梅干腹综合征发病机制及诊断与治疗研究进展  

Research Progress on Pathogenesis,Diagnosis and Treatment of Prune Belly Syndrome

作　　者：孙曼銮 姜惠跃 宋欣怡 刘丽丽 卜佳 SUN Man-luan;JIANG Hui-yue;SONG Xin-yi;LIU Li-li;BU Jia(Medical School,Shanxi Datong University,Datong Shanxi,037009;Department of Obstetrics,The Third People's Hospital of Datong,Datong Shanxi,037046)

机构地区：[1]山西大同大学医学院,山西大同037009 [2]大同市第三人民医院产科,山西大同037046

出　　处：《山西大同大学学报（自然科学版）》2023年第6期85-91,共7页Journal of Shanxi Datong University(Natural Science Edition)

基　　金：山西省大学生创新创业计划项目[20220798];山西大同大学大学生创新创业计划项目[XDC2021125]。

摘　　要：梅干腹综合征(Prune Belly Syndrome,PBS)是一种罕见的先天性疾病,临床表现多样,典型特征表现为三联征:腹壁肌肉缺如、泌尿系统畸形及隐睾。近年来对PBS诊断和治疗取得了较大进展,但其发病机制尚不明确。总结了PBS主流的发病机制假说,归纳了其病理、临床表现及临床分类系统的发展,并对目前常见的诊断和治疗进行了综述,通过进一步阐述PBS的目前治疗状况,为今后PBS的临床诊断、分类和治疗及预后判断提供相应参考依据。Prune belly syndrome(PBS) is a rare congenital disorder characterized by a triad of abdominal muscle deficiency,urinary malformation,and cryptorchidism.Significant progress has been made in the diagnosis and treatment of PBS in recent years,but its pathogenesis is still unclear.This article sums up the mainstream pathogenesis hypotheses of PBS,summarizes its pathology,clinical manifestations,and the development of clinical classification systems,and provides a review of common diagnoses and treatments.By further elaborating on the current treatment status of PBS,it provides corresponding reference basis for the clinical diagnosis,classification,treatment,and prognosis judgment of PBS in the future.

关 键 词：梅干腹综合征 Eagle-Barrett综合征 发病机制 罕见病 临床分类系统 

分 类 号：R715[医药卫生—妇产科学]