儿童特发性肾病综合征远期预后现状及影响因素

Long-term outcomes and influencing factors of idiopathic nephrotic syndrome in children

儿童特发性肾病综合征(idiopathic nephrotic syndrome,INS)是以大量蛋白尿、低蛋白血症、水肿及高脂血症为特征的临床综合征,病程长,病情容易复发和迁延,因长期使用激素、免疫抑制剂所带来的远期并发症影响患儿的身心健康及生活质量。大多数激素敏感型肾病综合征患儿在成年前治愈,部分患儿成年后仍可能复发;激素耐药型肾病综合征患儿远期预后较差。迄今为止,国内追踪INS患儿10年以上的长期预后及其影响因素的研究报道较为少见。该文综述了INS患儿的远期预后的相关文献,从肾脏生存、生长、心理健康、学习工作、结婚生育、病情复发及远期相关并发症等方面分析INS患儿的远期预后现状,探讨INS患儿远期预后不良的影响因素,并提出改善远期预后的方法,以期为INS治疗的临床决策及随访管理提供参考。

Pediatric idiopathic nephrotic syndrome(INS)is characterized by massive albuminuria,hypoproteinemia,edema and hyperlipidemia,with a long course and high probability of relapse and prolongation.Long-term complications caused by long-term usage of hormones and immunosuppressants in children with INS seriously affect their physical and mental health and quality of life.Most children with steroid-sensitive nephrotic syndrome can be cured before adulthood,while some of them relapse in adulthood.Long-term prognosis of children with steroid-resistant nephrotic syndrome is poor.There have been few studies in China followed the long-term outcomes and its related factors of children with INS over 10 years.The paper reviewed the literatures on the long-term outcomes of children with INS,including renal survival,growth,mental health,learning and work,marriage and fertility,disease recurrence and long-term related complications,to explore the factors related to the poor long-term outcomes of children with INS and to assist in clinical decision-making and follow-up management.