Wakabayashi Eduarda Almeida, Pantaleão Alexandre Negrão, Avendanha Renata Araújo, Brunheroto Felipe Baptista, Simões E Silva Ana Cristina
Interdisciplinary Laboratory of Medical Investigation, Department of Pediatrics, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Curr Med Chem. 2023;30(2):203-219. doi: 10.2174/0929867329666220629142556.
Solitary functioning kidney (SFK) is a subgroup of the Congenital Anomalies of the Kidneys and Urinary Tract (CAKUT). Although the prognosis of these patients was considered good in the past, numerous studies have shown different levels of kidney damage associated with this condition. Serum creatinine measurement is still the most used marker to assess renal function, even though the limitations are widely known.
The present review aims to summarize and update the scientific literature on congenital SFK, discussing its pathophysiology, diagnosis, complications, prognosis, role of novel urinary biomarkers, treatment, and follow-up.
The natural history of congenital SFK is still an unresolved issue due to several factors. Although it has not yet been proven in humans, Brenner's hyperfiltration hypothesis is the most concrete theory to explain the poor renal outcomes of patients born with one functioning kidney. The search for novel urinary biomarkers capable of assessing renal function and predicting renal outcomes has already started, but there are still few studies on this specific population. Among the most studied markers, Cystatin C, EGF and NGAL have shown potential usefulness for the follow-up of these patients. The treatment still relies on the search for kidney injury and general renoprotective measures.
Further research with a longer follow-up duration is needed to better understand the natural course of congenital SFK and the role of novel urinary biomarkers in this specific population. Thus, it will be possible to improve the prognosis of these patients.
孤立性功能肾(SFK)是先天性肾脏和尿路畸形(CAKUT)的一个亚组。尽管过去认为这些患者的预后良好,但大量研究表明,这种情况会导致不同程度的肾脏损害。血清肌酐测量仍然是评估肾功能最常用的指标,尽管其局限性广为人知。
本综述旨在总结和更新关于先天性SFK的科学文献,讨论其病理生理学、诊断、并发症、预后、新型尿液生物标志物的作用、治疗和随访。
由于多种因素,先天性SFK的自然病程仍是一个未解决的问题。尽管尚未在人类中得到证实,但布伦纳的超滤假说仍是解释单肾出生患者肾脏预后不良的最具体理论。寻找能够评估肾功能和预测肾脏预后的新型尿液生物标志物的研究已经开始,但针对这一特定人群的研究仍然很少。在研究最多的标志物中,胱抑素C、表皮生长因子(EGF)和中性粒细胞明胶酶相关脂质运载蛋白(NGAL)已显示出有助于对这些患者进行随访。治疗仍依赖于寻找肾脏损伤和一般的肾脏保护措施。
需要进行更长随访期的进一步研究,以更好地了解先天性SFK的自然病程以及新型尿液生物标志物在这一特定人群中的作用。这样,才有可能改善这些患者的预后。