近年来,已经发表了一些关于先天性孤立肾(CSK)儿童预后的研究,有争议的结果,在管理和后续行动方面缺乏全球共识。在这份共识声明中,意大利小儿肾脏病学会总结了CSK的最新知识,并提出了其管理建议,包括诊断方法,营养和生活习惯,和后续行动。我们建议通过新生儿超声(美国)确认任何产前怀疑/诊断为CSK,如果未检测到肾脏/泌尿道的其他异常,则避免常规使用进一步成像。没有额外异常的CSK预计会进行代偿性扩大,这应该由美国来评估。我们建议尿液分析,但不是血液测试或基因分析,在诊断为CSK代偿性增大的婴儿和儿童时常规进行。肾外畸形应该被检查,尤其是女性的生殖道畸形。应避免摄入过量的蛋白质和盐,而体育参与不应受到限制。我们建议终身随访,这应该是根据风险分层量身定制的,如下:低风险:具有补偿性扩大的CSK,中等风险:没有补偿性扩大和/或额外CAKUT的CSK,和高风险:降低GFR和/或蛋白尿,和/或高血压。我们建议在低风险周期性美国的儿童中,进行尿液分析和血压测量;在中等风险的人群中,我们建议也测量血清肌酐;在高危儿童中,时间表必须根据肾功能和临床数据进行调整。
In recent years, several studies have been published on the prognosis of children with congenital solitary kidney (CSK), with controversial results, and a worldwide
consensus on management and follow-up is lacking. In this
consensus statement, the Italian Society of Pediatric Nephrology summarizes the current knowledge on CSK and presents recommendations for its management, including diagnostic approach, nutritional and lifestyle habits, and follow-up. We recommend that any antenatal suspicion/diagnosis of CSK be confirmed by neonatal ultrasound (US), avoiding the routine use of further imaging if no other anomalies of kidney/urinary tract are detected. A CSK without additional abnormalities is expected to undergo compensatory enlargement, which should be assessed by US. We recommend that urinalysis, but not blood tests or genetic analysis, be routinely performed at diagnosis in infants and children showing compensatory enlargement of the CSK. Extrarenal malformations should be searched for, particularly genital tract malformations in females. An excessive protein and salt intake should be avoided, while sport participation should not be restricted. We recommend a lifelong follow-up, which should be tailored on risk stratification, as follows: low risk: CSK with compensatory enlargement, medium risk: CSK without compensatory enlargement and/or additional CAKUT, and high risk: decreased GFR and/or proteinuria, and/or hypertension. We recommend that in children at low-risk periodic US, urinalysis and BP measurement be performed; in those at medium risk, we recommend that serum creatinine also be measured; in high-risk children, the schedule has to be tailored according to kidney function and clinical data.