Sato Megumi, Otsuki Michio, Mori Tomomi, Yanagida Juro, Yamamoto Toshiyuki, Nakagami Tomoko
Division of Diabetology and Metabolism, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Shinjuku-ku, Tokyo 162-8666, Japan.
Division of Endocrinology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Shinjuku-ku, Tokyo 162-8666, Japan.
JCEM Case Rep. 2025 Jan 9;3(1):luae240. doi: 10.1210/jcemcr/luae240. eCollection 2025 Jan.
A 37-year-old man presented with symptoms of polyuria and weight loss over the past year. Initial laboratory examination showed elevated blood glucose level (468 mg/dL [25.9 mmol/L]; normal reference range [RR], 75-109 mg/dL [4.1-6.0 mmol/L]), high glycated hemoglobin A1c (13.2% [120 mmol/mol]; RR, 4.6-6.2% [26-44 mmol/mol]), low urinary C-peptide excretion (17.4 μg/day [5.76 nmol/day]; RR, 18.3-124.4 μg/day [6.0-41.1 nmol/day]), and ketosis, leading to a diagnosis of insulin-dependent diabetes mellitus. Subsequent investigations identified medullary thyroid carcinoma and bilateral pheochromocytomas. Given the detected gene variant and the patient's family history of multiple endocrine neoplasia type 2A (MEN2A), the diagnosis of MEN2A was confirmed. Upon hospital admission, intensive insulin therapy was commenced, which resolved the symptoms and normalized blood glucose levels. Subsequently, laparoscopic bilateral adrenalectomy was performed, after which the patient's glucose tolerance normalized, eliminating the need for diabetes treatment and avoiding hypoglycemia. This case highlights the potential for catecholamine-induced suppression of insulin secretion via α2 action on pancreatic β-cells to be remission and rapidly improved by adrenalectomy in individuals with MEN2A experiencing insulin-dependent diabetes mellitus.
一名37岁男性在过去一年中出现多尿和体重减轻症状。初始实验室检查显示血糖水平升高（468 mg/dL [25.9 mmol/L]；正常参考范围[RR]，75 - 109 mg/dL [4.1 - 6.0 mmol/L]），糖化血红蛋白A1c升高（13.2% [120 mmol/mol]；RR，4.6 - 6.2% [26 - 44 mmol/mol]），尿C肽排泄降低（17.4 μg/天 [5.76 nmol/天]；RR，18.3 - 124.4 μg/天 [6.0 - 41.1 nmol/天]），以及酮症，从而诊断为胰岛素依赖型糖尿病。随后的检查发现了甲状腺髓样癌和双侧嗜铬细胞瘤。鉴于检测到的基因变异以及患者的2A型多发性内分泌腺瘤病（MEN2A）家族史，确诊为MEN2A。入院后开始强化胰岛素治疗，症状得以缓解，血糖水平恢复正常。随后进行了腹腔镜双侧肾上腺切除术，术后患者的糖耐量恢复正常，不再需要糖尿病治疗并避免了低血糖。该病例突出了在患有胰岛素依赖型糖尿病的MEN2A个体中，儿茶酚胺通过对胰腺β细胞的α2作用抑制胰岛素分泌的情况有可能通过肾上腺切除术得到缓解并迅速改善。
