儿童遗传性胰腺炎的研究进展

于晓政; 吴捷

doi:10.12449/JCH240833

儿童遗传性胰腺炎的研究进展

DOI: 10.12449/JCH240833

于晓政,
吴捷^, ,

国家儿童医学中心，首都医科大学附属北京儿童医院消化科，北京 100045

基金项目:

北京市医院管理中心“登峰”人才培养计划 (DFL20221003)

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：于晓政负责查阅文献，资料分析，撰写论文，修改论文；吴捷负责拟定写作思路，指导撰写文章并最后定稿。

详细信息

通信作者:
吴捷， wujie@bch.com.cn （ORCID： 0000-0003-4028-6173）

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出版历程
- 收稿日期: 2023-11-22
- 录用日期: 2024-01-02
- 出版日期: 2024-08-25

Research advances in hereditary pancreatitis in children

Xiaozheng YU,
Jie WU^{,
,}

Department of Gastroenterology，Beijing Children’s Hospital，Capital Medical University，National Center for Children’s Health，Beijing 100045，China

Research funding:

Beijing Hospitals Authority’s Ascent Plan (DFL20221003)

More Information

Corresponding author: WU Jie， wujie@bch.com.cn （ORCID： 0000-0003-4028-6173）

摘要

摘要: 遗传性胰腺炎是儿童胰腺炎的主要致病因素之一，成年后癌变风险高。本文综述了遗传性胰腺炎的研究进展，包括历史发展、流行病学、致病机制及相关致病基因、临床特征与诊断及治疗等，旨在为延缓儿童遗传性胰腺炎疾病进程及基础研究提供新的参考信息。
- 遗传性胰腺炎 /
- 体征和症状 /
- 治疗学 /
- 儿童
Abstract: Hereditary pancreatitis （HP） is one of the major causes of pancreatitis in children and has a high risk of carcinogenesis after they become adults. This article reviews the recent research advances in HP， including historical development， epidemiology， pathogenic mechanism， and related pathogenic genes， clinical features， and diagnosis and treatment， in order to provide new reference information for delaying the progression of HP in children and conducting related basic research.
- Hereditary Pancreatitis /
- Signs and Symptoms /
- Therapeutics /
- Child

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表 1 不同年龄段CP患儿胰酶替代治疗推荐剂量

Table 1. Commended pancreatic enzyme replacement therapy dosing for children with chronic pancreatitis-associated exocrine pancreatic insufficiency

年龄	胰酶剂量
<1岁	每120 mL配方奶或母乳中加人2 000～4 000 U脂肪酶，或喂养膳食中每克脂肪含约2 000 U脂肪酶
≥1岁～4岁	喂养膳食中每克脂肪需含2 000～4 000 U脂肪酶，根据需要调整剂量，每日最大剂量为10 000 U脂肪酶/kg
≥4岁	每餐从500 U脂肪酶/kg开始，逐渐增加剂量至每餐1 000～2 500 U脂肪酶/kg，或每日10 000 U脂肪酶/kg，或喂养膳食中每克脂肪需含2 000～4 000 U脂肪酶

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