先天性高胰岛素血症患儿的临床病程和转归。
Clinical Course and Outcome in Children with Congenital Hyperinsulinism.
机构信息
Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Pediatric Department, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
出版信息
Arch Iran Med. 2022 Jul 1;25(7):422-427. doi: 10.34172/aim.2022.70.
BACKGROUND
Hyperinsulinism is the most common cause of persistent or recurrent neonatal hypoglycemia that may result in neurological deficits. The treatment goal in these patients is prevention of hypoglycemia to decrease mortality and morbidity. This study was done to determine the clinical course and outcome in children with congenital hyperinsulinism (CHI) referring to Mofid Children's Hospital from 2011 to 2017.
METHODS
This study was done on 22 children with CHI referring to Mofid Children's Hospital from 2011 to 2017. The demographic, perinatal, clinical, laboratory, imaging, pharmacological, treatment and follow up data of these children were collected and analyzed.
RESULTS
Among 22 children with CHI, the mortality rate was higher among those who received hydrocortisone versus those who did not receive hydrocortisone (46% versus 40%).
CONCLUSION
According to the results of this study, hydrocortisone had a negative impact on the outcomes of these children, which is important in the management of hypoglycemia. The clinical course and outcome of children with CHI was better with medical compared to surgical treatment.
背景
高胰岛素血症是导致新生儿持续性或复发性低血糖的最常见原因，可能导致神经功能缺陷。这些患者的治疗目标是预防低血糖以降低死亡率和发病率。本研究旨在确定 2011 年至 2017 年期间就诊于莫菲德儿童医院的先天性高胰岛素血症（CHI）患儿的临床过程和结局。
方法
本研究纳入了 2011 年至 2017 年期间就诊于莫菲德儿童医院的 22 例 CHI 患儿。收集并分析了这些患儿的人口统计学、围产期、临床、实验室、影像学、药物治疗、治疗和随访数据。
结果
在 22 例 CHI 患儿中，接受氢化可的松治疗的患儿死亡率高于未接受氢化可的松治疗的患儿（46%对 40%）。
结论
根据本研究结果，氢化可的松对这些患儿的结局有负面影响，这在低血糖的管理中很重要。与手术治疗相比，药物治疗可改善 CHI 患儿的临床过程和结局。
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