Noninsulinoma pancreatogenous hypoglycemia syndrome: A rare case of adult-onset nesidioblastosis
Noninsulinoma pancreatogenous hypoglycemia syndrome: A rare case of adult-onset nesidioblastosis
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DOI：
10.2169/internalmedicine.44.843
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发表时间：
2005-08-01
影响因子：
1.2
通讯作者：
Hirata, Y
中科院分区：
文献类型：
--
作者：
Tsujino, M;Sugiyama, T;Hirata, Y
成人高胰岛素血症性低血糖最常见的原因是胰岛素瘤。胰岛母细胞瘤病是一种罕见的，但公认的疾病，持续高胰岛素血症低血糖症在婴儿期，但成人发病胰岛母细胞瘤病与高胰岛素血症低血糖症，称为非胰岛素瘤胰源性低血糖综合征（NIPHS），已报告。在这里，我们描述了一个非常罕见的情况下，NIPHS的老年男子。一个78岁的男子被收住到我们的医院，由于低血糖昏迷。在过去的3个月里，他注意到下午三点多汗。他的低空腹血糖水平（27 mg/dl）和高免疫反应性胰岛素水平（11.1 μ U/ml）与胰岛素瘤的可能存在一致。局部研究，包括腹部计算机断层扫描和腹腔动脉造影均为阴性，但选择性动脉钙灌注（SACI）试验表明，在胰腺的体部和尾部存在胰岛素瘤。通过触诊和术中超声检查的手术探查未能发现胰腺中的任何肿块，并进行了60%的胰腺远端切除术。术后，他的低血糖发作完全消失。切除胰腺的组织学检查显示弥漫性胰岛细胞增生与胰岛母细胞增生症的病理诊断一致。因此，我们的病例是一个非常罕见的NIPHS，或成人发病nesidioblastosis，其中SACI测试被证明是一个有用的诊断工具，胰腺病变的定位。
The most common cause of hyperinsulinemic hypoglycemia in adults is insulinoma. Nesidioblastosis is a rare, but well-recognized disorder of persistent hyperinsulinemic hypoglycemia in infancy, but adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, termed noninsulinoma pancreatogenous hypoglycemic syndrome (NIPHS), has been reported. Here, we describe an extremely rare case of NIPHS in an elderly man. A 78-year-old man was admitted to our hospital due to hypoglycemic coma. During the previous 3 months, he noticed excessive sweating at midafternoon. His low fasting plasma glucose level (27 mg/dl) and high immunoreactive insulin level (11.1 mu U/ml) were consistent with the possible presence of insulinoma. Localizing studies including computed tomography of the abdomen and celiac arteriography were negative, but selective arterial calcium infusion (SACI) test suggested the presence of insulinoma in the body and tail of the pancreas. Surgical exploration by palpation and intraoperative ultrasonography failed to detect any mass in the pancreas, and 60% distal pancreatectomy was performed. Postoperatively, his hypoglycemic episodes completely disappeared. Histological examination of the resected pancreas revealed diffuse islet cell hyperplasia consistent with a pathological diagnosis of nesidioblastosis. Thus, our case is a very rare case of NIPHS, or adult-onset nesidioblastosis, in which SACI test was proven to be a useful diagnostic tool for localization of the pancreatic lesion.