局灶性先天性高胰岛素血症（CHI）的手术治疗——“德国国际高胰岛素血症”在30例儿童中的经验
Surgery in Focal Congenital Hyperinsulinism (CHI) - The "Hyperinsulinism Germany International" Experience in 30 Children.
作者信息
Barthlen Winfried, Varol Emine, Empting Susann, Wieland Ilse, Zenker Martin, Mohnike Wolfgang, Vogelgesang Silke, Mohnike Klaus
机构信息
University Medicine, Pediatric Surgery, Greifswald, Germany.
Otto-von-Guericke-University, Pediatrics, Magdeburg, Germany.
出版信息
Pediatr Endocrinol Rev. 2016 Dec;14(2):129-137. doi: 10.17458/PER.2016.BVE.Surgeryinfocal.
OBJECTIVES
Results of surgery for focal CHI in 30 children PATIENTS AND METHODS: All showed an ABCC8 or KCNJ11 mutation. After PET/CT in 29 children and PET/MRT in 1 case, frozen-section guided resection was performed, in left-sided cases by laparoscopy. Mean age at surgery was 11.7 months (2-49).
RESULTS
In 28/30 children, the PET/CT or MRT correlated with histopathology. In two cases, a focal lesion was undectable; one of these was cured, one not. In total, 24 children showed lesions with sizes of 5-12 mm. All were cured instantly. In four children with huge lesions in the pancreatic head, pathological cells remained at the resection margins. One child was cured instantly, two children after a 2nd surgery, and one child was not cured, even after three surgeries. The overall cure rate was 93%.
CONCLUSIONS
Imaging, surgical findings, histopathology and clinical outcome in surgery for focal CHI match in most, but not all cases.
目的
30例儿童局灶性先天性高胰岛素血症（CHI）的手术结果
患者与方法
所有患者均显示ABCC8或KCNJ11基因突变。29例儿童进行了PET/CT检查，1例进行了PET/MRT检查，之后在冰冻切片引导下进行切除，左侧病例采用腹腔镜手术。手术时的平均年龄为11.7个月（2 - 49个月）。
结果
28/30例儿童中，PET/CT或MRT与组织病理学结果相关。2例中未检测到局灶性病变；其中1例治愈，1例未治愈。总共24例儿童的病变大小为5 - 12毫米。所有这些患儿均立即治愈。4例胰头有巨大病变的儿童，切除边缘残留病理细胞。1例儿童立即治愈，2例儿童在二次手术后治愈，1例儿童即使经过三次手术仍未治愈。总体治愈率为93%。
结论
局灶性CHI手术中的影像学、手术发现、组织病理学和临床结果在大多数但并非所有病例中相符。
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