通过对比增强计算机断层扫描成像可清晰检测到的局灶性先天性高胰岛素血症。
Focal form of congenital hyperinsulinism clearly detectable by contrast-enhanced computed tomography imaging.
作者信息
Hashimoto Yukiko, Sakakibara Azumi, Kawakita Rie, Hosokawa Yuki, Fujimaru Rika, Nakamura Tetsuro, Fukushima Hiroko, Igarashi Aiko, Masue Michiya, Nishibori Hironori, Tamagawa Nobuyoshi, Murakami Akiko, Hatake Kazue, Yorifuji Tohru
机构信息
Department of Pediatric Endocrinology and Metabolism, Children's Medical Center, Osaka City General Hospital, 2-13-22 Miyakojima-hondori, Miyakojima, Osaka 534-0021 Japan.
Department of Pediatric Surgery, Osaka City General Hospital, Osaka, Japan.
出版信息
Int J Pediatr Endocrinol. 2015;2015(1):20. doi: 10.1186/s13633-015-0016-0. Epub 2015 Sep 15.
The focal form of congenital hyperinsulinism (CHI) is characterized by a cluster of abnormal insulin-oversecreting β cells within a restricted area of the pancreas. Although identification of the focal lesion is very important in the management of CHI, it has been reported that imaging studies, including computed tomography (CT), magnetic resonance imaging (MRI) scans, or angiography, are not helpful in identifying the focal lesion. Currently, fluorine-18-L-dihydroxyphenylalanine positron emission tomography ((18)F-DOPA PET) is believed to be the only imaging modality that can identify the focal lesions. In this report, however, we present a case of a 7-month-old girl with the focal form of CHI, caused by a loss-of-function mutation in the ABCC8 gene, whose lesion was clearly visible as a hyperenhancing nodule on contrast-enhanced CT and dynamic MRI imaging.
局灶性先天性高胰岛素血症（CHI）的特征是胰腺受限区域内有一群异常分泌胰岛素的β细胞。尽管局灶性病变的识别在CHI的管理中非常重要，但据报道，包括计算机断层扫描（CT）、磁共振成像（MRI）扫描或血管造影在内的影像学检查对识别局灶性病变并无帮助。目前，氟-18-L-二羟基苯丙氨酸正电子发射断层扫描（（18）F-DOPA PET）被认为是唯一能够识别局灶性病变的成像方式。然而，在本报告中，我们介绍了一例7个月大的患有局灶性CHI的女孩，其病因是ABCC8基因功能丧失突变，在增强CT和动态MRI成像上，其病变表现为明显强化的结节，清晰可见。
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