增多症（IHA）28例。CD、EAAH、AIMAH及PPNAH患者的24小时尿游离皮质醇（24 h UFC）排泄量分别为95.2～535.7μg（平均287.6μg）、24.8～808.2μg（平均307.9μg）、102.5～3127.0μg（平均852.5μg）和243.8～1124.6μg（平均564.3μg）。AIMAH、PPNAH及EAAH组的低剂量和高剂量地塞米松抑制试验（DDST）均未被抑制，但CD组的高剂量地塞米松抑制试验（HDDST）被抑制。180例患者的CT薄层扫描结果均显示患侧肾上腺增大。对102例皮质醇增多症患者实施了单侧肾上腺切除术。对21例IHA患者进行了局部病变切除术。57例患者进行了双侧肾上腺手术（39例双侧肾上腺全切术，16例一侧肾上腺全切加另一侧肾上腺次全切术，2例双侧肾上腺次全切术）。106例（59%）患者接受了4～158个月（平均32个月）的随访。
对于可手术的肾上腺皮质增生患者，单侧肾上腺切除术是首选。另一侧肾上腺的手术方式应根据增生类型及临床观察情况而定。
