• 제목/요약/키워드: pulmonary fibrosis

검색결과 295건 처리시간 0.019초

피부병변없이 간질성 폐섬유화로 표현된 경피증 1례 (A Case of Systemic Sclerosis Sine Scleroderma Presenting as Pulmonary Interstitial Fibrosis)

  • 곽진호;최원일;이승현;서창균;김경찬;김민수;권건영;서수지;박창권;전영준
    • Tuberculosis and Respiratory Diseases
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    • 제50권4호
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    • pp.493-498
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    • 2001
  • 저자들은 4개월간의 호흡곤란을 주소로 내원하여 피부병변은 없었지만 흉강경하 폐생검과 식도내압검사, 핵항체검사 등으로 경피증을 확진한 Systemic sclerosis sine scleroderma 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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Searching for Novel Candidate Small Molecules for Ameliorating Idiopathic Pulmonary Fibrosis: a Narrative Review

  • Kyung-il Kim;Rajib Hossain;Xin Li;Hyun Jae Lee;Choong Jae Lee
    • Biomolecules & Therapeutics
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    • 제31권5호
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    • pp.484-495
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    • 2023
  • Idiopathic pulmonary fibrosis (IPF) can be defined as a progressive chronic pulmonary disease showing scarring in the lung parenchyma, thereby resulting in increase in mortality and decrease in the quality of life. The pathophysiologic mechanism of fibrosis in IPF is still unclear. Repetitive microinjuries to alveolar epithelium with genetical predisposition and an abnormal restorative reaction accompanied by excessive deposition of collagens are involved in the pathogenesis. Although the two FDA-approved drugs, pirfenidone and nintedanib, are under use for retarding the decline in lung function of patients suffered from IPF, they are not able to improve the survival rate or quality of life. Therefore, a novel therapeutic agent acting on the major steps of the pathogenesis of disease and/or, at least, managing the clinical symptoms of IPF should be developed for the effective regulation of this incurable disease. In the present review, we tried to find a potential of managing the clinical symptoms of IPF by natural products derived from medicinal plants used for controlling the pulmonary inflammatory diseases in traditional Asian medicine. A multitude of natural products have been reported to exert an antifibrotic effect in vitro and in vivo through acting on the epithelial-mesenchymal transition pathway, transforming growth factor (TGF)- β-induced intracellular signaling, and the deposition of extracellular matrix. However, clinical antifibrotic efficacy of these natural products on IPF have not been elucidated yet. Thus, those effects should be proven by further examinations including the randomized clinical trials, in order to develop the ideal and optimal candidate for the therapeutics of IPF.

후코이단에 의한 인간 폐 섬유모세포의 활성 억제 효과 (Inhibitory Effect of Fucoidan on TGF-β1-Induced Activation of Human Pulmonary Fibroblasts)

  • 임미진;이대성;최그레이스;이정민;최일환
    • 한국수산과학회지
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    • 제49권6호
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    • pp.807-814
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    • 2016
  • Fucoidan, one of the dominant sulfated polysaccharides extracted from brown seaweed, possesses a wide range of biological activities. Transforming growth $factor-{\beta}$ ($TGF-{\beta}$) plays a pivotal role in the pathogenesis of pulmonary fibrosis, by stimulating the synthesis of profibrotic factors. In this study, we investigated the in vitro effects of fucoidan on collagen synthesis, ${\alpha}-smooth$ muscle actin (${\alpha}-SMA$) expression, and interleukin (IL)-6 production in $TGF-{\beta}$-stimulated human pulmonary fibroblasts. The expression of type I collagen and ${\alpha}-SMA$ was detected by Western blot, and the production of IL-6 by enzyme-linked immunosorbent assay. $TGF-{\beta}1$ treatment of pulmonary fibroblasts enhanced the expression of ${\alpha}-SMA$, type I collagen, and IL-6 whereas these effects were inhibited in cells pretreated with fucoidan. The activation of Smad2/3, p38 mitogen-activated protein kinases (MAPKs), and Akt was also inhibited in fucoidan-pretreated, $TGF-{\beta}1-stimulated$ human pulmonary fibroblasts. These data demonstrate the anti-fibrotic potential of fucoidan in $TGF-{\beta}-induced$ human pulmonary fibroblasts, via the inhibition of Smad2/3, p38 MAPKs, and Akt phosphorylation. Our results suggest the therapeutic potential of fucoidan in the prevention or treatment of pulmonary fibrosis.

Diagnostic Approaches for Idiopathic Pulmonary Fibrosis

  • Jae Ha Lee;Jin Woo Song
    • Tuberculosis and Respiratory Diseases
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    • 제87권1호
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    • pp.40-51
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    • 2024
  • Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia with a very poor prognosis. Accurate diagnosis of IPF is essential for good outcomes but remains a major medical challenge due to variability in clinical presentation and the shortcomings of existing diagnostic tests. Medical history collection is the first and most important step in the IPF diagnosis process; the clinical probability of IPF is high if the suspected patient is 60 years or older, male, and has a history of cigarette smoking. Systemic assessment for connective tissue disease is essential in the initial evaluation of patients with suspected IPF to identify potential causes of interstitial lung disease (ILD). Radiologic examination using high-resolution computed tomography plays a pivotal role in the evaluation of patients with ILD, and prone and expiratory computed tomography images can be considered. If additional tests such as surgical lung biopsy or transbronchial lung cryobiopsy are needed, transbronchial lung cryobiopsy should be considered as an alternative to surgical lung biopsy in medical centers with experience performing this procedure. Diagnosis through multidisciplinary discussion (MDD) is strongly recommended as MDD has become the cornerstone for diagnosis of IPF, and the scope of MDD has expanded to monitoring of disease progression and suggestion of appropriate treatment options.

폐 유육종증 1례 보고 (Pulmonary Sarcoidosis)

  • 백효채
    • Journal of Chest Surgery
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    • 제28권10호
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    • pp.928-931
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    • 1995
  • Sarcoidosis is a granulomatous disease of unknown etiology. It frequently involves thoracic lymph node and pulmonary parenchyme characterized by non-caseating granulomas and varying degrees of fibrosis. We have experienced a pulmonary sarcoidosis in the left lower lobe of the lung in a 40 year old man who underwent left lower lobectomy by video-assisted thoracic surgery.

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CHANGES IN SUBPOPULATION OF BRONCHOALVEOLAR LAVAGE FLUID IN THE PULMONARY FIBROSIS INDUCED BY BLEOMYCIN OR PEPLOMYCIN

  • Kim, Dae-Joong
    • Toxicological Research
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    • 제9권2호
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    • pp.241-251
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    • 1993
  • Present studies were carried out in order to estabilish the bronchoalveolar lavage method and to examine the response of bleomycin and peplomycin on the total cell number and the subpoulations of bronchoalveolar lavage fluid. A total of 24 male F344 rats, weighing 300-350 mg, were divided into 3 groups. Animals recelved either belomycin (BLM` 0.75 mg/0.2 ml/rat), peplomycin (PLM` 0.25mg/0.2ml/rat) for groups 2 and 3 or an equal volume of sterile saline lacking drugs for controls (group 1).

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미만성 간질성 폐섬유증의 경과 관찰에서의 고해상도 CT(HRCT)의 의의 (The Role of HRCT in the Follow-Up Evaluation of Diffuse Interstitial Pulmonary Fibrosis)

  • 김홍규;곽승민;송군식;임채만;고윤석;김우성;김동순;김원동
    • Tuberculosis and Respiratory Diseases
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    • 제41권6호
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    • pp.597-603
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    • 1994
  • 연구배경 : 최근 HRCT의 개발로 폐실질의 미세한 변화까지 관찰할 수 있게 되었고, 특히 비침습적 방법으로 용이하게 여러번 반복하여 실시할 수 있는 장점이 있기 때문에, HRCT가 다른 검사보다 병의 경과, 또는 스테로이드 등 치료의 효과를 객관적으로 판단하는데 도움을 줄 수 있을 것으로 추정하여 본 연구를 실시하였다. 방법: 폐섬유증으로 진단받고 두번 이상 HRCT를 시행하였던 환자 13명에 대하여 치료 전후의 임상 증상(호흡 곤란 지수)의 변화, 폐기능 검사 소견의 변화와 영상 분석기로 측정한 HRCT상의 병변 범위의 변화와의 관계를 분석하였다. 결과: 1) 영상 분석기를 이용한 HRCT에서의 병변 범위 변화와 호흡 곤란 지수의 변화는 r=0.716, p=0.0012로 매우 유의한 상관관계를 나타내었다. 2) 호흡 곤란 지수의 변화와 확산능 변화 사이에는 r=-0.707, p=0.0047로 유의한 역상관관계가 있는 것으로 나타났다. 3) HRCT의 변화와 폐확산능의 변화는 유의한 상관관계를 보였으나 FVC나 TLC는 뚜렷한 연관성을 찾을 수 없었다. 결론: HRCT의 변화는 임상 증상의 변화 및 폐확산능의 변화와 서로 유의한 상관 관계를 보이는 것을 알았고, 향후 환자의 경과 관찰에 임상 증상, 폐확산능 및 HRCT의 추적 검사가 도움이 될 것으로 사료되었다.

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Pulmonary Fibrosis caused by Asbestos Fibers in the Respiratory Airway

  • Jung, Ji-Woo;Kim, Eung-Sam
    • 대한의생명과학회지
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    • 제27권3호
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    • pp.111-120
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    • 2021
  • Asbestos products had been widely used until 2007 in Korea since the 1930s. A total ban on their production and applications has been imposed because of the toxic effect of asbestos fibers on the human health. The inhaled asbestos fibers increase reactive oxygen species and inflammatory reactions in the respiratory airway including the alveolar sac, resulting in DNA damages and secretion of several inflammatory cytokines or chemokines. These paracrine communications promote the proliferation of fibroblasts and the synthesis of collagen fibers, thereby depositing them into the extracellular matrix at the interstitial space of alveoli. The fibrotic tissue hindered the gas exchange in the alveolus. This reviews describes not only the cytotoxic effects of asbestos fibers with different physical or chemical characteristics but also the interaction of cells that make up the respiratory airway to understand the molecular or cellular mechanisms of asbestos fiber-induced toxicity. In addition, we propose a pulmonary toxicity research technique based on the mini-lung that can mimic human respiratory system as an alternative to overcome the limitations of the conventional risk assessment of asbestos fibers.

Adverse Effects of Air Pollution on Pulmonary Diseases

  • Ko, Ui Won;Kyung, Sun Young
    • Tuberculosis and Respiratory Diseases
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    • 제85권4호
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    • pp.313-319
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    • 2022
  • Environmental exposure to air pollution is known to have adverse effects on various organs. Air pollution has greater effects on the pulmonary system as the lungs are directly exposed to contaminants in the air. Here, we review the associations of air pollution with the development, morbidity, and mortality of pulmonary diseases. Short-and long-term exposure to air pollution have been shown to increase mortality risk even at concentrations below the current national guidelines. Ambient air pollution has been shown to be associated with lung cancer. Particularly long-term exposure to particulate matter with a diameter <2.5 ㎛ (PM2.5) has been reported to be associated with lung cancer even at low concentrations. In addition, exposure to air pollution has been shown to increase the incidence risk of chronic obstructive pulmonary disease (COPD) and has been correlated with exacerbation and mortality of COPD. Air pollution has also been linked to exacerbation, mortality, and development of asthma. Exposure to nitrogen dioxide (NO2) has been demonstrated to be related to increased mortality in patients with idiopathic pulmonary fibrosis. Additionally, air pollution increases the incidence of infectious diseases, such as pneumonia, bronchitis, and tuberculosis. Furthermore, emerging evidence supports a link between air pollution and coronavirus disease 2019 transmission, susceptibility, severity and mortality. In conclusion, the stringency of air quality guidelines should be increased and further therapeutic trials are required in patients at high risk of adverse health effects of air pollution.