儿童神经源性膀胱纤维化研究进展

Research progress in neurogenic bladder fibrosis in children

儿童神经源性膀胱(PNB)多为腰骶部脊髓和神经发育不良所致,至今仍无理想的治愈方法。PNB常发生纤维化,如何预防和治疗PNB的纤维化也是世界难题。PNB随着时间的推移大多发生膀胱纤维化,表现为膀胱壁增厚、膀胱顺应性减小和膀胱出口梗阻等。研究提示膀胱纤维化不仅与膀胱平滑肌细胞有关,膀胱上皮细胞和间质细胞也参与其中,但纤维化的机制仍不清楚。转化生长因子-β_(1)(TGF-β_(1))/Smad、膀胱高压及与之相关的血管紧张素Ⅱ(AngⅡ)等信号通路的变化与之有关,动物模型中已有针对各种因子的抗纤维化治疗方法,但临床仍缺乏验证。对残余尿增多的PNB患者尽早进行清洁间歇导尿是否可预防纤维化有待进一步探讨。现就PNB纤维化研究进展进行综述,以期为临床提供参考。

Pediatric neurogenic bladder(PNB)is mostly caused by the dysplasia of lumbosacral spinal cord and nerve,and there is no effective treatment available at present.Bladder fibrosis occurs frequently in PNB,and the prevention and treatment of PNB fibrosis is still a challenge worldwide.Most PNBs develop bladder fibrosis over time,which is characterized by the thickened bladder wall,decreased bladder compliance,and obstruction of the bladder outlet.According to some studies,bladder fibrosis is not only related to bladder smooth muscle cells,but also epithelial cells and mesenchymal cells of bladder.However,the mechanism of fibrosis remains unclear.It has been reported that it is associated with the changes of transforming growth factor-β_(1)(TGF-β_(1))/Smad,bladder high pressure and related angiotensinⅡsignal pathways.Although anti-fibrotic therapies that target a variety of factors have been employed in animal models,the clinical validation is still needed.It is required to conduct a further exploration on whether early clean intermittent catheterization could prevent the fibrosis in PNB patients with increased residual urine.In this paper,the research progress in PNB fibrosis would be reviewed,in order to provide reference for clinical practice.