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儿童中脑梗死伴原发性灾难性抗磷脂综合征:一例报告

Primary catastrophic antiphospholipid syndrome in children with midbrain infarction: a case report.

作者信息

Dong Qinghua, Yin Jianyun, Su Hang, Ni Qian

机构信息

The Second Clinical Medical College of Lanzhou University, Lanzhou University, Lanzhou, China.

Pediatric Respiratory Medicine, Lanzhou University Second Hospital, Lanzhou, China.

出版信息

Front Pediatr. 2024 Apr 8;12:1370843. doi: 10.3389/fped.2024.1370843. eCollection 2024.

Abstract

BACKGROUND

Catastrophic antiphospholipid syndrome (CAPS) is a multi-system autoimmune disease characterized by extensive thrombosis. Pediatric CAPS is extremely rare and associated with a high mortality rate, especially when midbrain infarction is involved. Hence, early diagnosis and prompt initiation of appropriate treatment for CAPS complicated by midbrain infarction are of utmost importance in achieving favorable outcomes.

CASE PRESENTATION

In this report, we present the case of a 14-year-old girl who presented with neurological symptoms and digestive system infection and was initially diagnosed with an "intracranial infection". After a series of rigorous diagnostic procedures, the patient was ultimately diagnosed with primary CAPS and was immediately transferred to the intensive care unit where she was treated with anticoagulation, glucocorticoids, intravenous immunoglobulin (IVIG) therapy, and multiple plasma infusions. Twenty-seven days after admission, the patient's condition improved with standardized treatment, and she was discharged and followed up regularly.

CONCLUSION

This case report provides a description of the clinical features observed in a pediatric patient with CAPS and concurrent midbrain infarction, highlighting the crucial role of early diagnosis and timely treatment in influencing patient prognosis.

摘要

背景

灾难性抗磷脂综合征(CAPS)是一种以广泛血栓形成为特征的多系统自身免疫性疾病。儿童CAPS极为罕见,且死亡率高,尤其是合并中脑梗死时。因此,对于合并中脑梗死的CAPS患者,早期诊断并及时开始适当治疗对于取得良好预后至关重要。

病例介绍

在本报告中,我们介绍了一名14岁女孩的病例,她出现神经症状和消化系统感染,最初被诊断为“颅内感染”。经过一系列严格的诊断程序,患者最终被诊断为原发性CAPS,并立即被转入重症监护病房,在那里接受了抗凝、糖皮质激素、静脉注射免疫球蛋白(IVIG)治疗和多次血浆输注。入院27天后,患者经标准化治疗病情好转,出院并定期随访。

结论

本病例报告描述了一名患有CAPS并并发中脑梗死的儿科患者的临床特征,强调了早期诊断和及时治疗对患者预后的关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3611/11033311/f02a5020bf46/fped-12-1370843-g001.jpg

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