儿童巨噬细胞活化综合征：诊断与管理。
Macrophage Activation Syndrome in Children: Diagnosis and Management.
机构信息
Division of Pediatric Rheumatology, Department of Pediatrics, AIIMS, New Delhi; India. Correspondence to: Dr Narendra Kumar Bagri, Associate Professor, Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110 027, India.
Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
出版信息
Indian Pediatr. 2021 Dec 15;58(12):1155-1161. doi: 10.1007/s13312-021-2399-8. Epub 2021 Mar 26.
Abstract
Macrophage activation syndrome is a severe yet under-recognized complication encountered in pediatric rheumatology. It manifests as secondary hemophagocytic lymphohistiocytosis leading to a hyper-inflammatory state resulting from an underlying cytokine storm. If unchecked, it may lead to multiorgan failure and mortality. Early diagnosis and timely initiation of specific therapy is pivotal for a successful outcome. This review outlines the key clinical and laboratory features and management of macrophage activation syndrome.
摘要
巨噬细胞活化综合征是儿科风湿病中一种严重但认识不足的并发症。它表现为继发噬血细胞性淋巴组织细胞增生症，导致潜在细胞因子风暴引起的炎症反应过度。如果不加控制，可能导致多器官衰竭和死亡。早期诊断和及时开始特异性治疗对于获得良好结果至关重要。本文综述了巨噬细胞活化综合征的关键临床和实验室特征及治疗。
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3
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