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儿童患者的原发性血栓性微血管病

Primary Thrombotic Microangiopathy in Pediatric Patients.

作者信息

Aranzazu Ceballos Andrés David, María Martínez Sánchez Lina, Pamplona Sierra Ana Paulina, Vergara Yánez Daniela, Franco Hincapié Liliana, Baquero Rodriguez Richard

机构信息

Universidad Pontificia Bolivariana, Medellín, Colombia.

Hospital Pablo Tobón Uribe, Medellín, Colombia.

出版信息

Glob Pediatr Health. 2024 Dec 16;11:2333794X241307535. doi: 10.1177/2333794X241307535. eCollection 2024.

DOI:10.1177/2333794X241307535
PMID:39691204
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11650636/
Abstract

. Primary thrombotic microangiopathy includes hemolytic uremic syndrome caused by Shiga toxin-producing , atypical hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. . A retrospective study that included patients younger than 18 years diagnosed with primary thrombotic microangiopathy between 2011 and 2021. . Thirty patients were included, of which 63% corresponded to a hemolytic uremic syndrome caused by Shiga toxin-producing , and 30% to the atypical hemolytic uremic syndrome. The median age was 2.8 years and female sex predominated at 57%. On admission to the emergency room, fever and fatigue were the most frequent symptoms (93%), followed by oliguria and anuria (80%). 48% of patients received hemodialysis during their care. Mortality was estimated at 13%. . This study constitutes the largest series of primary thrombotic microangiopathy in the pediatric population of Latin America, where the etiological and clinical behavior of this condition is described.

摘要

原发性血栓性微血管病包括由产志贺毒素引起的溶血尿毒综合征、非典型溶血尿毒综合征和血栓性血小板减少性紫癜。一项回顾性研究纳入了2011年至2021年间诊断为原发性血栓性微血管病的18岁以下患者。共纳入30例患者,其中63%为产志贺毒素引起的溶血尿毒综合征,30%为非典型溶血尿毒综合征。中位年龄为2.8岁,女性占主导,为57%。急诊入院时,发热和疲劳是最常见的症状(93%),其次是少尿和无尿(80%)。48%的患者在治疗期间接受了血液透析。死亡率估计为13%。本研究是拉丁美洲儿科人群中最大的原发性血栓性微血管病系列研究,描述了该疾病的病因和临床行为。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/29c0/11650636/1174a0380f2d/10.1177_2333794X241307535-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/29c0/11650636/7d219dd81de7/10.1177_2333794X241307535-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/29c0/11650636/1174a0380f2d/10.1177_2333794X241307535-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/29c0/11650636/7d219dd81de7/10.1177_2333794X241307535-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/29c0/11650636/1174a0380f2d/10.1177_2333794X241307535-fig2.jpg

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本文引用的文献

1
Performance of the PLASMIC score is improved with a positive score defined as ≥5 and with the inclusion of neurological symptoms.将血浆评分(PLASMIC score)的阳性评分定义为≥5并纳入神经症状后,其表现得到改善。
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Proteinuria Increases the PLASMIC and French Scores Performance to Predict Thrombotic Thrombocytopenic Purpura in Patients With Thrombotic Microangiopathy Syndrome.蛋白尿可提高PLASMIC评分和法国评分预测血栓性微血管病综合征患者血栓性血小板减少性紫癜的效能。
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Functional and Genetic Landscape of Complement Dysregulation Along the Spectrum of Thrombotic Microangiopathy and its Potential Implications on Clinical Outcomes.
血栓性微血管病谱系中补体调节异常的功能和遗传图谱及其对临床结局的潜在影响。
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Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients.血栓性微血管病伴巨噬细胞活化综合征:多国 23 例患者研究。
J Pediatr. 2021 Aug;235:196-202. doi: 10.1016/j.jpeds.2021.04.004. Epub 2021 Apr 7.
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Retrospective and Systematic Analysis of Causes and Outcomes of Thrombotic Microangiopathies in Routine Clinical Practice: An 11-Year Study.常规临床实践中血栓性微血管病病因及转归的回顾性和系统性分析:一项为期11年的研究
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6
Relative incidence of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome in clinically suspected cases of thrombotic microangiopathy.血栓性微血管病临床疑似病例中血栓性血小板减少性紫癜和溶血性尿毒症综合征的相对发病率。
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Incidence, Risk Factors for and Outcomes of Transplant-Associated Thrombotic Microangiopathy.移植相关性血栓性微血管病的发生率、危险因素和结局。
Br J Haematol. 2020 Jun;189(6):1171-1181. doi: 10.1111/bjh.16457. Epub 2020 Mar 2.
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A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score.改良的 PLASMIC 评分包括乳酸脱氢酶/正常上限比值,比原始 PLASMIC 评分更能准确识别中国血栓性血小板减少性紫癜患者。
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