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Whole exome sequencing of 20 patients with pulmonary hypertension caused by fibrosing mediastinitis
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作者 Bo Li Siyi Liu +10 位作者 Jingwen Zhang Jinrui Song Hongling Su Yanwei Li Aiping Tang Yating Zhao Tongtong Gao Huan Liu Min Zhang Yan Zhang Yunshan Cao 《Chinese Medical Journal》 2026年第3期466-468,共3页
To the Editor:Fibrous mediastinitis(FM)is a rare group of diseases characterized by excessive proliferation of fibrous tissue in the mediastinum,which can cause bronchial stenosis,superior vena cava(SVC)obstruction,an... To the Editor:Fibrous mediastinitis(FM)is a rare group of diseases characterized by excessive proliferation of fibrous tissue in the mediastinum,which can cause bronchial stenosis,superior vena cava(SVC)obstruction,and pulmonary artery(PA)and vein(PV)stenosis.Moreover,long-term obstruction of PAs and PVs may lead to pulmonary hypertension(PH),right heart failure(RHF),and death.[1]Although it is generally held that FM is an aberrant immunoproliferative response of the mediastinum to a triggering factor,such as infection of Mycobacterium tuberculosis and Histoplasma capsulatum,the exact etiology and pathogenesis of FM remain unclear;in particular,the genetic susceptibility of this fatal disease is unknown. 展开更多
关键词 Exome Sequencing Genetic Susceptibility Fibrosing Mediastinitis excessive proliferation fibrous tissue aberrant immunoproliferative response bronchial stenosissuperior vena cava svc obstructionand Pulmonary Hypertension
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