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新生儿高胰岛素血症:英国一家三级新生儿重症监护病房的临床表现与治疗回顾性研究

Neonatal hyperinsulinism: a retrospective study of presentation and management in a tertiary neonatal intensive care unit in the UK.

作者信息

Kalogeropoulou Maria-Sofia, Couch Helen, Thankamony Ajay, Beardsall Kathy

机构信息

University of Cambridge School of Clinical Medicine, Cambridge, UK.

Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

出版信息

Arch Dis Child Fetal Neonatal Ed. 2025 Apr 17;110(3):261-268. doi: 10.1136/archdischild-2024-327322.

DOI:10.1136/archdischild-2024-327322
PMID:39304222
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12013591/
Abstract

OBJECTIVE

Reports of hyperinsulinism typically focus on infants managed by highly specialised services. However, neonates with hyperinsulinism are initially managed by neonatologists and often not referred to specialists. This study aimed to characterise the diversity in presentation and management of these infants.

SETTING

Level 3 neonatal intensive care.

PATIENTS

Neonates with hyperinsulinism, defined as blood glucose <2.8 mmol/mL and insulin level >6 pmol/L.

DESIGN

7-year retrospective study (January 2015-December 2021).

RESULTS

99 cases were identified: -treated with diazoxide (20%), -clinically concerning hyperinsulinism not treated with diazoxide (30%), -biochemical hyperinsulinism (50%). Birth weight z-score was -1.02±2.30 (mean±SD), 42% were preterm, but neither variable correlated with clinical severity. The group received a higher concentration of intravenous glucose (27±12%) compared with the (15±7%) and (16±10%) groups (p<0.001). At diagnosis, the intravenous glucose intake was similar in the (7.43±5.95 mg/kg/min) and (5.09±3.86 mg/kg/min) groups, but higher compared with the group (3.05+/2.21 mg/kg/min) (p<0.001). In the group, term infants started diazoxide earlier (9.9±4.3 days) compared with preterm (37±26 days) (p=0.002). The national congenital hyperinsulinism service was consulted for 23% of infants, and 3% were transferred.

CONCLUSIONS

This study highlights the diversity in clinical presentation, severity and prognosis of neonatal hyperinsulinism, irrespective of birth weight and gestational age. More infants were small rather than large for gestational age, and the majority had transient hyperinsulinism and were not referred to the national centre, or treated with diazoxide. Further research is required to understand the breadth of neonatal hyperinsulinism and optimal management.

摘要

目的

高胰岛素血症的报告通常聚焦于由高度专业化服务机构管理的婴儿。然而,患有高胰岛素血症的新生儿最初由新生儿科医生管理,且往往不会转诊至专科医生处。本研究旨在描述这些婴儿在临床表现和治疗方面的多样性。

背景

三级新生儿重症监护病房。

患者

患有高胰岛素血症的新生儿,定义为血糖<2.8 mmol/mL且胰岛素水平>6 pmol/L。

设计

7年回顾性研究(2015年1月至2021年12月)。

结果

共识别出99例病例:接受二氮嗪治疗的(20%)、临床上有明显高胰岛素血症但未接受二氮嗪治疗的(30%)、生化性高胰岛素血症的(50%)。出生体重z评分-1.02±2.30(均值±标准差),42%为早产儿,但这两个变量均与临床严重程度无关。与未治疗组(15±7%)和生化性高胰岛素血症组(16±10%)相比,治疗组接受的静脉葡萄糖浓度更高(27±12%)(p<0.001)。诊断时,治疗组(7.43±5.95 mg/kg/min)和未治疗组(5.09±3.86 mg/kg/min)的静脉葡萄糖摄入量相似,但高于生化性高胰岛素血症组(3.05±2.21 mg/kg/min)(p<0.001)。在治疗组中,足月儿开始使用二氮嗪的时间(9.9±4.3天)早于早产儿(37±26天)(p=0.002)。23%的婴儿咨询了全国先天性高胰岛素血症服务机构,3%的婴儿被转诊。

结论

本研究强调了新生儿高胰岛素血症在临床表现、严重程度和预后方面的多样性,与出生体重和胎龄无关。更多婴儿为小于胎龄儿而非大于胎龄儿,且大多数患有短暂性高胰岛素血症,未转诊至全国性中心或接受二氮嗪治疗。需要进一步研究以了解新生儿高胰岛素血症的范围和最佳治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/c9506bf61995/fetalneonatal-110-3-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/36e91ff22b2c/fetalneonatal-110-3-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/0099a3092e53/fetalneonatal-110-3-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/c9506bf61995/fetalneonatal-110-3-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/36e91ff22b2c/fetalneonatal-110-3-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/0099a3092e53/fetalneonatal-110-3-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a42/12013591/c9506bf61995/fetalneonatal-110-3-g003.jpg

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本文引用的文献

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Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus.网络管理先天性高胰岛素血症的标准化实践:英国国家协作共识。
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