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系统性红斑狼疮背景下的两种并存的血栓性微血管病:一例报告

Two concomitant thrombotic microangiopathies in the background of systemic lupus erythematosus: A case report.

作者信息

AlGhobaishi Abdullah, Mousa Ahmed Hafez, Alshaltoni Reham Salama, Mohsen Amani Sail, Baothman Abdullah, Adem Hanan, Eisa Yasir, Amin Abeer, Edrees Burhan

机构信息

Department of Pediatrics, King Fahad Armed Forces Hospital, Jeddah, Saudi Arabia.

Department of Pediatrics, Saudi German Hospital, Jeddah, Saudi Arabia.

出版信息

Ann Med Surg (Lond). 2022 Sep 1;81:104541. doi: 10.1016/j.amsu.2022.104541. eCollection 2022 Sep.

DOI:10.1016/j.amsu.2022.104541
PMID:36147129
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9486710/
Abstract

INTRODUCTION AND IMPORTANCE

Lupus nephritis is particularly a very concerning occurrence due to the susceptibility for potential renal damage and ultimately renal failure. Cardiac involvement was present as well in the form of pericarditis. Our study reports a case of lupus nephritis that has had a very severe course of fluctuations between relapses and improvements which constantly necessitated an MDT interference at various points.

CASE PRESENTATION

We report a case of a 13-year-old female patient who presented with a 5-day history of fever, dizziness, joint pain, menorrhagia, convulsions, and visual disturbances. Essential diagnostic tests took place and a diagnosis of lupus nephritis was confirmed.

CONCLUSION

In conclusion we found that a combination of various treatment modalities and flexibility in decision making in response to changes in the clinical course are vital to treatment success. Utilization of plasma exchange which resulted in an enormous drop in the percentage of fragmented red blood cells (RBCs) from 9.8% to 1.8%.

摘要

引言与重要性

狼疮性肾炎尤其令人担忧,因为它有导致潜在肾损伤并最终发展为肾衰竭的风险。同时还存在以心包炎形式出现的心脏受累情况。我们的研究报告了一例狼疮性肾炎病例,其病情在复发和好转之间波动剧烈,在不同阶段都需要多学科团队(MDT)的干预。

病例介绍

我们报告一例13岁女性患者,有5天发热、头晕、关节疼痛、月经过多、抽搐和视觉障碍病史。进行了必要的诊断检查,确诊为狼疮性肾炎。

结论

总之,我们发现多种治疗方式相结合以及根据临床病程变化灵活决策对治疗成功至关重要。采用血浆置换后,破碎红细胞(RBC)百分比从9.8%大幅降至1.8%。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/76e958a41248/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/506b4778fa45/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/bf843c430904/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/c59aca29146d/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/76e958a41248/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/506b4778fa45/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/bf843c430904/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/c59aca29146d/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b53/9486710/76e958a41248/gr4.jpg

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本文引用的文献

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Atypical Hemolytic Uremic Syndrome: New Challenges in the Complement Blockage Era.非典型溶血尿毒综合征:补体阻断时代的新挑战。
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Long-term outcomes and response to treatment in diacylglycerol kinase epsilon nephropathy.二酰基甘油激酶 ε 肾病的长期预后和治疗反应。
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Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases.血栓性血小板减少性紫癜合并系统性红斑狼疮患者的临床特征和预后:25 例病例回顾。
Ital J Pediatr. 2019 Apr 29;45(1):55. doi: 10.1186/s13052-019-0641-y.
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Childhood-Onset Systemic Lupus Erythematosus: A Review and Update.儿童期起病的系统性红斑狼疮:综述与更新
J Pediatr. 2018 May;196:22-30.e2. doi: 10.1016/j.jpeds.2018.01.045.
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Thrombotic Microangiopathy and the Kidney.血栓性微血管病与肾脏。
Clin J Am Soc Nephrol. 2018 Feb 7;13(2):300-317. doi: 10.2215/CJN.00620117. Epub 2017 Oct 17.
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Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab.狼疮性肾炎继发非典型溶血尿毒综合征,对依库珠单抗有反应。
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