Idiopathic Pathological Ketotic Hypoglycemia: Finding the Needle in a Haystack.

IF 2.6 3区 医学 Q3 ENDOCRINOLOGY & METABOLISM
Joseph I Wolfsdorf, Terry G J Derks, Danielle Drachmann, Pratik Shah, Paul S Thornton, David A Weinstein
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引用次数: 0

Abstract

Sick children often have a decreased appetite and experience vomiting and diarrhea; however, hypoglycemia (plasma glucose concentration ≤50 mg/dL or 2.8 mmol/L) is rare. Ketotic hypoglycemia (KH) is the most common cause of hypoglycemia presenting to an Emergency Department in a previously healthy child between 6 months and 6 years of age. Ketosis and hypoglycemia are now well understood to be normal physiologic responses of young children to prolonged fasting.There is now substantial evidence that the term KH describes a variety of conditions including both the lower end of the normal distribution of fasting tolerance in young children as well as numerous rare disorders that impair fasting adaptation. Recent advances in molecular genetic testing have led to the discovery of these rare disorders. Idiopathic pathological KH is a diagnosis of exclusion that describes rare children who have abnormally limited fasting tolerance, experience recurrent episodes of KH, or develop symptoms of hypoglycemia despite elevated ketone levels, and in whom an explanation cannot be found despite extensive investigation. This review provides an approach to distinguishing between physiological KH and pathological KH and includes recommendations for management.

特发性病理性酮症低血糖:大海捞针。
患病儿童通常食欲下降、呕吐和腹泻,但低血糖症(血浆葡萄糖浓度≤50 毫克/分升或 2.8 毫摩尔/升)却很少见。酮症性低血糖(KH)是以前健康的 6 个月至 6 岁儿童在急诊科出现低血糖的最常见原因。酮症酸中毒和低血糖是幼儿对长期禁食的正常生理反应,这一点现已得到充分理解。现在有大量证据表明,KH 一词可以描述多种情况,包括幼儿禁食耐受性正常分布的下限以及许多损害禁食适应性的罕见疾病。分子基因检测技术的最新进展导致了这些罕见疾病的发现。特发性病理性酮症酸中毒是一种排除性诊断,用于描述空腹耐量异常受限、酮症酸中毒反复发作或在酮体水平升高的情况下仍出现低血糖症状的罕见儿童。本综述提供了一种区分生理性酮症酸中毒和病理性酮症酸中毒的方法,并提出了处理建议。
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来源期刊
Hormone Research in Paediatrics
Hormone Research in Paediatrics ENDOCRINOLOGY & METABOLISM-PEDIATRICS
CiteScore
4.90
自引率
6.20%
发文量
88
审稿时长
4-8 weeks
期刊介绍: The mission of ''Hormone Research in Paediatrics'' is to improve the care of children with endocrine disorders by promoting basic and clinical knowledge. The journal facilitates the dissemination of information through original papers, mini reviews, clinical guidelines and papers on novel insights from clinical practice. Periodic editorials from outstanding paediatric endocrinologists address the main published novelties by critically reviewing the major strengths and weaknesses of the studies.
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