Groen In 't Woud Sander, Westland Rik, Feitz Wout F J, Roeleveld Nel, van Wijk Joanna A E, van der Zanden Loes F M, Schreuder Michiel F
Department for Health Evidence, Radboud University Medical Center, Radboud Institute for Health Sciences, Nijmegen, The Netherlands.
Department of Pediatric Nephrology, Radboudumc Amalia Children's Hospital, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
Eur Urol Open Sci. 2021 Feb 3;25:11-20. doi: 10.1016/j.euros.2021.01.003. eCollection 2021 Mar.
A congenital solitary functioning kidney (cSFK) is a common developmental defect that predisposes to hypertension and chronic kidney disease (CKD) as a consequence of hyperfiltration. Every urologist takes care of patients with a cSFK, since some will need lifelong urological care or will come with clinical problems or questions to an adult urologist later in life.
We aim to provide clear recommendations for the initial clinical management and follow-up of children with a cSFK.
PubMed and EMBASE were searched to identify relevant publications, which were combined with guidelines on related topics and expert opinion.
Initially, cSFK diagnosis should be confirmed and risk factors for kidney injury should be identified using ultrasound. Although more research into early predictors of kidney injury is needed, additional congenital anomalies of the kidney or urinary tract and absence of compensatory kidney hypertrophy have repeatedly been associated with a worse prognosis. The role of voiding cystourethrography and antibiotic prophylaxis remains controversial, and is complicated by the exclusion of children with a cSFK from studies. A yearly follow-up for signs of kidney injury is recommended for children with a cSFK. As masked hypertension is prevalent, annual ambulatory blood pressure measurement should be considered. During puberty, an increasing incidence of kidney injury is seen, indicating that long-term follow-up is necessary. If signs of kidney injury are present, angiotensin converting enzyme inhibitors are the first-line drugs of choice.
This overview points to the urological and medical clinical aspects and long-term care guidance for children with a cSFK, who are at risk of hypertension and CKD. Monitoring for signs of kidney injury is therefore recommended throughout life. Large, prospective studies with long-term follow-up of clearly defined cohorts are still needed to facilitate more risk-based and individualized clinical management.
Many children are born with only one functioning kidney, which could lead to kidney injury later in life. Therefore, a kidney ultrasound is made soon after birth, and other investigations may be needed as well. Urologists taking care of patients with a solitary functioning kidney should realize the long-term clinical aspects, which might need medical management.
先天性孤立功能性肾（cSFK）是一种常见的发育缺陷，由于超滤作用，易引发高血压和慢性肾脏病（CKD）。每位泌尿外科医生都会诊治患有cSFK的患者，因为有些患者需要终身泌尿外科护理，或者在成年后会带着临床问题或疑问前来咨询成年泌尿外科医生。
我们旨在为患有cSFK的儿童的初始临床管理和随访提供明确的建议。
检索了PubMed和EMBASE以识别相关出版物，并结合了相关主题的指南和专家意见。
首先，应通过超声检查确认cSFK诊断，并确定肾损伤的危险因素。尽管需要对肾损伤的早期预测因素进行更多研究，但反复发现肾脏或尿路的其他先天性异常以及缺乏代偿性肾肥大与预后较差有关。排尿性膀胱尿道造影和抗生素预防的作用仍存在争议，并且由于在研究中排除了患有cSFK的儿童而变得复杂。建议对患有cSFK的儿童进行年度随访，以检查肾损伤迹象。由于隐匿性高血压很常见，应考虑每年进行动态血压测量。在青春期，肾损伤的发生率会增加，这表明需要进行长期随访。如果出现肾损伤迹象，血管紧张素转换酶抑制剂是首选的一线药物。
本综述指出了患有cSFK的儿童的泌尿外科和内科临床方面以及长期护理指导，这些儿童有患高血压和CKD的风险。因此，建议终生监测肾损伤迹象。仍需要对明确界定的队列进行长期随访的大型前瞻性研究，以促进更基于风险和个性化的临床管理。
许多儿童出生时只有一个功能性肾脏，这可能在以后的生活中导致肾损伤。因此，出生后应尽快进行肾脏超声检查，可能还需要进行其他检查。照顾患有孤立功能性肾脏患者的泌尿外科医生应了解长期临床情况，这可能需要药物治疗。
