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《Archivos de bronconeumología》2022,58(2):135-141
IntroductionIdiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials.MethodsSpanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed.Results225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6 min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern.ConclusionsPatients with preserved FVC but presenting UIP radiological pattern and moderate–severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF. 相似文献
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Defects of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface liquid, influencing the mucus composition and viscosity, which induces a severe condition of infection and inflammation along the whole life of CF patients. The introduction of CFTR modulators, novel drugs directly intervening to rescue the function of CFTR protein, opens a new era of experimental research. The review summarizes the most recent advancements to understand the characteristics of the infective and inflammatory pathology of CF lungs. 相似文献
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目的评估肝脏瞬时弹性成像、天冬氨酸转氨酶与血小板比值指数(APRI)及基于4因子的肝纤维化指数(FIB-4)对儿童非酒精性脂肪性肝病(NAFLD)肝纤维化的诊断价值。方法选取湖南省儿童医院2015年8月至2020年10月已行肝穿刺病理活检的非酒精性脂肪性肝病100例进行回顾性研究,收集肝脏病理组织和临床资料。采用受试者操作特征曲线(ROC曲线)分析肝脏硬度(LSM)值、APRI及FIB-4诊断儿童NAFLD所致不同肝脏纤维化的诊断价值。结果LSM值、APRI、FIB-4诊断肝纤维化(S≥1)的ROC曲线下面积(AUC)分别为0.701[95%可信区间(CI):0.579~0.822,P=0.011]、0.606(95%CI:0.436~0.775,P=0.182)、0.568(95%CI:0.397~0.740,P=0.387),最佳临界值分别为6.65 kPa、21.20、0.18;LSM值、APRI、FIB-4诊断显著肝纤维化(S≥2)的AUC分别为0.660(95%CI:0.552~0.768,P=0.006)、0.578(95%CI:0.464~0.691,P=0.182)、0.541(95%CI:0.427~0.655,P=0.482),最佳临界值分别为7.35 kPa、24.78、0.22;LSM值、APRI、FIB-4诊断进展期肝纤维化(S≥3)的AUC分别为0.639(95%CI:0.446~0.832,P=0.134)、0.613(95%CI:0.447~0.779,P=0.223)、0.587(95%CI:0.411~0.764,P=0.346),最佳临界值分别为8.55 kPa、26.66、0.27。结论瞬时弹性成像技术对儿童NAFLD肝纤维化有较好的诊断价值,优于APRI和FIB-4。 相似文献
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Wei Liu Xue Liu Mei Peng Gong-Quan Chen Peng-Hua Liu Xin-Wu Cui Fan Jiang Christoph F Dietrich 《World journal of gastroenterology : WJG》2021,27(34):5715-5726
Recently, increasing attention has been paid to the application of artificial intelligence (AI) to the diagnosis of diverse hepatic diseases, which comprises traditional machine learning and deep learning. Recent studies have shown the possible value of AI based data mining in predicting the incidence of hepatitis, classifying the different stages of hepatitis, diagnosing or screening for hepatitis, forecasting the progression of hepatitis, and predicting response to antiviral drugs in chronic hepatitis C patients. More importantly, AI based on radiology has been proven to be useful in predicting hepatitis and liver fibrosis as well as grading hepatocellular carcinoma (HCC) and differentiating it from benign liver tumors. It can predict the risk of vascular invasion of HCC, the risk of hepatic encephalopathy secondary to hepatitis B related cirrhosis, and the risk of liver failure after hepatectomy in HCC patients. In this review, we summarize the application of AI in hepatitis, and identify the challenges and future perspectives. 相似文献
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<正>纤维化是一种组织受到破坏后的修复反应,主要由于炎症因子过度刺激引发实质细胞坏死、细胞外基质(extracellular matrix,ECM)分泌过剩,大量沉积于细胞间质,形成胶原蛋白排列紊乱的病理表现,严重者导致器官结构紊乱和功能障碍,甚至发生器官衰竭[1]。据报道,纤维化是一种不可逆的病理过程[2],目前临床上对纤维化疾病以干预和对症治疗为主[3],尚无特定的治疗药物。肿瘤坏死因子α(tumor necrosis factor-α,TNF-α)和白细胞介素1β(interleukin-1β,IL-1β)[4-5]已被确定为多种纤维化疾病治疗的关键靶点。那么,阻断炎症因子的信号途径是否可控制纤维化的发生、发展?G蛋白偶联受体30(G-protein-coupled receptor 30,GPR30)是一种7次跨膜雌激素受体蛋白, 相似文献
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