Xiangwen Hu, S. Zeng, Yimin Zhu, Tian Yu, Bing Zhang, Tao Wang
{"title":"A case report of congenital hyperinsulinemia with focal hyperplasia of the pancreas and literature review","authors":"Xiangwen Hu, S. Zeng, Yimin Zhu, Tian Yu, Bing Zhang, Tao Wang","doi":"10.3760/CMA.J.ISSN.1673-4912.2020.01.015","DOIUrl":null,"url":null,"abstract":"目的 \n探讨先天性高胰岛素血症(CHI)患儿的临床特征、组织学分型，为儿科工作者提供诊断和治疗经验。 \n \n \n方法 \n回顾性分析我院收治的1例局灶增生型CHI患儿临床资料及预后，同时复习相关文献。 \n \n \n结果 \n患儿因反复低血糖、抽搐入院，我院诊断CHI，外院进一步18F-Dopa PET-CT及组织病理学检查，确诊为局灶增生型CHI。手术病灶切除治疗有效，随访血糖正常，但继发癫痫。 \n \n \n结论 \nCHI比较罕见，并且具有致死性及遗留神经系统后遗症，临床医生依据临床特征对高度怀疑CHI的患儿，应尽早完善18F-Dopa PET-CT检查，有助于早期确定CHI组织学分型，且能指导CHI的远期临床管理，减少神经系统损伤。","PeriodicalId":68901,"journal":{"name":"中国小儿急救医学","volume":"27 1","pages":"67-70"},"PeriodicalIF":0.0000,"publicationDate":"2020-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"中国小儿急救医学","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3760/CMA.J.ISSN.1673-4912.2020.01.015","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Objective: To explore the clinical characteristics and histological subtypes of congenital hyperinsulinemia (CHI) in children, and to provide diagnostic and therapeutic experience for pediatric workers. Method: A retrospective analysis was conducted on the clinical data and prognosis of a case of focal proliferative CHI admitted to our hospital, while reviewing relevant literature. The patient was admitted to the hospital due to repeated hypoglycemia and convulsions. Our hospital diagnosed CHI, and further 18F Dopa PET-CT and histopathological examinations in other hospitals confirmed the diagnosis of focal proliferative CHI. Surgical lesion resection was effective, and blood glucose levels were normal during follow-up, but secondary epilepsy occurred. Conclusion: CHI is relatively rare and has lethal and residual neurological sequelae. Clinicians should improve the 18F-Dopa PET-CT examination as early as possible for highly suspected CHI patients based on clinical characteristics, which helps to determine the histological classification of CHI in the early stage and can guide the long-term clinical management of CHI, reducing neurological damage.
期刊介绍：
Chinese Journal of Neurology was established in 1955, the predecessor of which is Chinese Journal of Neurology and Psychiatry. Chinese Journal of Neurology and Psychiatry has been indexed by MEDLINE until 1996, when it was divided into two journals, Chinese Journal of Neurology, and Chinese Journal of Psychiatry. Chinese Journal of Neurology is now indexed by EM, SCOPUS, AJ, WPRIM, CNKI, Wanfang Data, CSCD, etc. The impact factor of the journal is 2.755 in 2017, ranking the first among all neurological and psychological journals in China and among all the 142 medical journals published by the Chinese Medical Association. The journal is available both in print and online.
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