Idiopathic spontaneous hypoglycemia in children. Report of seven cases and review of the literature.
Idiopathic spontaneous hypoglycemia in children. Report of seven cases and review of the literature.
复制标题
儿童特发性自发性低血糖。
作者：
J. Haworth;F. J. Coodin
本文报道7例儿童特发性自发性低血糖症。回顾了文献中51例这种情况的病例报告。60%的病例在6个月以下出现症状，其中一半以上出现永久性神经系统后遗症。在超过6个月大时首次出现症状的23例中，只有3例后来出现永久性脑损伤。在58例病例中，90%在2岁以下首次出现症状。虽然在ACTH治疗期间经常看到血糖升高到正常水平，但一些患者对这种治疗或肾上腺皮质激素治疗反应不佳。通过饮食治疗的价值不大。在58例患者中，25例行部分胰腺切除术；15人痊愈，10人未痊愈。作者认为，对于1 ~ 2年内药物治疗无效的病例，应行部分胰腺切除术。对29例胰腺进行了组织学检查，其中9例有朗格汉斯胰岛肥大或增生的证据。除两个系列报道外，未发现家族性发病。在58例病例中，7例死亡；在3例中，死亡是低血糖的直接结果。并讨论了病因。虽然研究的病例很少，但先前有两例患者血清中循环胰岛素样活性高。此处记录一位患者的正常值。根据血糖对这种氨基酸的反应，病例可分为亮氨酸敏感型和亮氨酸不敏感型。另一个原因可能是无法通过肾上腺素的释放来提高血糖。作者认为McQuarrie所描述的“婴儿特发性低血糖综合征”不是一个单一的临床实体。
Seven cases of idiopathic spontaneous hypoglycemia in children are reported. A review is made of 51 case reports of this condition found in the literature. The onset of symptoms occurred under the age of 6 months in 60% of the cases and of these, over half showed permanent neurologic sequelae. Of 23 cases first developing symptoms when over 6 months of age only 3 later showed permanent brain damage. Of the total 58 cases, 90% first developed symptoms when under 2 years of age. Although an elevation of the blood sugar to normal levels is often seen during treatment with ACTH, some patients do not respond favorably to this treatment, nor to treatment with adrenocorticosteroids. Treatment by dietary means is of little value. Of the 58 cases, 25 underwent partial pancreatectomy; 15 were cured and 10 were not. It is the authors9 belief that partial pancreatectomy should be performed in those cases which do not respond to medical treatment within 1 to 2 years. The pancreas was examined histologically in 29 cases and in 9 of these there was evidence of hypertrophy or hyperplasia of the islets of Langerhans. No familial incidence was noted with the exception of two reported series. Of the total 58 cases, 7 died; in 3, the death was a direct result of the hypoglycemia. The etiology is discussed. Although few cases have been studied, high levels of circulating insulin-like activity in serum have been previously recorded in two patients. A normal value in one patient is recorded here. Cases may be classified as leucine-sensitive and leucine-insensitive according to the blood sugar response to this amino acid. The possibility exists that another cause may be an inability to raise the blood glucose by the release of adrenalin. The "syndrome of idiopathic hypoglycemia of infants" as described by McQuarrie is believed by the authors not to be a single clinical entity.