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一名乙型血友病患者反复出现弥漫性肺泡出血。

Repeated Diffuse Alveolar Hemorrhage in a Patient with Hemophilia B.

作者信息

Kasai Hajime, Terada Jiro, Hoshi Hiromasa, Urushibara Takashi, Kato Fumiaki, Nishimura Rintaro, Tatsumi Koichiro

机构信息

Department of Respirology, Graduate School of Medicine, Chiba University, Japan.

出版信息

Intern Med. 2017;56(4):425-428. doi: 10.2169/internalmedicine.56.7614. Epub 2017 Feb 15.

Abstract

Diffuse alveolar hemorrhage (DAH) is a life-threatening complication that occurs in association with various diseases including coagulation disorders. In rare cases, it is caused by hemophilia. A 48-year-old man was admitted to our hospital for a third time due to DAH. Although the cause of DAH could not be identified by bronchoscopy or laboratory tests, a good response to corticosteroids suggested idiopathic DAH with pulmonary capillaritis. The patient was diagnosed with hemophilia B based on the results of a detailed inquiry, a mildly prolonged activated partial thromboplastin time, and low factor IX activity. Hemophilia may be an underlying factor that exacerbates the bleeding of patients with DAH, even when they show a good response to corticosteroids.

摘要

弥漫性肺泡出血(DAH)是一种危及生命的并发症,与包括凝血障碍在内的多种疾病相关。在罕见情况下,它由血友病引起。一名48岁男性因DAH第三次入住我院。尽管通过支气管镜检查或实验室检查无法确定DAH的病因,但对皮质类固醇有良好反应提示为伴有肺毛细血管炎的特发性DAH。根据详细询问结果、活化部分凝血活酶时间轻度延长以及因子IX活性降低,该患者被诊断为B型血友病。血友病可能是加重DAH患者出血的潜在因素,即使他们对皮质类固醇表现出良好反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/77ae/5364196/0b868124ec99/1349-7235-56-0425-g001.jpg

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