肾上腺切除术后多发2A型内分泌瘤患者胰岛素依赖性糖尿病的缓解。

JCEM case reports Pub Date : 2025-01-09 eCollection Date: 2025-01-01 DOI:10.1210/jcemcr/luae240
Megumi Sato, Michio Otsuki, Tomomi Mori, Juro Yanagida, Toshiyuki Yamamoto, Tomoko Nakagami
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引用次数: 0

摘要

男性,37岁,过去一年出现多尿和体重下降的症状。初步实验室检查显示血糖升高(468 mg/dL [25.9 mmol/L];正常参考范围[RR], 75-109 mg/dL [4.1-6.0 mmol/L]),高糖化血红蛋白A1c (13.2% [120 mmol/mol];RR为4.6 ~ 6.2% [26 ~ 44 mmol/mol]),尿c肽排泄量低(17.4 μg/day [5.76 nmol/day];RR为18.3 ~ 124.4 μg/day [6.0 ~ 41.1 nmol/day]),伴有酮症,诊断为胰岛素依赖型糖尿病。随后的调查确定了甲状腺髓样癌和双侧嗜铬细胞瘤。结合检测到RET基因变异及患者多发性内分泌瘤2A型(MEN2A)家族史,确认MEN2A的诊断。入院后,开始强化胰岛素治疗,症状得到缓解,血糖水平恢复正常。随后行腹腔镜双侧肾上腺切除术,术后患者糖耐量恢复正常,无需糖尿病治疗,避免低血糖。该病例强调了儿茶酚胺通过α2作用于胰腺β细胞诱导的胰岛素分泌抑制的潜力,在MEN2A患者经历胰岛素依赖型糖尿病的肾上腺切除术后得到缓解和迅速改善。
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Remission of Insulin-Dependent Diabetes Mellitus in Multiple Endocrine Neoplasia Type 2A After Adrenalectomy.

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 RET 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.

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