IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome
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DOI：
10.1007/s00467-020-04519-1
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发表时间：
2020-05-07
影响因子：
3
通讯作者：
Haffner, Dieter
中科院分区：
文献类型：
--
作者：
Trautmann, Agnes;Vivarelli, Marina;Haffner, Dieter
特发性肾病综合征每年每10万名儿童中新发病1-3例。大约85%的病例显示糖皮质激素治疗后蛋白尿完全缓解。在糖皮质激素治疗4-6周内未达到完全缓解的患者称为类固醇抵抗性肾病综合征（SRNS）。在10-30%的类固醇耐药患者中，可检测到足细胞相关基因的突变，而在其余患者中，可能存在未定义的免疫来源循环因子。SRNS的诊断和治疗是一个巨大的挑战，因为它的病因不同，经常缺乏进一步免疫抑制治疗的缓解，以及严重的并发症，包括终末期肾脏疾病的发展和肾移植后复发。来自国际儿科肾病协会（IPNA）的儿科肾病学家和肾脏遗传学家、一名肾脏病理学家和一名成人肾病学家组成的专家小组现在已经就儿童SRNS的诊断和管理提出了全面的临床实践建议。该团队对9个临床相关PICO（患者或人群覆盖、干预、比较者、结果）问题进行了系统的文献综述，制定了建议，并在共识会议上对其进行了正式评分，患者代表、营养师（作为外部顾问）和儿科肾病专家投票小组提供了意见。并给出了研究建议。
Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.