非典型和药物性溶血性尿毒症综合征患者血液学缓解后停用Eculizumab治疗。

IF 1.6 Q2 MEDICINE, GENERAL & INTERNAL
Romanian Journal of Internal Medicine Pub Date : 2022-03-17 Print Date: 2022-03-01 DOI:10.2478/rjim-2021-0034
Hasan H Yeter, Ulver Derici, Turgay Arinsoy, Kadriye Altok, Yasemin Erten, Galip Guz
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引用次数: 1

摘要

介绍。目的是评估治疗性血浆置换(TPE)和eculizumab对非典型溶血性尿毒症综合征(aHUS)患者血液学和肾脏生存的影响,另外,检验eculizumab停止治疗的可靠性。这是一项观察性和回顾性研究,研究对象为18例诊断为aus的患者。研究人群的中位年龄为30岁(22-66岁)。18例患者中有4例单独使用TPE获得血液学缓解。然而,一名患者在三次TPE治疗后死亡。13例患者使用Eculizumab,未观察到死亡。治疗一年后,3例TPE患者中有2例(66%)肾功能改善,9例Eculizumab患者中有5例(56%)肾功能改善。我们停止了9例患者的eculizumab治疗。其中一名C3基因突变的患者在停药后出现疾病复发。Eculizumab停药后,没有药物相关性aHUS患者出现疾病复发。Eculizumab治疗是aHUS的救命疗法。对于肾功能稳定或肾生存期无预期的患者,在血液学缓解后至少6个月可考虑停药。此外,药物相关的病例似乎不容易长期复发。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Discontinuation of Eculizumab treatment after hematological remission in patients with atypical and drug-induced hemolytic uremic syndrome.

Introduction. The aim was to evaluate the effect of therapeutic plasma exchange (TPE) and eculizumab on hematological and renal survival in atypical hemolytic uremic syndrome (aHUS), and additionally, to examine the reliability of discontinuation of eculizumab treatment.Methods. This was an observational and retrospective study of 18 patients diagnosed with aHUS.Results. The median age of the study population was 30 (22-66) years. Four of 18 patients achieved hematological remission with the TPE alone. However, one patient died after three sessions of TPE. Eculizumab was used in 13 patients and no death was observed. One year after treatment, improved kidney function was observed in 2 of 3 (66%) patients for TPE and 5 of 9 (56%) patients for Eculizumab. We discontinued eculizumab treatment in 9 patients. One of the patients who had a C3 gene mutation experienced disease relapse after Eculizumab discontinuation. None of the patients who had drug associated aHUS developed disease relapse after Eculizumab discontinuation.Conclusion. Eculizumab treatment is a life-saving therapy in aHUS. Treatment discontinuation may be considered at least six months after hematologic remission in patients who had stable renal function or no expectancy for renal survival. Moreover, drug-associated cases seem to tend not to develop disease relapse in the long term.

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来源期刊
Romanian Journal of Internal Medicine
Romanian Journal of Internal Medicine MEDICINE, GENERAL & INTERNAL-
CiteScore
3.20
自引率
5.30%
发文量
35
审稿时长
15 weeks
期刊介绍: Romanian Journal of Physics is a journal publishing physics contributions on the following themes: •Theoretical Physics & Applied Mathematics •Nuclear Physics •Solid State Physics & Materials Science •Statistical Physics & Quantum Mechanics •Optics •Spectroscopy •Plasma & Lasers •Nuclear & Elementary Particles Physics •Atomic and Molecular Physics •Astrophysics •Atmosphere and Earth Science •Environment Protection
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