Objectives Transient hyperinsulinism (THI) is a hypoglycemia disorder which resolves spontaneously within the first weeks or months of life. The pathomechanism of THI is not elucidated yet; however, it is known that perinatal stress predisposes for THI. We aimed to characterize the clinical phenotype and treatment of children with THI, and to identify options for improved management. Methods A retrospective analysis of 36 children with THI treated at the University Children’s Hospital Düsseldorf between 2007 and 2019 was performed. Results All children had risk factors for neonatal hypoglycemia or indicators of perinatal stress. Eighty three percent were diagnosed with hypoglycemia on day of life (DOL)1. None of the six diagnosed later had routine blood glucose screening and showed significantly lower blood glucose levels at the time of first blood glucose measurement compared to the children diagnosed on DOL1. Ninety seven percent of all children received intravenous glucose, 42% received continuous glucagon and 81% were started on diazoxide. Diazoxide withdrawal and subsequent fasting tests lacked standardization and were based on clinical experience. Three patients had a subsequent episode of hypoglycemia, after fasting studies only demonstrated “clinical” remission without proving the ability to ketogenesis. Conclusions Any kind of perinatal stress might pose a risk to develop THI, and postnatal monitoring for hypoglycemia still needs to be improved. Diazoxide is effective in children with THI; however, further studies are needed to guide the development of criteria and procedures for the initiation and discontinuation of treatment. Furthermore, establishing consensus diagnostic criteria/definitions for THI would improve comparability between studies.

中文翻译：

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一过性高胰岛素血症的管理挑战——对 36 名严重受影响儿童的回顾性分析

目的 暂时性高胰岛素血症 (THI) 是一种低血糖症，可在出生后的最初几周或几个月内自行消退。THI的发病机制尚未阐明；然而，众所周知，围产期压力易导致 THI。我们旨在表征 THI 儿童的临床表型和治疗，并确定改善管理的选择。方法 对 2007 年至 2019 年在杜塞尔多夫大学儿童医院治疗的 36 例 THI 儿童进行回顾性分析。结果所有儿童均存在新生儿低血糖危险因素或围产期应激指标。83% 的人在出生当天 (DOL)1 被诊断出患有低血糖症。与在 DOL1 上诊断出的儿童相比，后来诊断出的六名儿童均未进行常规血糖筛查，并且在第一次血糖测量时显示出显着较低的血糖水平。97% 的儿童接受静脉注射葡萄糖，42% 接受持续胰高血糖素治疗，81% 开始服用二氮嗪。二氮嗪戒断和随后的空腹测试缺乏标准化，并且基于临床经验。三名患者随后出现低血糖，禁食研究仅证明“临床”缓解，而没有证明生酮能力。结论 任何一种围产期应激都有发生THI的风险，产后低血糖监测仍有待加强。二氮嗪对患有 THI 的儿童有效；然而，需要进一步的研究来指导制定开始和停止治疗的标准和程序。此外，为 THI 建立共识诊断标准/定义将提高研究之间的可比性。