Hyperinsulinism in infants and children
Hyperinsulinism in infants and children
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DOI：
10.1016/s0031-3955(05)70481-8
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发表时间：
1997-04-01
影响因子：
2.6
通讯作者：
Stanley, CA
中科院分区：
文献类型：
--
作者：
Stanley, CA
高胰岛素血症是婴儿早期低血糖的最常见原因。先天性高胰岛素血症，以前称为胰岛母细胞增多症，通常是由β细胞调节的遗传缺陷引起的，包括磺酰脲类药物受体的严重隐性疾病，轻度显性形式的高胰岛素血症，以及高胰岛素血症加高氨血症的综合征。短暂性新生儿高胰岛素血症可能与围产期窒息或早产儿出生体重和母体糖尿病有关。为了防止低血糖导致的永久性脑损伤，高胰岛素血症婴儿的治疗必须及时和积极。可能需要使用二氮嗪或奥曲肽（一种长效生长抑素类似物）进行药物治疗，并进行95%胰腺次全切除术。
Hyperinsulinism is the most common cause of hypoglycemia in early infancy. Congenital hyperinsulinism, formerly termed nesidioblastosis, is usually caused by genetic defects in beta-cell regulation, including a severe recessive disorder of the sulfonylurea receptor, a milder dominant form of hyperinsulinism, and a syn drome of hyperinsulinism plus hyperammonemia. Transient neonatal hyperinsulinism may be associated with perinatal asphyxia or small-for-dates birthweight, and maternal diabetes. To prevent permanent brain damage from hypoglycemia, the treatment of infants with hyperinsulinism must be prompt and aggressive. A combination of medical therapy with diazoxide or octreotide, a long-acting somatostatin analog, and surgical 95% subtotal pancreatectomy may be required.