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儿童特发性肺含铁血黄素沉着症中的自身免疫与延迟诊断

Autoimmunity and Delayed Diagnosis in Pediatric Idiopathic Pulmonary Hemosiderosis.

作者信息

AlJassmi Abdulrahman M

机构信息

Pediatric Hematology/Oncology Department, Dubai Hospital, Dubai Health Authority, Dubai, United Arab Emirates.

出版信息

J Pediatr Hematol Oncol. 2020 May;42(4):e240-e243. doi: 10.1097/MPH.0000000000001513.

DOI:10.1097/MPH.0000000000001513
PMID:31107369
Abstract

Idiopathic pulmonary hemosiderosis is characterized by a triad of iron-deficiency anemia, hemoptysis, and radiographic diffuse lung infiltrates. However, the inconsistent initial presentation in children may cause a significant delay in diagnosis. Autoimmune reactivity seems to be the most acceptable theory of pathogenesis. We reported an 8-year-old boy presenting with a cough, fever, and difficulty breathing with a history of iron-deficiency anemia and an abnormal autoimmune response in the last 3 years. Perinuclear antineutrophil cytoplasmic antibodies were positive and chest computed tomography revealed patchy ground glass haziness. Bronchoalveolar lavage fluid showed hemosiderin-laden macrophages. The respiratory symptoms improved with oral corticosteroids.

摘要

特发性肺含铁血黄素沉着症的特征为缺铁性贫血、咯血和影像学上的弥漫性肺部浸润三联征。然而,儿童最初表现不一致可能导致诊断显著延迟。自身免疫反应似乎是最可接受的发病机制理论。我们报告了一名8岁男孩,出现咳嗽、发热和呼吸困难,过去3年有缺铁性贫血病史和异常自身免疫反应。核周抗中性粒细胞胞浆抗体呈阳性,胸部计算机断层扫描显示斑片状磨玻璃样模糊影。支气管肺泡灌洗 fluid显示含铁血黄素巨噬细胞。口服糖皮质激素后呼吸道症状改善。 (注:原文中“Bronchoalveolar lavage fluid”表述有误,应该是“Bronchoalveolar lavage fluid”,翻译为“支气管肺泡灌洗液体”,这里根据语境推测为“支气管肺泡灌洗液体中的”,整体翻译为“支气管肺泡灌洗液体显示含铁血黄素巨噬细胞” )

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引用本文的文献

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Exp Ther Med. 2020 Sep;20(3):2291-2297. doi: 10.3892/etm.2020.8938. Epub 2020 Jun 25.
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Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature.特发性肺含铁血黄素沉着症误诊为肺血管炎 1 例并文献复习
Curr Allergy Asthma Rep. 2020 Apr 4;20(5):13. doi: 10.1007/s11882-020-00907-7.