先天性高胰岛素血症：非手术治疗同样有效

Conservative management of congenital hyperinsulinism shows good neurological outcomes
非手术治疗先天性高胰岛素血症对神经系统作用良好

Congenital hyperinsulinism (CH) is a genetic disorder characterized by inadequate suppression of insulin secretion in the presence of recurrent hypoglycemia. Children with CH are at increased risk of developmental disorders such as psychomotor retardation, learning disabilities and other neurological problems. Although surgical management is currently recommended in many centers, the long-term adverse effects of this treatment can be considerable. Mazor-Aronovitch and colleagues studied the neurodevelopmental outcomes in a homogenous group of patients with CH who were treated with a conservative, nonsurgical approach.
先天性高胰岛素血症（CH）是一种遗传性疾病，其特点是胰岛素分泌抑制不足，经常发生低血糖症。儿童CH患者发育障碍如精神发育迟滞、学习障碍及其他神经系统疾病风险增加。虽然目前大多数中心推荐手术治疗，但手术的远期副作用很值得重视。Mazor-Aronovitch及其同事研究了某种非手术的保守治疗方法对同族CH患者神经发育的影响。

Of the 21 patients conservatively treated between 1975 and 1997, 12 had diffuse disease (homozygous mutant alleles) and 9 had presumed focal disease (paternal mutant allele only). The management of these patients included medication to maintain normoglycemia (octreotide, diazoxide and glucagons) and feedings with a gastronomy tube. Clinical data were obtained from medical records and interviews with the parents of the patients, with information from each patient's nearest-age sibling used as control data.
1975年至1997年期间接受保守治疗的21例患者中，12例为弥漫性病变（等位基因突变纯合子），9例为局灶性病变（仅父系等位基因突变）。这些患者的治疗包括服用药物（奥曲肽，二氮嗪及胰高血糖素）以维持正常血糖以及胃管喂养。临床资料来自病历及与患儿家长的面谈，对照组资料来自与每例患者年龄最近的兄弟姊妹。

The occurrence of motor and speech problems in infancy were similar between patients and controls; only gross motor problems (clumsiness) were more common among patients than controls (38% versus 10%, P = 0.036), although these generally improved by school age. At school age, the proportion of patients with learning difficulties or attention, hyperactivity, social skills or behavioral problems was not statistically different from controls. No cases of overt diabetes were reported.
患者组与对照组运动及言语问题发生率相近，粗大运动障碍（笨拙 ）更为常见（ 38 ％比10 ％ ，P= 0.036 ），但这些在学龄时普遍改善。学龄期，发生学习困难或注意力障碍、多动、社交能力或行为问题的患者比例与对照组无统计学差异。无显性糖尿病病例。

The authors conclude that conservative treatment of children with CH results in neurological outcomes that are at least as good as those associated with surgical management.
作者总结，保守治疗儿童CH对于神经系统的作用目前至少与外科治疗同样有效。