Moossa A R, Baker L, Lavelle-Jones M
West J Med. 1987 May;146(5):585-8.
Persistent, severe hypoglycemia due to inappropriate or excessive insulin secretion is an infrequent but devastating metabolic emergency in infancy and childhood. The primary therapeutic goal is to normalize blood glucose levels and to prevent the catastrophic sequelae of permanent brain damage and mental retardation due to refractory hypoglycemia. Overall, the use of diazoxide, the mainstay of medical treatment, has proved disappointing and has led to the reevaluation of aggressive, early surgical intervention. Currently, subtotal (80% to 90%) pancreatectomy is the most widely used operative procedure in the treatment of hyperinsulinism. It is, however, still associated with a relatively high failure rate, particularly in neonates or during early infancy when either nesidioblastosis or islet cell hyperplasia-both diffuse proliferative beta-cell disorders-is the most common cause of inappropriate insulin secretion. In these cases, "total" or "near-total" pancreatectomy may be necessary to control the hypoglycemic crisis.
由于胰岛素分泌不当或过量导致的持续性严重低血糖，在婴幼儿期是一种罕见但极具破坏性的代谢急症。主要治疗目标是使血糖水平正常化，并预防因难治性低血糖导致的永久性脑损伤和智力发育迟缓等灾难性后果。总体而言，作为主要药物治疗手段的二氮嗪的使用效果令人失望，这促使人们重新评估积极的早期手术干预。目前，胰腺次全切除术（80%至90%）是治疗高胰岛素血症最广泛使用的手术方法。然而，它仍然具有相对较高的失败率，特别是在新生儿期或婴儿早期，此时胰岛细胞增殖症或胰岛细胞增生（均为弥漫性增殖性β细胞疾病）是胰岛素分泌不当最常见的原因。在这些情况下，可能需要进行“全”或“近全”胰腺切除术来控制低血糖危机。
