当前位置: X-MOL 学术BMC Nephrol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Late renal recovery after treatment over 1 year post-onset in an atypical hemolytic uremic syndrome: a case report.
BMC Nephrology ( IF 2.3 ) Pub Date : 2020-06-22 , DOI: 10.1186/s12882-020-01897-4
Yusuke Kuroki 1 , Koji Mitsuiki 1 , Kaneyasu Nakagawa 1 , Kazuhiko Tsuruya 2 , Ritsuko Katafuchi 3 , Hideki Hirakata 1 , Toshiaki Nakano 4
Affiliation  

Atypical hemolytic uremic syndrome (aHUS) is a life-threatening disease that leads to end-stage kidney disease if only a poor response to plasma exchanges (PEs) or eculizumab therapy is achieved. A 58-year-old Japanese man presented with thrombocytopenia, anemia, and kidney failure requiring dialysis without any underlying disease. A kidney biopsy revealed marked mesangiolysis in all glomeruli, compatible with thrombotic microangiopathy (TMA). Based on the positive anti- factor H antibody and negative result for secondary TMA, we diagnosed him as aHUS. Despite eculizumab administration after eight sessions of PE, neither platelet normalization nor kidney recovery was achieved. Eight months later, we discontinued eculizumab therapy due to anaphylactic reaction. At 15 months after the onset of TMA, his platelet count increased gradually from 40 to 150 × 103/μL with a decreased serum creatinine level and increased urine output, eventually allowing the withdrawal of dialysis therapy. A second kidney biopsy showed mesangial widening compatible with the healing of TMA. This case indicates that aHUS with PEs and eculizumab therapy has the potential for renal recovery even if over 1 year has passed.

中文翻译:

非典型溶血性尿毒症综合征发病 1 年后肾功能恢复晚期:病例报告。

非典型溶血性尿毒症综合征 (aHUS) 是一种危及生命的疾病,如果对血浆置换 (PE) 或依库珠单抗治疗的反应不佳,则会导致终末期肾病。一名 58 岁的日本男性因血小板减少症、贫血和肾衰竭就诊,需要透析而无任何基础疾病。肾活检显示所有肾小球都有明显的系膜溶解,与血栓性微血管病 (TMA) 相符。基于抗 H 因子抗体阳性和继发性 TMA 阴性结果,我们诊断他为 aHUS。尽管在八次 PE 后给予依库珠单抗,但血小板正常化和肾脏恢复均未实现。八个月后,由于过敏反应,我们停止了依库珠单抗治疗。在 TMA 发病后 15 个月,他的血小板计数从 40 逐渐增加到 150 × 103/μL,血清肌酐水平下降,尿量增加,最终允许停止透析治疗。第二次肾活检显示系膜增宽与 TMA 愈合相符。该病例表明,即使超过 1 年,aHUS 与 PE 和依库珠单抗治疗仍有肾脏恢复的潜力。
更新日期:2020-06-23
down
wechat
bug