抗磷脂综合征起病时伴有溶血性贫血和心脑事件:病例报告。

IF 2.1 3区 医学 Q2 PEDIATRICS Frontiers in Pediatrics Pub Date : 2024-10-18 eCollection Date: 2024-01-01 DOI:10.3389/fped.2024.1370285
Jie Zheng, Zhao-Yu Wei, Shi-Chao Lin, Yong Wang, Xin Fang
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引用次数: 0

摘要

背景:抗磷脂综合征(APS)是一种全身性自身免疫性疾病,可表现为儿童血栓形成,其特点是抗磷脂抗体持续阳性。APS在儿童中并不常见,可能是非标准表现:一名六岁的中国女孩因黄疸和深色尿液就诊,诊断为溶血性贫血。泼尼松治疗最初改善了她的肤色,但后来她出现了神经系统症状。进一步的实验室检查显示,她出现了血管内溶血、凝血异常和狼疮抗凝物(LA)阳性检测结果。磁共振成像(MRI)扫描显示脑桥和小脑半球信号异常,基底动脉部分闭塞。她随后被诊断为自身免疫性脑炎,接受了免疫球蛋白(IG)和大剂量糖皮质激素(GC)治疗,临床症状有所改善。然而,两年后溶血性贫血症状加重。随后的实验室评估显示存在血管内溶血、凝血异常以及抗心磷脂、LA 和抗-beta2 糖蛋白 I 抗体检测阳性。肌钙蛋白 I 和 N 端前脑钠肽水平升高,加上心电图和超声心动图检查结果,表明患者患有心肌梗死,左心耳有血栓样肿块。脑磁共振成像显示多灶性梗死和脑血管阻塞。她被诊断为伴有溶血性贫血、脑血管阻塞和心肌梗死的 APS。在使用 GC、IG、利妥昔单抗、羟氯喹单用低分子量肝素钠和华法林治疗数周后,患者病情明显好转:儿科医生应熟悉小儿 APS 的各种表现,及时发现可能与 aPL 相关的并发症,并尽早采取适当的治疗策略。
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Antiphospholipid syndrome onset with hemolytic anemia and accompanied cardiocerebral events: a case report.

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder that can manifest as thrombosis in the pediatric population, characterized by persistently positive antiphospholipid antibodies. APS is infrequently observed in children and could represent non-criteria manifestations.

Case presentation: A six-year-old Chinese female presented with jaundice and dark urine, leading to a diagnosis of hemolytic anemia. Prednisone therapy initially improved her complexion, but she later developed neurological symptoms. Further laboratory tests showed intravascular hemolysis, coagulation abnormalities, and a positive lupus anticoagulant (LA) test result. Magnetic resonance imaging (MRI) scan revealed abnormal signals in the pons and cerebellar hemispheres, and an occluded part of the basilar artery. She was subsequently diagnosed with autoimmune encephalitis and received IG(immunoglobulin) and high-dose glucocorticoid (GC) treatment, leading to improvement in her clinical symptoms. However, the symptoms of hemolytic anemia worsened after two years. Subsequent laboratory assessments demonstrated the presence of intravascular hemolysis, coagulation abnormalities, and positive tests of anticardiolipin, LA, and anti-beta2 glycoprotein I antibodies. Elevated troponin I and N-terminal pro-brain natriuretic peptide levels, along with electrocardiogram and echocardiogram findings, indicated a myocardial infarction and a thrombus-like mass in the left auricle. Brain MRI showed multifocal infarction and cerebrovascular obstruction. She was diagnosed with APS accompanied by hemolytic anemia, cerebrovascular obstruction, and myocardial infarction. After several weeks of treatment with GC, IG, rituximab, hydroxychloroquine alone with low-molecular-weight heparin sodium, and warfarin, there was a marked improvement in the patient's condition.

Conclusion: Pediatricians should be familiar with various presentations of pediatric APS to promptly detect possible aPL-related complications and initiate appropriate management strategies early on.

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来源期刊
Frontiers in Pediatrics
Frontiers in Pediatrics Medicine-Pediatrics, Perinatology and Child Health
CiteScore
3.60
自引率
7.70%
发文量
2132
审稿时长
14 weeks
期刊介绍: Frontiers in Pediatrics (Impact Factor 2.33) publishes rigorously peer-reviewed research broadly across the field, from basic to clinical research that meets ongoing challenges in pediatric patient care and child health. Field Chief Editors Arjan Te Pas at Leiden University and Michael L. Moritz at the Children''s Hospital of Pittsburgh are supported by an outstanding Editorial Board of international experts. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Pediatrics also features Research Topics, Frontiers special theme-focused issues managed by Guest Associate Editors, addressing important areas in pediatrics. In this fashion, Frontiers serves as an outlet to publish the broadest aspects of pediatrics in both basic and clinical research, including high-quality reviews, case reports, editorials and commentaries related to all aspects of pediatrics.
期刊最新文献
Antiphospholipid syndrome onset with hemolytic anemia and accompanied cardiocerebral events: a case report. Analysis of early and treatment related deaths among children and adolescents with acute myeloid leukemia in Poland: 2005-2023. Case Report: Functional characterization of a missense variant in INSR associated with hypoketotic hypoglycemia. Clinical prognostic models in children with sepsis in low- and middle-income countries: a systematic review and meta-analysis. Decreased TREC and KREC levels in newborns with trisomy 21.
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