TY -的A2 -伯格纳,拉乌尔AU - Coutinho) j . AU - Chorao r . s . AU -奥利维拉,m . AU -桑托斯,c . r . PY - 2019 DA - 2019/03/17 TI -治疗肿瘤坏死因子受体相关的周期性发热综合征在终末期肾功能衰竭SP - 6819476六世- 2019 AB -肿瘤坏死因子受体相关的周期性综合症(陷阱)是一种罕见的单基因疾病autoinflammatory。最严重的表现是继发性淀粉样变。一位44岁男性出现肾病综合征。肾活检是结论性的继发性淀粉样变。病人和他的孩子从婴儿期就反复发热的历史时期。所有受试者积极TNFRSF1A基因的杂合的变体,证实诊断陷阱。病人发展到终末期肾功能衰竭和发达复发性心包炎集。他开始anakinra在血液透析他的显著降低血清淀粉样蛋白(SAA)的水平。与此同时他收到了尸肾移植和维护anakinra治疗。尽管肾功能衰竭最担心的并发症AA淀粉样变性引起的陷阱,小数据可用的安全anti-IL-1治疗严重肾衰竭患者。 The authors report this case of a patient on dialysis treated with anakinra in which no complications were registered. Though amyloidosis is established, the authors believe containing its progression and reducing inflammatory activity can improve patient prognosis and reduce recurrence of amyloidosis in kidney transplant, as has been demonstrated in transplanted patients due to familial Mediterranean fever amyloidosis. SN - 2090-6641 UR - https://doi.org/10.1155/2019/6819476 DO - 10.1155/2019/6819476 JF - Case Reports in Nephrology PB - Hindawi KW - ER -