Zhang Li, Yang Chunjiang, Liu Xing, He Dawei, Lin Tao, Zhang Yuanyuan, Wei Guanghui, Zhang Deying
Department of Pediatric Surgery, West China Second University Hospital, Sichuan University, Chengdu, China.
Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, China.
Pediatr Res. 2025 Feb 25. doi: 10.1038/s41390-025-03950-0.
Renal dysplasia is a common congenital birth defect in childhood, caused by fetal genetic defects, epigenetic modification disorders, or environmental factors. Maternal malnutrition, placental insufficiency, and exposure to harmful substances such as alcohol, angiotensin-converting enzyme inhibitors, and cocaine during pregnancy increase the risk of fetal renal dysplasia. The pathogenesis of this disease involves abnormal formation of renal units, leading to structural and functional abnormalities of the kidney. If left untreated, renal dysplasia can progress to chronic kidney disease (CKD) in children. This review explores the etiology and pathogenesis of renal dysplasia, emphasizing the intrinsic link between renal dysplasia and CKD through various pathological pathways. Additionally, we propose potential therapeutic agents targeting these mechanisms. We also highlight future research directions to further understand and address this issue. We hope this review will deepen clinicians' understanding of renal dysplasia and promote further laboratory research in this area. IMPACT: 1. This review comprehensively summarizes and elucidates the complex relationship between renal dysplasia and chronic kidney disease (CKD) based on previous research, offering new directions for related studies. 2. It expands upon conservative treatment approaches for renal dysplasia, providing more clinical options for therapeutic intervention.
肾发育不良是儿童期常见的先天性出生缺陷，由胎儿基因缺陷、表观遗传修饰紊乱或环境因素引起。母亲营养不良、胎盘功能不全以及孕期接触酒精、血管紧张素转换酶抑制剂和可卡因等有害物质会增加胎儿肾发育不良的风险。该病的发病机制涉及肾单位的异常形成，导致肾脏结构和功能异常。若不治疗，肾发育不良可进展为儿童慢性肾脏病（CKD）。本综述探讨肾发育不良的病因和发病机制，通过各种病理途径强调肾发育不良与CKD之间的内在联系。此外，我们提出了针对这些机制的潜在治疗药物。我们还强调了未来的研究方向，以进一步理解和解决这一问题。我们希望本综述能加深临床医生对肾发育不良的理解，并促进该领域进一步的实验室研究。影响：1. 本综述基于以往研究全面总结并阐明了肾发育不良与慢性肾脏病（CKD）之间的复杂关系，为相关研究提供了新方向。2. 它扩展了肾发育不良的保守治疗方法，为治疗干预提供了更多临床选择。
