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胰岛素瘤:仅见于成人吗?——病例报告与文献综述

Insulinoma: only in adults?-case reports and literature review.

作者信息

Gozzi Graf Tiziana, Brändle Michael, Clerici Thomas, l'Allemand Dagmar

机构信息

Clinic of Adolescent Medicine, Division of Paediatric Endocrinology and Diabetology, Ostschweizer Kinderspital, Claudiusstrasse 6, 9006, St. Gallen, Switzerland,

出版信息

Eur J Pediatr. 2014 May;173(5):567-74. doi: 10.1007/s00431-013-2005-8. Epub 2013 Apr 21.

Abstract

Insulinomas first presenting as refractory seizure disorders are well documented in adulthood but rarely found in children. Only a few cases of childhood insulinoma have been reported so far. We report on two adolescents with hyperinsulinaemic hypoglycaemia, initially misdiagnosed as epilepsy and migraine accompagnée, and compare those to other cases published. Localization of insulinoma was challenging and, in one patient, angiography with selective arterial calcium stimulation and hepatic venous sampling in addition to CT and MRI was necessary. In these patients, long-term recovery was achieved by laparoscopic distal pancreatic resection in one and by conventional enucleation in the pancreatic head in the second patient. In contrast to adults, macrosomy and a decrease in school performance were the main symptoms and, during fasting, impaired cognitive function occurred after a relatively short period and at a higher glucose threshold or lower insulin/glucose ratio, respectively. Neuroglycopenic signs may be attributed to behaviour abnormalities or seizure disorders but in children and adolescents may already be caused by insulinoma. In these cases, timely diagnosis as well as tumour resection ensure long-term cure.

摘要

胰岛素瘤首发表现为难治性癫痫发作在成人中已有充分记录,但在儿童中很少见。迄今为止,仅报道了少数儿童胰岛素瘤病例。我们报告了两名患有高胰岛素血症性低血糖症的青少年,最初被误诊为癫痫和偏头痛伴发症,并将其与已发表的其他病例进行比较。胰岛素瘤的定位具有挑战性,在一名患者中,除了CT和MRI外,还需要进行选择性动脉钙刺激和肝静脉采样的血管造影。在这些患者中,一名患者通过腹腔镜远端胰腺切除术实现了长期康复,另一名患者通过传统的胰头摘除术实现了康复。与成人不同,巨大儿和学业成绩下降是主要症状,在禁食期间,认知功能障碍分别在相对较短的时间内、在较高的血糖阈值或较低的胰岛素/葡萄糖比值时出现。神经低血糖体征可能归因于行为异常或癫痫发作,但在儿童和青少年中可能已经由胰岛素瘤引起。在这些情况下,及时诊断以及肿瘤切除可确保长期治愈。

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