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赫曼斯基-普德拉克综合征合并非特异性间质性肺炎一例

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Author:: No author available

Journal Title:: Chinese Journal of Tuberculosis and Respiratory Diseases

Issue:: 4

DOI:: 10.3760/cma.j.cn112147-20200903-00968

Key Word:: No keyword available

Abstract：本文报道1例赫曼斯基-普德拉克综合征合并非特异性间质性肺炎（NSIP）的患者，主要临床表现为进行性呼吸困难，胸部高分辨率CT示双肺小叶间隔增厚，可见双侧胸膜下网格影及斑片状磨玻璃影。肺组织病理学符合NSIP，最终经基因分析为HPS1基因突变确诊。给予醋酸泼尼松联合吡非尼酮治疗后，患者呼吸困难症状有所改善。

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