PDF(Pubmed)
Abstract：
Idiopathic ketotic hypoglycemia (IKH) is defined as bouts of hypoglycemia with increased blood or urine ketones in certain children after prolonged fasting or during illness. IKH is divided into physiological IKH, which is most frequently observed in normal children with intercurrent acute illness, and pathological IKH, which occurs in children who lack counter-regulatory hormones, have a metabolic disease, or have Silver-Russell syndrome. The typical patient is a young child between the ages of 10 months and 6 years. Episodes nearly always occur in the morning after overnight fasting. Symptoms include those of neuroglycopenia, ketosis, or both. IKH may be diagnosed after ruling out various metabolic and hormonal conditions associated with ketotic hypoglycemia. Sufficient amounts of carbohydrates and protein, avoidance of prolonged fasting, and increased frequency of food ingestion are the main modes of treating IKH. It is crucial to understand the pathogenesis of IKH and to distinguish physiological IKH from pathological IKH. In this mini-review, we present a brief summary of IKH in terms of its definition, types, clinical presentation, diagnosis, and therapeutic approach in children.
摘要：
特发性酮症性低血糖（IKH）定义为某些儿童在长时间禁食或患病后发生的血液或尿酮增加的低血糖发作。IKH分为生理性IKH，最常见于并发急性疾病的正常儿童和病理性IKH，常见于缺乏反调节激素的儿童或患有某些代谢疾病或Silver-Russell综合征的儿童。典型的患者是年龄在10个月至6岁之间的幼儿。事件几乎总是在过夜后的早晨发生。症状包括神经性糖减少症，酮症,或者两者兼而有之。在排除与KH相关的各种代谢和激素状况之后,可以诊断IKH。提供足量的碳水化合物和蛋白质，避免长时间禁食，增加喂食频率是IKH治疗的主线。了解IKH的发病机制以及区分生理性IKH和病理性IKH至关重要。在这个小型审查中，我们简要回顾了IKH的定义，类型,临床表现,,儿童IKH的诊断和治疗方法。
