Palladino Andrew A, Stanley Charles A
Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania 19104, USA.
Semin Pediatr Surg. 2011 Feb;20(1):32-7. doi: 10.1053/j.sempedsurg.2010.10.008.
Hyperinsulinism (HI) is the most common cause of transient and permanent forms of hypoglycemia in infancy. Establishing the correct diagnosis and initiating appropriate therapy without delay is of utmost importance. Once the diagnosis is made and if medical therapy with diazoxide fails, one should assume that the infant has a K(ATP) channel defect and may require surgery. In this case, the infant should be referred to a center that specializes in HI with 18-fluoro L-3,4-dihydroxyphenylalanine positron emission tomography scan. This report describes a center specializing in HI with a team of experts consisting of endocrinologists, nurse practitioners, geneticists, radiologists, pathologists, and a surgeon. It describes the center's paradigm for managing severe HI on the basis of more than 250 cases of HI in the past 10 years, including the diagnosis of HI, medical options, genetics of HI, imaging in HI, the surgical approach to HI, and outcomes.
高胰岛素血症（HI）是婴儿期短暂性和永久性低血糖最常见的病因。及时做出正确诊断并立即开始适当治疗至关重要。一旦做出诊断且用二氮嗪进行的药物治疗失败，就应假定婴儿存在K（ATP）通道缺陷，可能需要手术治疗。在这种情况下，应将婴儿转诊至一家通过18氟-L-3,4-二羟基苯丙氨酸正电子发射断层扫描专门诊治HI的中心。本报告介绍了一家专门诊治HI的中心，该中心有一个由内分泌学家、执业护士、遗传学家、放射科医生、病理学家和一名外科医生组成的专家团队。报告描述了该中心基于过去10年250多例HI病例管理严重HI的模式，包括HI的诊断、医疗选择、HI的遗传学、HI的影像学检查、HI的手术方法及治疗结果。
