赫尔曼斯基-普德拉克综合征合并肺纤维化：影像学与病理学相关性及肺部并发症综述
Hermansky-pudlak syndrome complicated by pulmonary fibrosis: radiologic-pathologic correlation and review of pulmonary complications.
作者信息
Kelil Tatiana, Shen Jeanne, O'Neill Ailbhe C, Howard Stephanie A
机构信息
Department of Imaging, Brigham and Women's Hospital, Boston, MA, USA.
Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA.
出版信息
J Clin Imaging Sci. 2014 Oct 27;4:59. doi: 10.4103/2156-7514.143437. eCollection 2014.
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous hypopigmentation, platelet dysfunction, and in many cases, life-threatening pulmonary fibrosis. We report the clinical course, imaging, and postmortem findings of a 38-year-old female with HPS-related progressive pulmonary fibrosis, highlighting the role of imaging in assessment of disease severity and prognosis.
赫尔曼斯基-普德拉克综合征（HPS）是一种罕见的常染色体隐性疾病，其特征为眼皮肤色素减退、血小板功能障碍，在许多情况下还会出现危及生命的肺纤维化。我们报告了一名38岁患有HPS相关进行性肺纤维化女性的临床病程、影像学表现及尸检结果，强调了影像学在评估疾病严重程度和预后中的作用。
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