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Atypical hemolytic uremic syndrome: Unique clinical presentation linked to rare mutation.

作者信息

Menotti Sofia, Donini Martino, Pessolano Giuseppina, Tiro Livia, Cantini Maurizio, Croce Jacopo, Morandi Matteo, Mazzi Filippo, Donadello Katia, Olivieri Oliviero, Dima Francesco, De Marchi Sergio, Gambaro Giovanni, Polati Enrico, De Franceschi Lucia

机构信息

Department of Medicine University of Verona and AOUI Verona Verona Italy.

Department of Transfusion Medicine University Hospital Verona Italy.

出版信息

EJHaem. 2021 Sep 14;2(4):838-841. doi: 10.1002/jha2.288. eCollection 2021 Nov.

DOI:10.1002/jha2.288
PMID:35845199
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9175842/
Abstract
摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa0b/9175842/ab526a5ccd78/JHA2-2-838-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa0b/9175842/ab526a5ccd78/JHA2-2-838-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa0b/9175842/ab526a5ccd78/JHA2-2-838-g001.jpg

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The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naïve to complement inhibitor treatment.长效 C5 抑制剂 ravulizumab 对补体抑制剂治疗初治的儿童非典型溶血尿毒症综合征患者有效且安全。
Kidney Int. 2021 Jul;100(1):225-237. doi: 10.1016/j.kint.2020.10.046. Epub 2020 Dec 8.
3
Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study.
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Blood. 2021 May 6;137(18):2438-2449. doi: 10.1182/blood.2020009280.
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Gene Variations Provide Insights in the Pathogenesis of the Kidney Diseases Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy.基因变异为肾脏疾病非典型溶血尿毒症综合征和 C3 肾小球病的发病机制提供了新见解。
J Am Soc Nephrol. 2020 Feb;31(2):241-256. doi: 10.1681/ASN.2019050515. Epub 2020 Jan 24.
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Profiles of Coagulation and Fibrinolysis Activation-Associated Molecular Markers of Atypical Hemolytic Uremic Syndrome in the Acute Phase.在急性期，非典型溶血尿毒综合征相关凝血和纤溶激活分子标志物的特征。
J Atheroscler Thromb. 2020 Apr 1;27(4):353-362. doi: 10.5551/jat.49494. Epub 2019 Sep 4.
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Haematologica. 2019 May;104(5):919-928. doi: 10.3324/haematol.2018.198622. Epub 2019 Jan 10.
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