Talbot-Smith A, Syn W-K, MacQuillan G, Neil D, Elias E, Ryan P
Department of Respiratory Medicine, Hereford County Hospital, UK.
Thorax. 2009 May;64(5):440-3. doi: 10.1136/thx.2008.099796.
This is the first report of familial idiopathic pulmonary fibrosis associated with hepatic nodular regenerative hyperplasia and bone marrow hypoplasia. Four members of one family presented with this triad of organ dysfunction. The response to immunosuppressive treatment was poor and all four members succumbed to the disease processes. The current literature is reviewed and mechanisms that could have been involved in the development of this new syndrome are proposed.
这是关于家族性特发性肺纤维化合并肝结节性再生性增生和骨髓发育不全的首例报告。一个家族的四名成员出现了这种三联征器官功能障碍。免疫抑制治疗反应不佳，所有四名成员均死于疾病进程。本文回顾了当前文献，并提出了可能与这种新综合征发生有关的机制。
