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一名患有原发性单核细胞杀伤缺陷的儿童的曲霉性脊椎骨髓炎:对粒细胞巨噬细胞集落刺激因子治疗的反应

Aspergillus vertebral osteomyelitis in a child with a primary monocyte killing defect: response to GM-CSF therapy.

作者信息

Abu Jawdeh L, Haidar R, Bitar F, Mroueh S, Akel S, Nuwayri-Salti N, Dbaibo G S

机构信息

Department of Pediatrics, American University of Beirut, Lebanon.

出版信息

J Infect. 2000 Jul;41(1):97-100. doi: 10.1053/jinf.2000.0673.

Abstract

We report the first case of vertebral aspergillosis in a child with a primary defect in monocyte killing, an extremely rare immunodeficiency The diagnosis of defective monocyte killing was made by an in vitro assay that showed normal killing of Staphylococcus aureus by the patient's neutrophils but impaired killing by his monocytes. Importantly, the extensive granulomatous infection that involved the vertebral column, posterior mediastinum, pleura, and lung was not responsive to aggressive treatment with a combination of liposomal amphotericin B. intralesional amphotericin B. itraconazole, and granulocyte transfusions. Dramatic clinical and radiological improvement was only seen after the addition of granulocyte macrophage-colony stimulating factor (GM-CSF) to his treatment regimen. The use of GM-CSF in the treatment of invasive aspergillosis in immunocompromised patients requires further evaluation.

摘要

我们报告了首例患有单核细胞杀伤原发性缺陷的儿童椎体曲霉菌病,这是一种极其罕见的免疫缺陷病。单核细胞杀伤缺陷的诊断是通过体外试验做出的,该试验显示患者的中性粒细胞对金黄色葡萄球菌的杀伤正常,但单核细胞的杀伤受损。重要的是,累及脊柱、后纵隔、胸膜和肺部的广泛肉芽肿性感染对脂质体两性霉素B、病灶内注射两性霉素B、伊曲康唑和粒细胞输注的积极联合治疗无反应。仅在治疗方案中添加粒细胞巨噬细胞集落刺激因子(GM-CSF)后,才出现显著的临床和影像学改善。GM-CSF在免疫受损患者侵袭性曲霉菌病治疗中的应用需要进一步评估。

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