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[1例以低血糖发作为表现的多发性内分泌腺瘤1型(MEN1)患者]

[A Patient with Multiple Endocrine Neoplasia Type1(MEN1)Presenting with Hypoglycemic Attacks].

作者信息

Bando Kazuhiko, Ebisutani Daizo

机构信息

Department of Neurosurgery, Anankyoei Hospital.

出版信息

No Shinkei Geka. 2015 May;43(5):451-6. doi: 10.11477/mf.1436203045.

DOI:10.11477/mf.1436203045
PMID:25926542
Abstract

Here, we report the case of a woman with multiple endocrine neoplasia type 1(MEN1) who experienced hypoglycemic attacks. At the age of 59, she underwent parathyroid tumor resection for hyperparathyroidism. At the age of 65, she presented with dizziness at our hospital. Magnetic resonance imaging (MRI) revealed a left cerebellopontine (CP) angle tumor and a pituitary tumor. The CP angle tumor (acoustic neurionoma) was removed;the pituitary adenoma (prolactinoma) was managed by using bromocriptine. At the age of 77, she lost consciousness and was transferred to a local hospital. Her blood sugar level was 24 mg/dL. Due to the frequent recurrence of hypoglycemic attacks, she was readmitted to our hospital. MRI revealed the almost complete removal of the acoustic tumor and that her pituitary gland was atrophied. Despite her baseline pituitary hormone levels being normal, we suspected panhypopituitarism and administered cortisol (15 mg/day). As her hypoglycemia failed to improve, we performed a 75-g oral glucose tolerance test, and its result was not indicative of diabetes mellitus. Her pretest immunoreactive insulin (IRI) level was 6.8μU/mL;ΔIRI/ΔBS was 0.62, indicative of insulin hypersecretion. Contrast-enhanced abdominal computed tomography revealed multiple pancreatic tumors (insulinomas), and she underwent resection of the uncal tumor and pancreas body and tail. Her postoperative IRI level was normalized and she experienced no further hypoglycemic attacks. Based on her hyperparathyroidism, pancreatic tumor, and pituitary adenoma, we diagnosed her with MEN1.

摘要

在此,我们报告一例患有1型多发性内分泌腺瘤病(MEN1)并经历低血糖发作的女性病例。59岁时,她因甲状旁腺功能亢进接受了甲状旁腺肿瘤切除术。65岁时,她在我院出现头晕症状。磁共振成像(MRI)显示左侧小脑桥脑角(CP)肿瘤和垂体肿瘤。切除了CP角肿瘤(听神经瘤);垂体腺瘤(催乳素瘤)采用溴隐亭治疗。77岁时,她失去意识并被转至当地医院。她的血糖水平为24mg/dL。由于低血糖发作频繁复发,她再次入住我院。MRI显示听神经瘤几乎完全切除,且她的垂体萎缩。尽管其基础垂体激素水平正常,但我们怀疑她患有全垂体功能减退症,并给予皮质醇(15mg/天)治疗。由于她的低血糖症状未改善,我们进行了75g口服葡萄糖耐量试验,结果不提示糖尿病。她试验前的免疫反应性胰岛素(IRI)水平为6.8μU/mL;ΔIRI/ΔBS为0.62,提示胰岛素分泌过多。腹部增强计算机断层扫描显示多个胰腺肿瘤(胰岛素瘤),她接受了枕骨肿瘤及胰体尾切除术。术后她的IRI水平恢复正常,且未再发生低血糖发作。根据她的甲状旁腺功能亢进、胰腺肿瘤和垂体腺瘤,我们诊断她患有MEN1。

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