Bränström Robert, Berglund Erik, Curman Pontus, Forsberg Lars, Höög Anders, Grimelius Lars, Berggren Per-Olof, Mattsson Per, Hellman Per, Juntti-Berggren Lisa
The Rolf Luft Research Center for Diabetes and Endocrinology, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
J Med Case Rep. 2010 Sep 23;4:315. doi: 10.1186/1752-1947-4-315.
Non-insulinoma pancreatogenous hypoglycemic syndrome is a rare disorder among adults, and, to our knowledge, only about 40 cases have been reported in the literature.
The patient is a previously healthy 35-year-old Caucasian man. His symptoms began four years ago when he suddenly felt weakness in his legs and started sweating for unknown reasons. The symptoms worsened, and laboratory tests revealed hypoglycemia and hyperinsulinemia at the time of the symptoms. All diagnostics attempts using magnetic resonance imaging, computed tomography, and endoscopic ultrasound did not reveal any abnormalities. At this stage, surgical intervention was planned, and a distal 80% pancreatectomy was performed. The histopathologic and immunohistochemical investigations of the pancreas showed an increased number of islets of different sizes, more or less evenly distributed in the gland, but no insulinoma. Patch-clamp recordings from isolated pancreatic β-cells showed that, even at a low glucose concentration (3 mmol/L), the β-cell membrane was depolarized, and action potentials were seen. Surprisingly, in patch-clamp experiments, the addition of diazoxide had a marked effect on K-ATP channel activity and membrane potential, but no effect on insulin levels in vivo before surgery.
This case report adds new information on the pathogenesis of non-insulinoma pancreatogenous hypoglycemic syndrome, as we performed an electrophysiologic characterization of isolated islet cells. We show, for the first time, that β-cells isolated from a non-insulinoma pancreatogenous hypoglycemic syndrome patient are constantly depolarized, even at low glucose levels, but display normal K-ATP channel physiology.
非胰岛素瘤胰腺源性低血糖综合征在成年人中是一种罕见疾病，据我们所知，文献中仅报道了约40例。
该患者是一名此前健康的35岁白种男性。他的症状始于四年前，当时他突然感到腿部无力并开始不明原因地出汗。症状逐渐加重，症状发作时实验室检查显示低血糖和高胰岛素血症。使用磁共振成像、计算机断层扫描和内镜超声进行的所有诊断尝试均未发现任何异常。在此阶段，计划进行手术干预，并实施了远端80%胰腺切除术。胰腺的组织病理学和免疫组织化学检查显示不同大小的胰岛数量增加，在腺体中分布或多或少均匀，但未发现胰岛素瘤。对分离的胰腺β细胞进行的膜片钳记录显示，即使在低葡萄糖浓度（3 mmol/L）下，β细胞膜也发生去极化，并可见动作电位。令人惊讶的是，在膜片钳实验中，二氮嗪的添加对K-ATP通道活性和膜电位有显著影响，但对术前体内胰岛素水平无影响。
本病例报告为非胰岛素瘤胰腺源性低血糖综合征的发病机制增添了新信息，因为我们对分离的胰岛细胞进行了电生理特征分析。我们首次表明，从非胰岛素瘤胰腺源性低血糖综合征患者分离的β细胞即使在低葡萄糖水平下也持续去极化，但显示正常的K-ATP通道生理学。
