中国实用儿科杂志2025,Vol.40Issue(11):881-885,5.DOI:10.19538/j.ek2025110601
重视儿童Peutz-Jeghers综合征的全流程规范化管理
Standardized whole-process management of Peutz-Jeghers syndrome in children:a critical need
摘要
Abstract
Peutz-Jeghers syndrome(PJS)is an autosomal dominant disorder caused by pathogenic variants in the STK11 gene,characterized by mucocutaneous pigmentation,gastrointestinal hamartomatous polyps,and an increased susceptibility to cancer.In pediatric patients,complications due to gastrointestinal polyps are the primary factors that threaten the life and health of PJS children.Currently,many challenges remain in the clinical management of PJS,including delayed diagnosis,high rates of complications,and non-standardized management approaches.This review focuses on early detection of PJS in children,management strategy of polyps,tumor monitoring protocols,and key points in familial management,aiming to improve the long-term prognosis of PJS children by emphasizing the importance of standardized,whole-process management.关键词
Peutz-Jeghers综合征/错构瘤性息肉/规范化管理Key words
Peutz-Jeghers syndrome/hamartomatous polyps/standardized management分类
医药卫生引用本文复制引用
FANG Ying,XIONG Shi-qiu..重视儿童Peutz-Jeghers综合征的全流程规范化管理[J].中国实用儿科杂志,2025,40(11):881-885,5.基金项目
陕西省重点研发计划(2024SF2-GJHX-15) (2024SF2-GJHX-15)