Section of Oncologic Imaging, Radiology Department, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Blvd, Philadelphia, PA 19104, USA.
Section of Oncologic Imaging, Radiology Department, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; The Roberts Center for Pediatric Research, Room 8255, 2715 South Street, Philadelphia, PA 19146, USA.
PET Clin. 2020 Jul;15(3):349-359. doi: 10.1016/j.cpet.2020.03.004.
Congenital hyperinsulinism (HI) is the most common cause of persistent hypoglycemia in neonates and infants. Several genetic mutations have been identified and are associated with 2 distinct histopathologic forms of disease: diffuse and focal. Targeted clinical evaluation to distinguish medically treatable disease from disease requiring surgical management can prevent life-threatening complications. Detection and localization of a surgically curable focal lesion using PET imaging with 18-F-L 3,4-dihydroxyphenylalanine ([18F]-FDOPA) has become standard of care. This article provides guidelines for the selection of patients who can benefit from [18F]-FDOPA-PET/computed tomography and protocols and tips used to diagnose a focal lesion of HI.
先天性高胰岛素血症(HI)是新生儿和婴儿持续性低血糖的最常见原因。已经发现了几种基因突变,与 2 种不同的组织病理学疾病形式相关:弥漫性和局灶性。有针对性的临床评估可以区分需要药物治疗的疾病和需要手术治疗的疾病,从而预防危及生命的并发症。使用 18-F-L-3,4-二羟基苯丙氨酸 ([18F]-FDOPA) 的 PET 成像检测和定位可手术治疗的局灶性病变已成为标准治疗方法。本文提供了选择可从 [18F]-FDOPA-PET/计算机断层扫描中获益的患者的指南,以及用于诊断 HI 局灶性病变的方案和技巧。