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Ⅰ型神经纤维瘤病继发肺动脉高压二例

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Author:: No author available

Journal Title:: Chinese Journal of Tuberculosis and Respiratory Diseases

Issue:: 4

DOI:: 10.3760/cma.j.issn.1001-0939.2019.04.015

Key Word:: No keyword available

Abstract： Ⅰ型神经纤维瘤病是一种常染色体显性遗传疾病,源于胚胎发育时期神经嵴细胞发育异常,主要累及皮肤及神经系统,可对肺血管造成损害,引起肺动脉高压.虽然其并发肺动脉高压的发病率低,但患者一旦合并肺动脉高压,预后极差,目前病例多为个案报道.本文报道北京朝阳医院呼吸与危重症医学科诊治的2例Ⅰ型神经纤维瘤病继发性肺动脉高压病例,结合现有国内外文献,总结该病的临床特点.

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