130 results on '"Kairalla, Ronaldo Adib"'
Search Results
102. Respiratory amyloidosis: a case series from a Brazilian referral center.
- Author
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Colares PFB, Santana ANC, Athanazio RA, Kairalla RA, and Baldi BG
- Subjects
- Humans, Male, Brazil, Female, Middle Aged, Aged, Adult, Amyloidosis pathology
- Published
- 2024
- Full Text
- View/download PDF
103. Rheumatoid arthritis-associated airway disease: longitudinal pulmonary function behavior.
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Sabbag MLB, Molina CA, Sawamura MVY, Bonfiglioli K, Medeiros-Ribeiro AC, Pugliesi A, Nakagawa RH, Arimura FE, Athanazio RA, Kairalla RA, Baldi BG, and Kawano-Dourado L
- Subjects
- Humans, Lung, Arthritis, Rheumatoid complications, Respiration Disorders
- Published
- 2024
- Full Text
- View/download PDF
104. Nephrobronchial fistula: a diagnostic challenge in a patient with IgG4-related disease.
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Plens GCM, Bridi GDP, Nascimento ECTD, Chate RC, Baldi BG, Arimura FE, and Kairalla RA
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- Humans, Immunoglobulin G4-Related Disease complications, Immunoglobulin G4-Related Disease diagnosis
- Published
- 2024
- Full Text
- View/download PDF
105. Idiopathic pulmonary fibrosis: current diagnosis and treatment.
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Amaral AF, Colares PFB, and Kairalla RA
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- Humans, Idiopathic Pulmonary Fibrosis diagnosis, Idiopathic Pulmonary Fibrosis drug therapy, Lung Diseases, Interstitial diagnosis, Lung Diseases, Interstitial drug therapy
- Abstract
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.
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- 2023
- Full Text
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106. Tomographic pleuropulmonary manifestations in rheumatoid arthritis: a pictorial essay.
- Author
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Bridi GDP, Sawamura MVY, Wanderley M, Souza LVS, Kairalla RA, Kawano-Dourado L, and Baldi BG
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- Humans, Male, Risk Factors, Lung, Autoantibodies, Arthritis, Rheumatoid complications, Arthritis, Rheumatoid diagnostic imaging, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial etiology
- Abstract
Rheumatoid arthritis (RA) is an autoimmune inflammatory and heterogeneous disease that affects several systems, especially the joints. Among the extra-articular manifestations of RA, pleuropulmonary involvement occurs frequently, with different presentations, potentially in all anatomic thoracic compartments, and may determine high morbidity and mortality. The most common pleuropulmonary manifestations in patients with RA include interstitial lung disease (ILD), pleural disease, pulmonary arterial hypertension, rheumatoid lung nodules, airway disease (bronchiectasis and bronchiolitis), and lymphadenopathy. Pulmonary hypertension and ILD are the manifestations with the greatest negative impact in prognosis. HRCT of the chest is essential in the evaluation of patients with RA with respiratory symptoms, especially those with higher risk factors for ILD, such as male gender, smoking, older age, high levels of rheumatoid factor, or positive anti-cyclic citrullinated peptide antibody results. Additionally, other etiologies that may determine tomographic pleuropulmonary manifestations in patients with RA are infections, neoplasms, and drug-induced lung disease. In these scenarios, clinical presentation is heterogeneous, varying from being asymptomatic to having progressive respiratory failure. Knowledge on the potential etiologies causing tomographic pleuropulmonary manifestations in patients with RA coupled with proper clinical reasoning is crucial to diagnose and treat these patients.
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- 2023
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107. COVID-19 and pulmonary alveolar proteinosis: an unusual combination.
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Colares PFB, Silva NFD, Kairalla RA, and Baldi BG
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- Humans, Pulmonary Alveolar Proteinosis diagnostic imaging, COVID-19
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- 2023
- Full Text
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108. PET/CT and interstitial lung disease.
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Sawamura MVY, Kairalla RA, and Buchpigel CA
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- Fluorodeoxyglucose F18, Humans, Lung diagnostic imaging, Lung Diseases, Interstitial diagnostic imaging, Positron Emission Tomography Computed Tomography
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- 2022
- Full Text
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109. Brazilian guidelines for the pharmacological treatment of idiopathic pulmonary fibrosis. Official document of the Brazilian Thoracic Association based on the GRADE methodology.
- Author
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Baddini-Martinez J, Ferreira J, Tanni S, Alves LR, Cabral Junior BF, Carvalho CRR, Cezare TJ, Costa CHD, Gazzana MB, Jezler S, Kairalla RA, Kawano-Dourado L, Lima MS, Mancuzo E, Moreira MAC, Rodrigues MP, Rodrigues SCS, Rubin AS, Rufino RL, Steidle LJM, Storrer K, and Baldi BG
- Subjects
- Acetylcysteine therapeutic use, Aged, Anti-Inflammatory Agents therapeutic use, Brazil, Humans, Indoles therapeutic use, Male, Pyridones therapeutic use, Idiopathic Pulmonary Fibrosis drug therapy, Practice Guidelines as Topic
- Abstract
Idiopathic pulmonary fibrosis (IPF) is a form of chronic interstitial lung disease of unknown cause, which predominantly affects elderly men who are current or former smokers. Even though it is an uncommon disease, it is of great importance because of its severity and poor prognosis. In recent decades, several pharmacological treatment modalities have been investigated for the treatment of this disease, and the classic concepts have therefore been revised. The purpose of these guidelines was to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of IPF in Brazil. We sought to provide guidance on the practical issues faced by clinicians in their daily lives. Patients of interest, Intervention to be studied, Comparison of intervention and Outcome of interest (PICO)-style questions were formulated to address aspects related to the use of corticosteroids, N-acetylcysteine, gastroesophageal reflux medications, endothelin-receptor antagonists, phosphodiesterase-5 inhibitors, pirfenidone, and nintedanib. To formulate the PICO questions, a group of Brazilian specialists working in the area was assembled and an extensive review of the literature on the subject was carried out. Previously published systematic reviews with meta-analyses were analyzed for the strength of the compiled evidence, and, on that basis, recommendations were developed by employing the Grading of Recommendations Assessment, Development and Evaluation approach. The authors believe that the present document represents an important advance to be incorporated in the approach to patients with IPF, aiming mainly to improve its management, and can become an auxiliary tool for defining public policies related to IPF.
- Published
- 2020
- Full Text
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110. Hard metal lung disease: a case series.
- Author
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Mizutani RF, Terra-Filho M, Lima E, Freitas CS, Chate RC, Kairalla RA, Carvalho-Oliveira R, and Santos UP
- Subjects
- Adrenal Cortex Hormones therapeutic use, Adult, Biopsy, Humans, Lung diagnostic imaging, Lung pathology, Lung Diseases, Interstitial drug therapy, Lung Diseases, Interstitial pathology, Male, Middle Aged, Occupational Diseases drug therapy, Occupational Diseases pathology, Retrospective Studies, Tomography, X-Ray Computed, Alloys adverse effects, Alloys toxicity, Cobalt adverse effects, Cobalt toxicity, Lung Diseases, Interstitial diagnosis, Occupational Diseases diagnosis, Occupational Exposure adverse effects, Tungsten adverse effects, Tungsten toxicity
- Abstract
Objective:: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic., Methods:: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013., Results:: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3., Conclusions:: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis., Objetivo:: Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura., Métodos:: Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de São Paulo, entre 2010 e 2013., Resultados:: Entre 320 pacientes atendidos no período do estudo, 5 (1,56%) foram diagnosticados com DPMD. Todos os pacientes eram do sexo masculino, com média de idade de 42,0 ± 13,6 anos e média de tempo de exposição a metal duro de 11,4 ± 8,0 anos. Os pacientes foram submetidos a avaliação clinica, história ocupacional, TCAR de tórax, prova de função pulmonar, broncoscopia com LBA e biópsia pulmonar. Todos apresentaram distúrbio ventilatório restritivo. O achado de imagem à TCAR de tórax mais frequente foi de opacidades em vidro fosco (em 80%). Em 4 pacientes, o LBA revelou presença de células gigantes multinucleadas. Em 3, foi diagnosticada pneumonia intersticial por células gigantes na biópsia pulmonar. Houve o diagnóstico de pneumonia intersticial descamativa associada à bronquiolite celular em 1 paciente e de pneumonite de hipersensibilidade em 1. Todos foram afastados da exposição e tratados com corticoide. Houve melhora em 2 pacientes e progressão da doença em 3., Conclusões:: Apesar de ser uma entidade rara, a DPMD deve ser sempre considerada em trabalhadores com risco ocupacional elevado de exposição a metais duros. A história clínica e ocupacional associada a achados em TCAR de tórax e LBA sugestivos da doença podem ser suficientes para o diagnóstico.
- Published
- 2016
- Full Text
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111. Epipericardial Fat Necrosis: Who Should Be a Candidate?
- Author
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Giassi KS, Costa AN, Bachion GH, Kairalla RA, and Filho JRP
- Abstract
Objective: The objectives of the present study were to estimate the frequency of epipericardial fat necrosis among patients undergoing chest CT for chest pain and to compare the clinical and laboratory data between patients with epipericardial fat necrosis and control subjects., Materials and Methods: A retrospective review of 7463 chest CT studies performed in the emergency department from July 2011 to December 2014 was conducted to collect data regarding patient demographic characteristics and CT findings. Twenty patients who had epipericardial fat necrosis diagnosed were defined as the main patient group. An age- and sex-matched control group was selected from patients with chest pain who underwent chest CT during the same period. Linear regression models were used to assess data, and statistical significance was expressed as p values and 95% CIs., Results: We found that the frequency of epipericardial fat necrosis was 2.15% among patients who underwent chest CT for chest pain. Epipericardial fat necrosis was statistically significantly associated with a lack of medication use (p = 0.01; 95% CI, -3.33 to -0.40) and the absence of other symptoms (p = 0.005; 95% CI, -5.83 to -1.27). Epipericardial fat necrosis was most often observed as an ovoid lesion with mixed fat attenuation with little stranding in the left paracardiac region accompanied by pleural effusion., Conclusion: Epipericardial fat necrosis is a significant clinical condition. For patients seen in the emergency department with isolated acute chest pain but no additional clinical history, no medication history, and normal laboratory results, chest CT is recommended to support a diagnosis of epipericardial fat necrosis.
- Published
- 2016
- Full Text
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112. Clinical course and characterisation of lymphangioleiomyomatosis in a Brazilian reference centre.
- Author
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Baldi BG, Freitas CS, Araujo MS, Dias OM, Pereira DA, Pimenta SP, Kairalla RA, and Carvalho CR
- Subjects
- Adult, Brazil, Disease Progression, Doxycycline therapeutic use, Emotions, Exercise Tolerance, Female, Forced Expiratory Volume, Hormone Antagonists therapeutic use, Humans, Lymphangioleiomyomatosis drug therapy, Lymphangioleiomyomatosis mortality, Lymphangioleiomyomatosis physiopathology, Lymphangioleiomyomatosis psychology, Male, Middle Aged, Predictive Value of Tests, Retrospective Studies, Severity of Illness Index, Survival Analysis, Time Factors, Treatment Outcome, Exercise Test, Lung physiopathology, Lymphangioleiomyomatosis diagnosis, Quality of Life, Respiratory Function Tests, Surveys and Questionnaires
- Abstract
Background and Objective: Lymphangioleiomyomatosis (LAM) is a rare disease that promotes pulmonary cystic destruction and impairs pulmonary function. We aim to describe features and clinical course of LAM patients from Brazil., Methods: We described the clinical and functional features, performance in six minute walk test (6MWT), management details, survival and clinical course of 84 LAM patients followed in a Brazilian reference centre., Results: All subjects were women, the average age at onset of symptoms was 38 years, and the average at diagnosis was 42 years. The major symptoms during the course of the disease were dyspnoea and pneumothorax. The patients experienced impaired quality of life, with worse scores in the physical and emotional domains. The most common abnormalities in pulmonary function tests were an obstructive pattern and reduced diffusion capacity, whereas a quarter of the patients had normal spirometric results. In the 6MWT, although patients had preserved exercise capacity, more than half of the patients had significant desaturation. Hormonal blockage and doxycycline were the most common treatment modalities employed in our patients. The survival probability from diagnosis was 90% at 5 years, whereas the mean annual rate of decline in FEV1 was 60 ± 78 mL., Conclusions: Clinical and functional features of the LAM patients from our centre are similar to those from other countries. Our sample showed preserved exercise capacity, with desaturation in the 6MWT, and impaired quality of life. Survival was similar, whereas the annual rate of decline of FEV1 was slightly lower than in recent studies.
- Published
- 2014
113. Guidelines for the diagnosis of rheumatoid arthritis.
- Author
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Mota LM, Cruz BA, Brenola CV, Pereira IA, Rezende-Fronza LS, Bertolo MB, Freitas MV, Silva NA, Louzada-Junior P, Giorgi RD, Lima RA, Kairalla RA, Kawassaki Ade M, Bernardo WM, and Pinheiro Gda R
- Subjects
- Arthritis, Rheumatoid classification, Arthritis, Rheumatoid diagnostic imaging, Early Diagnosis, Humans, Magnetic Resonance Imaging, Prognosis, Radiography, Ultrasonography, Arthritis, Rheumatoid diagnosis
- Published
- 2013
114. Usual interstitial pneumonia: a pattern or a disease? A reflection upon the topic.
- Author
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Kawano-Dourado L and Kairalla RA
- Subjects
- Diagnosis, Differential, Humans, Idiopathic Pulmonary Fibrosis diagnosis, Idiopathic Pulmonary Fibrosis classification
- Published
- 2013
- Full Text
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115. Doxycycline use in patients with lymphangioleiomyomatosis: biomarkers and pulmonary function response.
- Author
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Pimenta SP, Baldi BG, Kairalla RA, and Carvalho CR
- Subjects
- Adult, Biomarkers blood, Biomarkers urine, Doxycycline adverse effects, Exercise Test, Female, Humans, Lymphangioleiomyomatosis metabolism, Matrix Metalloproteinase Inhibitors adverse effects, Quality of Life, ROC Curve, Statistics, Nonparametric, Vascular Endothelial Growth Factor A metabolism, Walking, Doxycycline therapeutic use, Lymphangioleiomyomatosis drug therapy, Matrix Metalloproteinase 2 metabolism, Matrix Metalloproteinase 9 metabolism, Matrix Metalloproteinase Inhibitors therapeutic use, Vital Capacity drug effects
- Abstract
Objective: To assess blockade of matrix metalloproteinase (MMP)-2 and MMP-9, as well as the variation in FEV1, in patients with lymphangioleiomyomatosis (LAM) treated with doxycycline (a known MMP inhibitor) for 12 months., Methods: An open-label, single-arm, interventional clinical trial in which LAM patients received doxycycline (100 mg/day) for 12 months. Patients underwent full pulmonary function testing, a six-minute walk test, and quality of life assessment, as well as blood and urine sampling for quantification of MMP-2, MMP-9, and VEGF-D levels-at baseline, as well as at 6 and 12 months after the initiation of doxycycline., Results: Thirty-one LAM patients received doxycycline for 12 months. Although there was effective blockade of urinary MMP-9 and serum MMP-2 after treatment, there were no significant differences between pre- and post-doxycycline serum levels of MMP-9 and VEGF-D. On the basis of their response to doxycycline (as determined by the variation in FEV1), the patients were divided into two groups: the doxycycline-responder (doxy-R) group (n = 13); and the doxycycline-nonresponder (doxy-NR) group (n = 18). The patients with mild spirometric abnormalities responded better to doxycycline. The most common side effects were mild epigastric pain, nausea, and diarrhea., Conclusions: In patients with LAM, doxycycline treatment results in effective MMP blockade, as well as in improved lung function and quality of life in those with less severe disease. However, these benefits do not seem to be related to the MMP blockade, raising the hypothesis that there is a different mechanism of action. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-6g8yz9 [http://www.ensaiosclinicos.gov.br]).
- Published
- 2013
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116. Birt-Hogg-Dubé syndrome: metalloproteinase activity and response to doxycycline.
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Pimenta SP, Baldi BG, Nascimento EC, Mauad T, Kairalla RA, and Carvalho CR
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- Adult, Birt-Hogg-Dube Syndrome enzymology, Female, Humans, Pedigree, Birt-Hogg-Dube Syndrome drug therapy, Doxycycline therapeutic use, Matrix Metalloproteinase 9 metabolism, Matrix Metalloproteinase Inhibitors therapeutic use
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- 2012
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117. Transient lymphadenopathy secondary to nephrotic syndrome.
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Dias OM, Costa AN, Carvalho CR, and Kairalla RA
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- Humans, Male, Mediastinum, Middle Aged, Lymphatic Diseases etiology, Nephrotic Syndrome complications
- Abstract
Mediastinal lymphadenomegaly secondary to hypervolemia is an underdiagnosed tomographic finding. Herein we describe, in a patient with normal cardiac function, findings of pulmonary congestion associated to lymph node enlargement. The nephrotic syndrome causing hypoalbuminemia, low plasma colloid osmotic pressure and augmented transcapillary fluid leakage was the probable cause of the radiological findings.
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- 2012
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118. Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases.
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Baldi BG, Pereira CA, Rubin AS, Santana AN, Costa AN, Carvalho CR, Algranti E, Capitani EM, Bethlem EP, Coletta EN, Arakaki JS, Martinez JA, Carvalho JF, Steidle LJ, Rocha MJ, Lima MS, Soares MR, Caramori ML, Aidé MA, Ferreira RG, Kairalla RA, Oliveira RK, Jezler S, Rodrigues SC, and Pimenta SP
- Subjects
- Brazil, Humans, Lung Diseases, Interstitial classification, Lung Diseases, Interstitial diagnosis, Lung Diseases, Interstitial therapy, Societies, Medical
- Abstract
Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.
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- 2012
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119. Immunophenotyping and extracellular matrix remodeling in pulmonary and extrapulmonary sarcoidosis.
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Silva PH, Parra ER, Zocolaro WS, Narde I, Rodrigues F, Kairalla RA, Carvalho CR, and Capelozzi VL
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- Adult, Aged, Aged, 80 and over, Analysis of Variance, Biopsy, Collagen immunology, Elastic Tissue immunology, Elastic Tissue pathology, Extracellular Matrix pathology, Female, Granuloma, Respiratory Tract immunology, Granuloma, Respiratory Tract pathology, Humans, Lung immunology, Lung pathology, Lymph Nodes immunology, Lymph Nodes pathology, Lymphocytes immunology, Macrophages, Alveolar immunology, Male, Middle Aged, Sarcoidosis pathology, Sarcoidosis, Pulmonary immunology, Sarcoidosis, Pulmonary pathology, Extracellular Matrix immunology, Immunity, Cellular, Immunophenotyping methods, Sarcoidosis immunology
- Abstract
Objective: To investigate the significance of cellular immune markers, as well as that of collagen and elastic components of the extracellular matrix, within granulomatous structures in biopsies of patients with pulmonary or extrapulmonary sarcoidosis., Methods: We carried out qualitative and quantitative evaluations of inflammatory cells, collagen fibers, and elastic fibers in granulomatous structures in surgical biopsies of 40 patients with pulmonary and extrapulmonary sarcoidosis using histomorphometry, immunohistochemistry, picrosirius red staining, and Weigert's resorcin-fuchsin staining., Results: The extrapulmonary tissue biopsies presented significantly higher densities of lymphocytes, macrophages, and neutrophils than did the lung tissue biopsies. Pulmonary granulomas showed a significantly higher number of collagen fibers and a lower density of elastic fibers than did extrapulmonary granulomas. The amount of macrophages in the lung samples correlated with FVC (p < 0.05), whereas the amount of CD3+, CD4+, and CD8+ lymphocytes correlated with the FEV1/FVC ratio and VC. There were inverse correlations between TLC and the CD1a+ cell count (p < 0.05), as well as between DLCO and collagen/elastic fiber density (r = -0.90; p = 0.04)., Conclusions: Immunophenotyping and remodeling both showed differences between pulmonary and extrapulmonary sarcoidosis in terms of the characteristics of the biopsy samples. These differences correlated with the clinical and spirometric data obtained for the patients, suggesting that two different pathways are involved in the mechanism of antigen clearance, which was more effective in the lungs and lymph nodes.
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- 2012
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120. Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade.
- Author
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Pimenta SP, Baldi BG, Acencio MM, Kairalla RA, and Carvalho CR
- Subjects
- Adult, Angiogenesis Inhibitors pharmacology, Anti-Bacterial Agents pharmacology, Case-Control Studies, Doxycycline pharmacology, Female, Humans, Lung Neoplasms blood, Lung Neoplasms pathology, Lymphangioleiomyomatosis blood, Lymphangioleiomyomatosis pathology, Matrix Metalloproteinases blood, Matrix Metalloproteinases urine, Prospective Studies, Protease Inhibitors therapeutic use, Angiogenesis Inhibitors therapeutic use, Anti-Bacterial Agents therapeutic use, Doxycycline therapeutic use, Lung Neoplasms drug therapy, Lymphangioleiomyomatosis drug therapy, Matrix Metalloproteinase Inhibitors
- Abstract
Objective: Lymphangioleiomyomatosis (LAM) is characterized by lung cysts, whose development is associated with matrix metalloproteinase (MMP) hyperactivity, principally that of MMP-2 and MMP-9. Our objective was to compare LAM patients and controls in terms of the levels of these MMPs, as well as to determine the safety and efficacy of treatment with doxycycline, a potent MMP inhibitor., Methods: Prospective clinical study involving female LAM patients who received doxycycline (100 mg/day) for six months. Urine and blood samples were collected for the quantification of MMP-2 and MMP-9 before and after the treatment period. Samples from 10 healthy women were also collected., Results: Of the 41 LAM patients who started the treatment, 34 completed the protocol. Serum and urinary MMP-9 levels were significantly lower in the controls than in the LAM patients (p < 0.0001). Comparing pre- and post-treatment values, we found that the median level of MMP-9 in serum decreased from 919 ng/mL to 871 ng/mL (p = 0.05), whereas that of MMP-9 in urine decreased from 11,558 pg/mL to 7,315 pg/mL (p = 0.10). After treatment, the median level of MMP-2 in serum was significantly lower (p = 0.04) and urinary MMP-2 levels were undetectable. Nausea, diarrhea, and epigastric pain were the most prevalent adverse affects and were often self-limiting. There was only one case in which the patient discontinued the treatment because of side effects., Conclusions: We have demonstrated, for the first time, a decrease in serum and urine levels of MMPs in LAM patients treated with doxycycline, which proved to be a safe medication, with mild and well-tolerated side effects.
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- 2011
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121. Takotsubo cardiomyopathy triggered by β(2) adrenergic agonist.
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Salemi VM, Atik E, Kairalla RA, Queiroz EL, Rosa LV, and Kalil Filho R
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- Aged, Echocardiography, Female, Humans, Takotsubo Cardiomyopathy diagnosis, Adrenergic beta-Antagonists adverse effects, Takotsubo Cardiomyopathy chemically induced
- Published
- 2011
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122. Evolution of pulmonary function after treatment with goserelin in patients with lymphangioleiomyomatosis.
- Author
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Baldi BG, Medeiros Junior P, Pimenta SP, Lopes RI, Kairalla RA, and Carvalho CR
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- Adult, Female, Hormones metabolism, Humans, Lymphangioleiomyomatosis drug therapy, Middle Aged, Retrospective Studies, Statistics, Nonparametric, Young Adult, Antineoplastic Agents, Hormonal therapeutic use, Goserelin therapeutic use, Lymphangioleiomyomatosis physiopathology, Vital Capacity drug effects
- Abstract
In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.
- Published
- 2011
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123. Biopsy-proven pulmonary determinants of heart disease.
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Zampieri FM, Parra ER, Canzian M, Antonângelo L, Luna Filho B, de Carvalho CR, Kairalla RA, and Capelozzi VL
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- Acute Lung Injury diagnostic imaging, Acute Lung Injury etiology, Adolescent, Adult, Aged, Biopsy, Blood-Air Barrier diagnostic imaging, Female, Heart Diseases complications, Heart Diseases diagnostic imaging, Humans, Male, Middle Aged, Predictive Value of Tests, Pulmonary Edema diagnostic imaging, Pulmonary Edema etiology, Retrospective Studies, Tomography, X-Ray Computed, Acute Lung Injury pathology, Blood-Air Barrier pathology, Heart Diseases pathology, Pulmonary Edema pathology
- Abstract
Heart disease (HD) can stress the alveolar blood-gas barrier, resulting in parenchymal inflammation and remodeling. Patients with HD may therefore display any of the symptoms commonly attributed to primary pulmonary disease, although tissue documentation of corresponding changes through surgical lung biopsy (SLB) is rarely done. Intent on exploring the basis of HD-related alveolar-capillary barrier dysfunction, a retrospective analysis of SLB histopathology was conducted in patients with clinically diagnosed HD, diffuse pulmonary infiltrates, and no evidence of primary pulmonary disease. Patients eligible for the study had a clinical diagnosis of heart disease, acute or chronic, and presented with diffuse infiltrates on chest X-ray. All qualified subjects (N = 23) who underwent diagnostic SLB between January 1982 and December 2005 were subsequently examined. Specific biopsy parameters investigated included demonstrable edema, siderophage influx, hemorrhage, venous and lymphatic ectasia, vascular sclerosis, capillary congestion, and fibroblast proliferation. Based on observed alveolar-capillary barrier (ACB) alterations, three main morphologic groups emerged: one group (6 patients) with alveolar edema; a second group (11 patients) characterized by pulmonary congestion; and a final group (6 patients) showing microscopic foci of acute ACB lung injury. Alveolar-capillary stress due to acute high-pressure or volume overload often manifests as diffuse pulmonary infiltrates with variable but generally predictable histopathology. In patients with biopsy-proven alveolar edema, pulmonary congestion, or acute microscopic lung injury, the clinician must be alert for the possibility of primary heart disease, particularly if the patient is elderly or when a history of myocardial, valvular, or coronary vascular disease exists.
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- 2010
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124. [Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy].
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Santana AN, Antunes T, Barros JM, Kairalla RA, Carvalho CR, and Barbas CS
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- Adult, Aneurysm diagnostic imaging, Anti-Inflammatory Agents therapeutic use, Behcet Syndrome diagnostic imaging, Behcet Syndrome drug therapy, Chlorambucil therapeutic use, Female, Hemorrhage diagnostic imaging, Humans, Hypertension, Pulmonary diagnostic imaging, Immunosuppressive Agents therapeutic use, Male, Middle Aged, Prednisone therapeutic use, Pulmonary Alveoli diagnostic imaging, Pulmonary Embolism diagnostic imaging, Radiography, Retrospective Studies, Survival Rate, Aneurysm etiology, Behcet Syndrome complications, Hypertension, Pulmonary etiology, Pulmonary Artery diagnostic imaging, Pulmonary Embolism etiology
- Abstract
Objective: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement., Methods: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts., Results: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 +/- 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8%, as was the five-year survival rate., Conclusions: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.
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- 2008
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125. Structural features of epithelial remodeling in usual interstitial pneumonia histologic pattern.
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Baptista AL, Parra ER, Filho JV, Kairalla RA, de Carvalho CR, and Capelozzi VL
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- Aged, Epithelial Cells pathology, Epithelium pathology, Epithelium physiology, Female, Humans, Lung physiology, Male, Middle Aged, Lung Diseases, Interstitial pathology
- Abstract
Epithelial remodeling probably contributes to parenchymal deterioration in usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF), but understanding its mechanisms is still a challenge. The aim of our study was to examine apoptosis and the epithelial changes in the histologic pattern of UIP. After immunohistochemical staining we quantified the content of type I cells, type II cells, surfactant-A protein, bcl-2, and Fas-ligand (Fas-L) in control and alveolar collapse, fibroblastic foci, and honeycomb in UIP areas of 23 open lung biopsies. A significant association was found between epithelial changes and parenchymal deterioration (p < 0.05). Type I epithelial cell density was similar between control (1.7 +/- 0.7%) and UIP alveolar collapse areas (1.8 +/- 0.6%) but decreased progressively in fibroblastic foci zones (0.8 +/- 0.4%) and honeycomb changes (0.4 +/- 0.3%). Type II cell density increased from control (25.6 +/- 8.3%) to areas of alveolar collapse (34.5 +/- 11.4%), then decreased toward fibroblastic foci (15.4 +/- 6.0%) and honeycomb change areas (23.1 +/- 8.6%). The surfactant-A protein increased from control (6.7 +/- 3.2%) to areas of alveolar collapse (31.1 +/- 9.5%) and decreased toward fibroblastic foci (14.5 +/- 4.9%) and honeycomb change areas (21.1 +/- 8.9%). Fas-L positive epithelial cell density presented a progressive decline from control (48.5 +/- 9.5%), alveolar collapse (37.9 +/- 12.4%), fibroblastic foci (27.4 +/- 6.8%), and honeycomb change areas (21.9 +/- 6.5%). A similar decline in density was found for bcl-2 positive epithelial cells from control (20.4 +/- 2.7%), alveolar collapse (18.9 +/- 5.1%), and fibroblastic foci areas (13.8 +/-2.9%), then increased honeycomb change areas (16.3 +/- 2.8%). We conclude that loss of the nuclear (bcl-2) and membrane (Fas-L) regulation of normal cell population density and suppression of cell death by apoptosis in UIP may be a determinant of the abnormal epithelial/parenchymal remodeling in UIP.
- Published
- 2006
- Full Text
- View/download PDF
126. Nonhomogeneous density of CD34 and VCAM-1 alveolar capillaries in major types of idiopathic interstitial pneumonia.
- Author
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Parra ER, Silvério da Costa LR, Ab'Saber A, Ribeiro de Carvalho CR, Kairalla RA, Fernezlian SM, Teixeira LR, and Capelozzi VL
- Subjects
- Capillaries metabolism, Cell Proliferation, Endothelium, Vascular cytology, Fibrosis metabolism, Immunohistochemistry, Pulmonary Alveoli blood supply, Antigens, CD34 metabolism, Lung Diseases, Interstitial metabolism, Pulmonary Alveoli metabolism, Vascular Cell Adhesion Molecule-1 metabolism
- Abstract
Integrin-immunoglobulin family ligand (CAMs) interactions between lung parenchymal cells (fibroblasts and epithelial cells) and integrin-extracellular matrix component interactions may be involved in the pathogenesis of idiopathic interstitial pneumonia (IIP). Among these, CD34 immunoquantitation allows determination of the degree of vascular proliferation (angiogenesis), whereas VCAM-1 immunoquantitation allows evaluation of the degree of endothelial activity and is strong evidence of inflammation. To validate the importance of vascular proliferation and endothelial cell activity within the alveolar walls and to explore the quantitative relationship between this factor and organizing fibrosis after parenchymal remodeling, we studied surgical lung biopsies in major IIP histologic patterns. We evaluated alveolar vascularity and activity in relation to the various degrees of organizing fibrosis in surgical lung biopsies of diffuse alveolar damage, nonspecific interstitial pneumonia, and usual interstitial pneumonia. Alveolar capillary endothelial cells were intensely immunoreactive with CD34 and VCAM-1. Vascular activity progressively increased in no-organizing fibrotic areas (normal, collapsed, and inflammatory septal areas), whereas vascular density gradually decreased as the degree of organizing fibrosis increased and was lower than that in control lungs in the most extensively fibrotic lesions (mural organizing fibrosis of usual interstitial pneumonia). These results indicate the presence of temporal nonhomogeneic vascular remodeling indiopathic interstitial pneumonia.
- Published
- 2005
- Full Text
- View/download PDF
127. Heterogeneous remodeling of lung vessels in idiopathic pulmonary fibrosis.
- Author
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Parra ER, David YR, da Costa LR, Ab'Saber A, Sousa R, Kairalla RA, de Carvalho CR, Filho MT, and Capelozzi VL
- Subjects
- Humans, Pulmonary Alveoli pathology, Pulmonary Fibrosis physiopathology, Respiratory Function Tests, Statistics, Nonparametric, Pulmonary Fibrosis pathology
- Abstract
Recently, several reports suggest differences in the vascularization of the various histopathologic patterns of parenchymal remodeling seen in usual interstitial pneumonia (UIP). In this study, we sought to validate the importance of vascular remodeling in patients with idiopathic pulmonary fibrosis (IPF) and to examine the relationship between vascular remodeling and parenchymal remodeling or pulmonary function. Open lung biopsies were performed in 57 patients with IPF, and vascular changes in alternating areas of parenchymal remodeling (UIP histologic patterns) were studied. Quantitative analysis of the internal area, internal perimeter, wall thickness, and surrounding cellularity of medium or large pulmonary arteries, as well as their distribution according to air/parenchymal ratios, was performed. Semiquantitative analysis also was used to determine the grade of vascular occlusion. An inverse association was found between vascularization and UIP parenchymal remodeling (p < 0.05); that is, the decreased internal luminal area and perimeter as well as the increased wall thickness run in parallel with progression from alveolar collapse toward severe mural-organizing fibrosis with honeycombing. Vascular regression (diminished internal area and perimeter of vessels) was also associated with higher FEV(1), FVC, and RV values (r = 0.48, p< 0.05), reflecting a tight relationship between vascular remodeling and pulmonary function. A progressive regression of vascularization, reflected by different degrees of luminal occlusion after vascular remodeling, coincided with parenchymal remodeling (alveolar collapse, mural-organizing fibrosis, and honeycombing). This vascular regression may be responsible for the impaired wound healing and progressive fibroproliferation found in patients with IPF. Further studies are needed to determine whether this relationship is causal or consequential.
- Published
- 2005
- Full Text
- View/download PDF
128. Recruitment maneuver and PEEP response in acute exacerbation of nonspecific interstitial pneumonia.
- Author
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Ferreira JC, Kairalla RA, Borges-Sobrinho JB, de Souza R, Schettino IA, and Carvalho CR
- Subjects
- Adult, Female, Humans, Lung Diseases, Interstitial complications, Respiratory Insufficiency etiology, Lung Diseases, Interstitial therapy, Positive-Pressure Respiration methods, Respiratory Insufficiency therapy
- Published
- 2004
129. Pulmonary and mediastinal "sarcoidosis" following surgical resection of cancer.
- Author
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Parra ER, Canzian M, Saber AM, Coêlho RS, Rodrigues FG, Kairalla RA, de Carvalho CR, and Capelozzi VL
- Subjects
- Aged, Diagnosis, Differential, Digestive System Surgical Procedures, Female, Granuloma pathology, Humans, Lung Neoplasms secondary, Lymph Nodes pathology, Male, Middle Aged, Thoracic Surgical Procedures, Tomography, X-Ray Computed, Lung Neoplasms pathology, Lung Neoplasms surgery, Mediastinal Diseases pathology, Sarcoidosis, Pulmonary pathology, Stomach Neoplasms surgery
- Abstract
Previous reports indicate that enlarged hilar and mediastinal lymph nodes caused by sarcoid-like reactions may develop after curative resection of cancer, and their presence does not necessarily denote neoplastic recurrence. Reports further suggest that coexisting pulmonary infiltrates in this setting may be related to sarcoidosis. In this study, we describe two patients who had resected lung and gastric cancer and who later developed pulmonary interstitial infiltrate, concurrent with progressive mediastinal lymphadenopathy initially thought to be caused by intrathoracic dissemination of their cancer. These changes were shown by open lung biopsy to be a benign, granulomatous reaction interpreted as sarcoidosis. Thus, it is important to recognize this clinical pattern when pulmonary infiltrates develop after complete treatment of cancer in an otherwise relapse-free patient and to encourage lung or lymph node biopsy in these particular settings in order to confirm a sarcoid-like reaction, thereby avoiding unnecessary chemotherapy for presumed tumor recurrence.
- Published
- 2004
- Full Text
- View/download PDF
130. Centrilobular fibrosis: a novel histological pattern of idiopathic interstitial pneumonia.
- Author
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de Carvalho ME, Kairalla RA, Capelozzi VL, Deheinzelin D, do Nascimento Saldiva PH, and de Carvalho CR
- Subjects
- Aged, Female, Forced Expiratory Volume, Humans, Lung Diseases, Interstitial physiopathology, Male, Middle Aged, Necrosis, Pulmonary Fibrosis pathology, Pulmonary Fibrosis physiopathology, Residual Volume, Respiratory Mucosa pathology, Retrospective Studies, Vital Capacity, Lung pathology, Lung Diseases, Interstitial classification, Lung Diseases, Interstitial pathology
- Abstract
The classification of idiopathic interstitial pneumonias (IIP) is still under debate. In this context, we observed in some of our patients with a clinical and radiological diagnosis of IIP a different histological picture with an aggressive centrilobular scarring centered in the bronchiolar epithelia, but involving the surrounding parenchyma, which underwent extensive remodeling. We hypothesized that this pattern is a form of IIP that could be separated out histologically from the previously described patterns, in particular from usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP). Forty-nine patients with clinical and radiological diagnosis of IIP and open-lung biopsies were retrospectively selected from 1982 to 1998. The biopsies were reviewed according to the following criteria: derangement of lobular architecture, temporal homogeneity and subpleural or bronchocentric distribution of the lesions, fibroblast foci, bronchial epithelium necrosis and regeneration, exposure of the basal membrane, squamous metaplasia, basophilic intraluminal contents, and foreign bodies within the remodeling airspaces. Three groups were found: UIP (24 patients), NSIP (13), and a third that we named centrilobular fibrosis (CLF) (12). All histological parameters were significantly different among the three groups (p < 0.001). CLF is a specific, homogeneous, and recognizable histological pattern of IIP, and can be isolated from UIP and NSIP.
- Published
- 2002
- Full Text
- View/download PDF
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