Gough M H
Br J Surg. 1984 Jan;71(1):75-8. doi: 10.1002/bjs.1800710125.
Hyperinsulinaemic hypoglycaemia in infancy may be transient or persistent. Persistent hypoglycaemia is caused by nesidioblastosis of the pancreas, a diffuse pancreatic endocrine abnormality involving all the endocrine tissue, or by an islet cell adenoma. The cause of nesidioblastosis is not known but unless controlled its hypoglycaemic effects lead to convulsions, mental retardation or death. Patients with transient hyperinsulinaemic hypoglycaemia, and some with persistent hypoglycaemia, respond to medical therapy but those with an adenoma and many of those with nesidioblastosis will require partial or total pancreatectomy. The surgical treatment of six infants and a child is discussed. Five infants had nesidioblastosis; one infant and one nine-year-old girl had an islet cell adenoma. It is suggested that in the very young when there is no evidence of an insulinoma, resection at the initial operation should be greater--up to 90 per cent--than the previously recommended 75 per cent.
婴儿期高胰岛素血症性低血糖可能是短暂性的或持续性的。持续性低血糖由胰腺成神经细胞瘤(一种累及所有内分泌组织的弥漫性胰腺内分泌异常)或胰岛细胞瘤引起。成神经细胞瘤的病因尚不清楚,但除非得到控制,其低血糖效应会导致惊厥、智力发育迟缓或死亡。短暂性高胰岛素血症性低血糖患者以及部分持续性低血糖患者对药物治疗有反应,但患有腺瘤的患者和许多患有成神经细胞瘤的患者将需要进行部分或全胰腺切除术。本文讨论了六名婴儿和一名儿童的手术治疗情况。五名婴儿患有成神经细胞瘤;一名婴儿和一名九岁女孩患有胰岛细胞瘤。建议在没有胰岛素瘤证据的非常年幼的患者中,初次手术的切除范围应比之前推荐的75%更大,可达90%。