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家族性地中海热患者的肾移植。

Renal transplantation in patients with familial Mediterranean fever.

机构信息

Department of Nephrology, Faculty of Medicine, Ondokuz Mayıs University, Samsun 55139, Turkey.

出版信息

Clin Rheumatol. 2012 Aug;31(8):1183-6. doi: 10.1007/s10067-012-1992-6. Epub 2012 May 5.

Abstract

Amyloidosis is the most common and devastating complication of familial Mediterranean fever (FMF). Renal transplantation is the choice of treatment of in most end-stage renal disease (ESRD). We report our experience on the outcomes in eight patients who underwent renal transplantation for ESRD due to FMF secondary to amyloidosis, and we provide a discussion on the current evidence on this topic of study. The clinical charts of eight renal transplant patients (seven male, one female) who underwent ESRD due to FMF-related amyloidosis were investigated. Five patients underwent living-donor renal transplantation and three patients underwent deceased-donor renal transplant. The mean follow-up period was 35 months (range 3-72). All patients were on triple immunosuppressive treatment and received colchicine. All allografts are currently functioning well with a mean serum creatinine level of 1.4 (range 0.7-2.6) mg/dL. Posttransplantation complications included acute rejection (n = 4), chronic rejection (n = 1), severe gastroenteritis (n = 2), and erythrocytosis (n = 5). None of the patients had proteinuria. During follow-up, we did not observe clinically severe FMF attack, septicemia, rhabdomylosis, symptoms related to vasculitis, and clinical neuropathy. The clinical outcome of the patients in this cohort was similar to that of other renal transplant patients with ESRD due to other causes. This study shows favorable prognosis of eight ESRD patients due to amyloidosis caused by FMF after renal transplantation. Renal transplantation is a safe procedure for ESRD patients having amyloidosis due to FMF. Regular use of colchicine after transplantation should be mentioned.

摘要

淀粉样变性是家族性地中海热(FMF)最常见和最具破坏性的并发症。在大多数终末期肾病(ESRD)患者中,肾移植是首选治疗方法。我们报告了 8 例因 FMF 相关淀粉样变性导致 ESRD 而接受肾移植的患者的结果,并就该研究主题的现有证据进行了讨论。

研究调查了 8 例因 FMF 相关淀粉样变性导致 ESRD 而接受肾移植的肾移植患者(7 名男性,1 名女性)的临床病历。5 例患者接受活体供肾移植,3 例患者接受尸肾移植。平均随访时间为 35 个月(范围 3-72)。所有患者均接受三联免疫抑制治疗和秋水仙碱治疗。所有同种异体移植物目前功能良好,血清肌酐水平平均为 1.4(范围 0.7-2.6)mg/dL。移植后并发症包括急性排斥反应(n = 4)、慢性排斥反应(n = 1)、严重胃肠炎(n = 2)和红细胞增多症(n = 5)。无蛋白尿。在随访期间,我们未观察到临床严重 FMF 发作、败血症、横纹肌溶解症、与血管炎相关的症状和临床神经病。该队列患者的临床结果与其他因其他原因导致 ESRD 的肾移植患者相似。

本研究表明,8 例因 FMF 导致淀粉样变性的 ESRD 患者在肾移植后预后良好。肾移植是 FMF 引起淀粉样变性导致 ESRD 患者的一种安全手术。应提及移植后常规使用秋水仙碱。

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