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Clinical features and prognosis of pleuroparenchymal fibroelastosis: a single center experience
( Jae Ha Lee ),( Eun Jin Chae ),( Joon Seon Song ),( Dong Soon Kim ),( Jin Woo Song ) 대한결핵 및 호흡기학회 2017 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.124 No.-
Background: Pleuroparenchymal fibroelastosis (PPFE) is one of rare idiopathic interstitial pneumonias (IIP); however, clinical features and prognosis are not well defined. Methods: Patients (n=18), who were diagnosed by lung biopsy (surgical lung biopsy: 10, transbronchial lung biopsy: 8) from 2010 to 2017 at Asan medical center, were included and clinical data of them were retrospectively reviewed. Results: Median follow-up period was 12.5 months. Mean age was 62.7±9.9 years old, 67% were men and 55.6% were smokers. Cough (77.8%) and dyspnea (88.9%) were the most frequent presenting symptoms. About half of the subjects had a history of pulmonary infection (38.9%), chemotherapy (5.6%) and organ transplantation (5.6%). Most patients (66.7%) showed restrictive pattern on lung function test (mean FVC: 61.5±21.5% predicted, DLco: 57.6±18.6% predicted) and mild neutrophilia (mean: 10.1±13.8%) on bronchoalveolar larvage. Ten (55.6%) patients showed findings of other interstitial lung disease (ILD) in both lower lobes on chest CT scan images, and on lung biopsy, UIP pattern was found in 16.7% and OP pattern in 22.2%, additionally. Fourteen (77.8%) patients were treated with steroid±immunosuppresants, pneumothorax was developed in 38.9% and 27.8% died during follow-up (1-year survival rate: 81.5%, 5-year: 36.2%). Respiratory infection (40%) was the most common cause of death. Conclusion: Half of the subjects had a relevant exposure history and other ILD on chest CT, and prognosis was similar to non-IPF-IIPs.
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어수택 대한의사협회 2009 대한의사협회지 Vol.52 No.1
Idiopathic pulmonary fibrosis (IPF) is characterized by chronic progressive parenchymal lung fibrosis. Although extensive researches for IPF pathogenesis have been reported for several decades, the precise mechanisms are still unknown and the specific treatments for elimination of fibrosis and prolongation of survival are also still unknown. The role of inflammation as initial insult of lung fibrosis is still debating by controversial results of animal model experiments. The recent proposed mechanism for IPF is a dysregulation of epithelial-mesenchymal interactions which have critical role in tissue repair process and fibrosis. This hypothesis suggests impaired communications between epithelium and mesenchymal cells in terms of abnormal proliferation of mesenchymal cells instead of normal proliferation of epithelium. At recent, epithelial mesenchymal transition is regarded as an important source of myofiborblast which are major cells producing extracellular matrix. Classical treatment agents including steroid are already known to be ineffective in treatment of IPF, and also, IFN- one of newly emerging drug, is proved to be ineffective in treatment of IPF. Now new drugs involved in the molecular levels of signal transduction of fibrotic pathway, inhibition of various growth factors (TGF, CTGF, VEGF), and direct inhibition of fibrotic cytokines are under investigated in animal experiments and human clinical studies. Further studies should be focused on the evaluation of precipitating factors, genetic markers, drugs for inhibiting specific molecules responsible for lung fibrosis, and agents for controlling ECM precipitation.
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어수택 대한내과학회 2013 대한내과학회지 Vol.84 No.4
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal fibrotic lung disease in many patients. In spite of extensive research for many decades, the exact pathogenesis of IPF is unknown. At recent, the role of alveolar epithelial cells has been focused in the initiation of IPF in terms of epithelial-mesenchymal transition, dysregulated Wnt signaling, and activation of transforming growth factor-β (TGF-β). The resulting excess collagen deposition and destruction of lung architecture by myofibroblasts and fibroblastic foci leads to the development of IPF. IPF can be diagnosed by typical high resolution chest tomogram (HRCT) or by multidisciplinary discussion based on the new guideline published on 2010. 현재까지도 IPF의 발생 원인은 알려져 있지 않지만, 여러연구의 발달로 병인을 밝히는 데 많은 발전이 있었다. 특히후생학 연구의 발달로 IPF의 병인을 DNA부터 전사 후(post-transcription)의 변화까지 알 수 있게 되었다. 하지만 섬유화에 가장 중요한 TGF-β 활성화만으로는 IPF의 병인을 모두 설명할 수 없으며, 섬유화에 이르게 하는 여러 세포와 단백질이 상호 활성화시키면서 섬유화가 계속 진행하는 것으로 보이며, 이에 대한 연구가 진행되어야 할 것이다. 병인과 달리 진단은 많은 발전이 있었다. 특히 최근에 발표된 지침을 통하여 폐 조직 검사 없이도 진단할 수 있는 근거를 마련하였으며, IPF의 진단을 위하여 관계하는 과간의상의를 통하여 진단할 수 있게 되었다.
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