Kobayashi Takaaki, Miwa Takashi, Kan Kenshi, Takeda Misato, Sakai Hiroyuki, Kanazawa Akira, Tanaka Akihiko, Namiki Kazunori, Nagao Toshitaka, Odawara Masato
Division of Diabetes, Metabolism and Endocrinology, The Third Department of Internal Medicine, Tokyo Medical University, Japan.
Intern Med. 2012;51(13):1709-13. doi: 10.2169/internalmedicine.51.7041. Epub 2012 Jul 1.
Adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is a rare disease which causes Cushing's syndrome. Bilateral adrenalectomy has been recommended as the treatment of choice for AIMAH. However, bilaterally adrenalectomized patients require lifelong steroid replacement therapy. Therefore, an increasing number of patients have undergone unilateral adrenalectomy for AIMAH. We report a case of AIMAH due to refractory diabetes in whom unilateral adrenalectomy initially yielded good diabetes control, but in whom poor glycemic control developed after 5 years, requiring eventual additional contralateral adrenalectomy. In elderly patients with AIMAH, one-stage bilateral adrenalectomy may be the treatment of choice.
促肾上腺皮质激素(ACTH)非依赖性大结节性肾上腺增生(AIMAH)是一种导致库欣综合征的罕见疾病。双侧肾上腺切除术已被推荐为AIMAH的首选治疗方法。然而,双侧肾上腺切除术后的患者需要终身接受类固醇替代治疗。因此,越来越多的患者因AIMAH接受了单侧肾上腺切除术。我们报告一例因难治性糖尿病导致的AIMAH病例,该患者最初接受单侧肾上腺切除术后糖尿病控制良好,但5年后血糖控制变差,最终需要进行对侧肾上腺切除术。对于老年AIMAH患者,一期双侧肾上腺切除术可能是首选治疗方法。