McCaffrey James, Lennon Rachel, Webb Nicholas J A
Department of Paediatric Nephrology, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Oxford Road, Manchester, M13 9WL, UK.
Institute of Human Development, Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK.
Pediatr Nephrol. 2016 Sep;31(9):1383-402. doi: 10.1007/s00467-015-3241-0. Epub 2015 Nov 10.
Idiopathic nephrotic syndrome (INS) is one of the most common renal diseases found in the paediatric population and is associated with significant complications, including infection and thrombosis. A high proportion of children enter sustained remission before adulthood, and therapy must therefore mitigate the childhood complications, while minimising the long-term risk to health. Here we address the main complications of INS and summarise the available evidence and guidance to aid the clinician in determining the appropriate treatment for children with INS under their care. Additionally, we highlight areas where no consensus regarding appropriate management has been reached. In this review, we detail the reasons why routine prophylactic antimicrobial and antithrombotic therapy are not warranted in INS and emphasise the conservative management of oedema. When pharmacological intervention is required for the treatment of oedema, we provide guidance to aid the clinician in determining the appropriate therapy. Additionally, we discuss obesity and growth, fracture risk, dyslipidaemia and thyroid dysfunction associated with INS. Where appropriate, we describe how recent developments in research have identified potential novel therapeutic targets.
特发性肾病综合征(INS)是儿科人群中最常见的肾脏疾病之一,与包括感染和血栓形成在内的严重并发症相关。很大一部分儿童在成年前进入持续缓解期,因此治疗必须减轻儿童期并发症,同时将长期健康风险降至最低。在此,我们阐述INS的主要并发症,并总结现有证据和指南,以帮助临床医生确定其照护下INS患儿的适当治疗方法。此外,我们强调在适当管理方面尚未达成共识的领域。在本综述中,我们详细说明INS中不适合进行常规预防性抗菌和抗血栓治疗的原因,并强调水肿的保守管理。当需要药物干预治疗水肿时,我们提供指导以帮助临床医生确定适当的治疗方法。此外,我们讨论与INS相关的肥胖与生长、骨折风险、血脂异常和甲状腺功能障碍。在适当的情况下,我们描述研究的最新进展如何确定了潜在的新型治疗靶点。