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Background: Idiopathic pulmonary fibrosis (IPF) is a heterogeneous and progressive fibrosing interstitial lung disease with a poor prognosis. However, there are currently no effective biomarker that can reliably predict the prognosis for IPF in clinic. The serum level of soluble suppression of tumorigenicity-2 (sST2), which is involved in the immune response, has proven to be a prognostic predictor for various diseases. Previous studies have confirmed that the immune dysfunction plays an important role in the pathogenesis of IPF and the serum sST2 concentrations in patients with IPF are elevated. However, the relationship between sST2 and the prognosis of IPF remains unknown. Methods: A total of 83 patients with IPF and 20 healthy controls from 2016 to 2021 were enrolled and demographic variables, indices of lung function testing as well as the biomarkers including the sST2 were obtained at baseline. During follow-up, the primary endpoint was defined as all-cause death and clinical deterioration. Cox hazard models and Kaplan-Meier method were used to assess the prognostic value of various indices including sST2. Results: Mean duration of follow-up was 29 months, during which 49 patients had an event, and of them, 35 patients died. The sST2 level was higher in the IPF patients compared with the healthy controls. Although the sST2 level did not directly predict all-cause death in the present study, it was proved to be an independent predictor of event-free survival. Multivariate forward stepwise model which was adjusted by age, sex, and body surface area (BSA) showed that the overexpression of sST2 increased the hazard ratio [1.005, 95% confidence interval (CI): 1.001-1.010]. A higher sST2 serum level heralded more deterioration and the poor outcomes. Moreover, the effect of sST2 on the prognosis of IPF may not necessarily involve the development of IPF-related pulmonary hypertension (PH). Conclusions: In our study, the sST2 serum level was significantly elevated and a higher serum level of sST2 predicted more deterioration and poor outcomes in patients with IPF. Thus, sST2 can serve as a valuable prognostic biomarker for the outcome of IPF. However, further multicenter clinical trials of larger sample size are needed in the future.
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OBJECTIVE: to improve understanding of the novel influenza A (H1N1) virus infection complicated with pneumonia. METHOD: clinical presentations, radiological data, laboratory data and treatments of 2 patients with pneumonia caused by the novel influenza A (H1N1) virus were retrospectively analyzed. RESULTS: one patient was a middle-aged woman, and the other was a young man with a history of asthma. Both patients had fever (temperatures more than 39 degrees C), cough, and dyspnea. At the time of admission, both patients had hypoxemia. Chest computed tomography (CT) scan showed patchy distribution of consolidation and ground-glass opacity mainly in peripheral subpleural regions, as well as peribrochovascular lesions with air-bronchogram. The decreased and lymphocytes, elevated lactate dehydrogenase and creatine kinase were found in both patients. After admission, the patients received oseltamivir with a dose of 75 mg twice a day for a minimum of 5 days. Other treatments included antibiotics and methylprednisolone. One patient needed intermittent non-invasive ventilation for respiratory distress. Both patients recovered and the opacities on their chest CT were almost absorbed. WBC counts increased to normal and the levels of LDH and CK decreased. CONCLUSIONS: patients with the novel influenza A (H1N1) virus infection may develop pneumonia. The manifestations are non-specific. Chest CT scan reveals patchy distribution of consolidation and ground-glass opacity. In addition to antiviral therapy, glucocorticoids may have some effects on the pulmonary lesions and be useful for improving the clinical conditions.
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Hipóxia/virologia , Influenza Humana/complicações , Pneumonia/virologia , Adulto , Feminino , Humanos , Hipóxia/complicações , Hipóxia/diagnóstico por imagem , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Pneumonia/complicações , Pneumonia/diagnóstico por imagem , Radiografia , Estudos RetrospectivosRESUMO
Chronic thromboembolic pulmonary hypertension (CTEPH) is similar to pulmonary arterial hypertension (PAH) in its pathogenesis. Changed hemodynamic parameters in acute vasoreactivity testing (AVT) have proved to be prognostic predictors of PAH. We wanted to determine whether these changed indices also impacted the prognosis of CTEPH. Data was retrieved for 86 CTEPH patients who underwent right heart catheterization (RHC) with AVT at Shanghai Pulmonary Hospital from 2009 to 2018 and following up for 20 ± 15 months for event. Cox proportional hazards models were performed to determine the predictors of independent event-free survival. Receiver operating characteristic curve was plotted to determine the cut-off value of independent parameters in CTEPH. Kaplan-Meier method and log-rank test were used to perform the Survival analyses. Forty seven patients had an event. Many hemodynamic indices improved after AVT. The event-free group had better mean right atrial pressure, mean pulmonary arterial pressure, pulmonary vascular resistance (PVR) and oxygen saturation of mixed venous blood (SvO2) both at baseline and after AVT. The event-free group also showed higher cardiac output (CO) and cardiac index (CI) after AVT. Among the changed hemodynamic parameters during the AVT, ΔCO, ΔCO/baseline CO, ΔCI, ΔCI/baseline CI and ΔPVR/baseline PVR were significantly higher in the event-free group. Foremost, ΔPVR/baseline PVR, PVR after AVT and baseline SvO2 were independent predictors for event-free survival. Patients with SvO2 ≥ 61.65% at baseline or PVR < 8.09 WU after AVT or ΔPVR/baseline PVR ≥ 0.054 had significantly better survival. Hemodynamic indices both at baseline and after AVT as well as the changes in these indices reflected the severity of CTEPH. Baseline SvO2, PVR after AVT, and ΔPVR/baseline PVR could be used as independent predictors to estimate the outcomes of CTEPH patients.
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OBJECTIVE: To highlight the clinical, radiological and pathological characteristics of Objective To highlight the clinical, radiological and pathological characteristics of giant cell interstitial pneumonia (GIP) associated with Hard metals. METHOD: The clinical, radiological and pathological data of a patient with hard metal lung disease confirmed by video-assisted thoracoscopic biopsy of the right lung were presented, and relevant literatures were reviewed. RESULTS: A 30-year-old female patient presented with cough and dyspnea on exertion for more than 40 days. She had worked for grinding tungsten rod, with exposure to metal dusts containing cobalt and tungsten for more than 3 years. Chest radiographs and CT demonstrated bilateral ground-glass attenuations and ill-defined small nodules. Lung function studies showed the mixed type of ventilation dysfunction with a low diffusion capacity (DLCO of 39% of predicted). Lung biopsy specimens showed desquamative interstitial pneumonia like reaction with alveolar macrophages and numerous large multinucleated histiocytes that ingested inflammatory cells in alveolar spaces, which was characteristics of GIP. The lung parenchyma also showed patchy chronic inflammatory-cell infiltrates centered predominantly around the bronchioles and interstitial fibrosis. Considering the exposure history and the histological findings of the lung, the diagnosis of hard metal lung disease and GIP associated with hard metals was made. Her cough, dyspnea, and radiological changes showed marked improvement after corticosteroid therapy and avoidance of further exposure to hard metals. CONCLUSION: GIP is almost pathognomonic for hard metal lung disease. Meticulous history taking on occupational exposure is important for the diagnosis and differential diagnosis of suspected interstitial lung disease.
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Ligas/efeitos adversos , Cobalto/efeitos adversos , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/patologia , Exposição Ocupacional , Tungstênio/efeitos adversos , Adulto , Poeira , Feminino , Células Gigantes/patologia , HumanosRESUMO
OBJECTIVES: The purpose of this study was to determine the diagnostic and prognostic values of serum KL-6 levels in Chinese patients with interstitial lung disease (ILDs). METHODS: A total of 1084 subjects including 373 cases of ILDs, 584 cases of non-ILD pulmonary diseases, and 127 healthy individuals were recruited from three clinical centers in China between January 2011 and December 2013. A total of 106 patients undergoing treatments for ILDs in Shanghai Pulmonary Hospital between January 2011 and December 2013 were enrolled. Baseline and posttreatment serum KL-6 levels were determined. RESULTS: Serum KL-6 levels in patients with ILDs were significantly higher than those in patients with non-ILD pulmonary diseases or in healthy individuals (1492.09 ± 2230.08 U/mL vs 258.67 ± 268.73 U/mL or 178.73 ± 71.17 U/mL, all P < 0.05). At the cut-off value of 500 U/mL, the sensitivity and specificity of serum KL-6 as a diagnostic marker for ILDs was 77.75% and 94.51%, respectively. The Kappa value was 0.743 (P < 0.001). The area below the receiver operating characteristic curve was 0.922 with a 95% Confidence interval of 0.904-0.941 (P < 0.001). The posttreatment serum KL-6 levels significantly reduced in patients with improved ILDs, whereas markedly increased in patients with exacerbated ILDs (All P < 0.05). CONCLUSIONS: Serum KL-6 levels might be a promising diagnostic biomarker for ILDs in Chinese patients. The prognostic value of serum KL-6 levels for ILDs remains to be verified by large-scaled studies.
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Povo Asiático/genética , Biomarcadores/sangue , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/diagnóstico , Mucina-1/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , China/epidemiologia , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Doenças Pulmonares Intersticiais/metabolismo , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória/métodos , Capacidade Vital/fisiologiaRESUMO
BACKGROUND: Acute fibrinous and organizing pneumonia (AFOP) is a unique pathological entity with intra-alveolar fibrin in the form of "fibrin balls" and organizing pneumonia. It was divided into rare idiopathic interstitial pneumonia according to the classification notified by American Thoracic Society/European Respiratory Society in 2013. As a rare pathological entity, it is still not well known and recognized by clinicians. We reviewed the clinical features of 20 patients with AFOP diagnosed in a teaching hospital. METHODS: The medical records of 20 patients with biopsy-proven diagnosis of AFOP were retrospectively reviewed. The patients' symptoms, duration of the disease, comorbidities, clinical laboratory data, pulmonary function testing, radiographic studies, and the response to treatment were extracted and analyzed. RESULTS: Fever was the most common symptom and was manifested in 90% of AFOP patients. For clinical laboratory findings, systematic inflammatory indicators, including C-reactive protein and erythrocyte sedimentation rate, were significantly higher than normal in AFOP patients. In accordance with this increased indicators, injured liver functions were common in AFOP patients. Inversely, AFOP patients had worse clinical conditions including anemia and hypoalbuminemia. For pulmonary function testing, AFOP patients showed the pattern of restrictive mixed with obstructive ventilation dysfunction. For high-resolution computerized tomography (HRCT) findings, the most common pattern for AFOP patients was lobar consolidation which was very similar to pneumonia. However, unlike pneumonia, AFOP patients responded well to glucocorticoids. CONCLUSION: Patients with AFOP manifest as acute inflammatory-like clinical laboratory parameters and lobar consolidation on HRCT, but respond well to steroid.