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Pulmonary artery stenosis is a frequent complication after arterial switch operation. This case study presented a complex pulmonary artery stenosis initially treated with bilateral pulmonary artery stenting. The patient was found to have aortic valve endocarditis and recurrent pulmonary artery stenosis. The aortic valve was replaced and the pulmonary valve was repaired, followed by interposition of a Y-shaped graft from the sinotubular junction to both pulmonary arteries. The patient had an uneventful recovery and was discharged home asymptomatic after endocarditis antibacterial treatment.
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Transposição das Grandes Artérias , Estenose da Valva Pulmonar , Transposição dos Grandes Vasos , Transposição das Grandes Artérias/efeitos adversos , Seguimentos , Humanos , Complicações Pós-Operatórias , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Reoperação , Transposição dos Grandes Vasos/cirurgiaRESUMO
INTRODUCTION: Silicosis is a fibrotic lung disease resulting from the inhalation of crystalline silica and can be classified as simple or complicated according to the International Labour Organization criteria. Furthermore, individuals exposed to crystalline silica also have a higher risk for the development of tuberculosis (Tb). The contribution of inflammatory cytokines to the risk of silicosis and Tb in different populations has previously been reported. Since genetic background might be related to susceptibility to silicosis and Tb, the study of polymorphisms within IL-1α, IL-1ß, and tumor necrosis factor protein-coding genes may contribute to elucidating the genetic basis of these diseases. METHODS: Single nucleotide polymorphisms (SNPs) were genotyped by polymerase chain reaction using restriction fragment length polymorphism or by Taqman methodology, in a sample of 102 silica-exposed patients from Brazil. RESULTS: No significant associations were observed between the SNPs studied and the severity of silicosis. However, significant associations were found between Tb and the C allele (odds ratio [OR] = 1.93, 95% confidence interval [CI], 1.01-3.73) and the CC genotype (OR = 2.34, 95% CI, 1.04-5.31) of IL1A -899C>T. The IL1B +3954C>T polymorphism also showed an association with Tb (T allele dominant model OR = 2.38, 95% CI, 1.04-5.41). CONCLUSION: These preliminary results demonstrate that the IL1A and IL1B gene variations may contribute to some extent to susceptibility to Tb, but not silicosis. However, additional studies are still needed to confirm these results.
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Interleucina-1alfa/genética , Interleucina-1beta/genética , Exposição Ocupacional , Polimorfismo de Nucleotídeo Único , Silicose/genética , Tuberculose/genética , Alelos , Brasil , Feminino , Predisposição Genética para Doença , Variação Genética , Genótipo , Humanos , Masculino , Fatores de Risco , Dióxido de Silício/toxicidade , Fator de Necrose Tumoral alfa/genéticaRESUMO
Hb F production is under the influence of major quantitative trait loci (QTL). The present study aims: i) to replicate the association with Hb F for representative genetic variants in the three major Hb F QTLs in a Portuguese sample of ß-thalassemia (ß-thal) carriers; and ii) to test different genetic multi-locus models to account for the genetic component of Hb F variation. A population sample of 79 Portuguese ß-thal carriers (39 males, 40 females), aged between 2 to 70 years old, were genotyped for polymorphisms in the locus control region (LCR)-5' hypersensitive site 4 (5'HS4) rs16912979, XmnI-HBG2 rs7482144, BCL11A rs1427407 and HMIP rs66650371, using standard biomolecular procedures. Univariate linear regression models were used to test for genetic associations with Hb F. The minor alleles of the individual variants BCL11A rs1427407 (T) (0.165), HMIP rs66650371 (3 bp del) (0.247) and XmnI-HBG2 rs7482144 (T) (0.196), were found to be significantly associated with increased levels of Hb F (p = 0.029, p = 0.002 and p = 0.0004, respectively), explaining about 6.0, 12.0 and 15.0% of Hb F variation, respectively. In a multiple linear regression approach, the three loci accounted for about 30.0% of Hb F variance. Two genetic risk scores (GRS), rationalizing the number of minor alleles into a single genetic variable, explained about 30.0 and 32.0% of the Hb F variation. In conclusion, we replicated in ß-thal carriers previously reported associations with Hb F. Multi-locus models combining three representative variants of Hb F influencing QTLs can explain a larger amount of Hb F variability.
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Hemoglobina Fetal/genética , Talassemia beta/genética , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Frequência do Gene , Variação Genética , Humanos , Região de Controle de Locus Gênico , Masculino , Pessoa de Meia-Idade , Polimorfismo de Nucleotídeo Único , Portugal/epidemiologia , Locos de Características Quantitativas , Adulto Jovem , Talassemia beta/epidemiologiaRESUMO
OBJECTIVE: The aim of this study was to compare image quality and radiation dose of coronary computed tomography (CT) angiography performed with dual-source CT scanner using 2 different protocols in patients with atrial fibrillation. METHODS: Forty-seven patients with AF underwent 2 different acquisition protocols: double high-pitch (DHP) spiral acquisition and retrospective spiral acquisition. The image quality was ranked according to a qualitative score by 2 experts: 1, no evident motion; 2, minimal motion not influencing coronary artery luminal evaluation; and 3, motion with impaired luminal evaluation. A third expert solved any disagreement. RESULTS: A total of 732 segments were evaluated. The DHP group (24 patients, 374 segments) showed more segments classified as score 1 than the retrospective spiral acquisition group (71.3% vs 37.4%). Image quality evaluation agreement was high between observers (κ = 0.8). There was significantly lower radiation exposure for the DHP group (3.65 [1.29] vs 23.57 [10.32] mSv). CONCLUSIONS: In this original direct comparison, a DHP spiral protocol for coronary CT angiography acquisition in patients with atrial fibrillation resulted in lower radiation exposure and superior image quality compared with conventional spiral retrospective acquisition.
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Fibrilação Atrial/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Angiografia Coronária/métodos , Tomografia Computadorizada Espiral/métodos , Idoso , Técnicas de Imagem de Sincronização Cardíaca , Feminino , Humanos , Masculino , Exposição à Radiação , Interpretação de Imagem Radiográfica Assistida por ComputadorRESUMO
Aiming to study the performance, carcass characteristics, nutrient digestibility, blood parameters, salivary cortisol levels, and economic viability of pigs administered aspartic protease, a total of 135 pigs were housed in pens in a randomized block design, divided into five treatments with nine replications. The experimental diets were positive control (PC), basic diet with a 5.0% reduction in protein and amino acid requirements; negative control (NC) with a 7.5% reduction in protein and amino acid requirements; NC + 100 g/mT of aspartic protease (NC100); NC + 150 g/mT of aspartic protease (NC150); and NC + 200 g/mT of neutral serine protease (NC200). The inclusion of protease, independently of the source and amount, increased the average daily weight gain (P < 0.05) of animals compared with the control treatments (PC and NC), improved feed conversion (P < 0.05) in early stages, and improved diet digestibility (P < 0.05) compared with the PC. Treatment with NC150 and NC200 resulted in greater carcass weights (P < 0.05) than treatment with the PC. NC100 led to a greater carcass yield than PC (P < 0.05), and NC150 resulted in a greater loin eye area than PC (P < 0.05). No differences (P > 0.05) in the blood parameters or salivary cortisol levels were found. Regarding economic viability, proteases increased the profitability, with NC150 leading to the best results. Thus, the use of aspartic proteases is recommended to improve performance and further facilitate pork production.
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Ração Animal , Ácido Aspártico Proteases , Suplementos Nutricionais , Digestão , Hidrocortisona , Saliva , Animais , Hidrocortisona/sangue , Hidrocortisona/metabolismo , Suínos , Ração Animal/análise , Saliva/metabolismo , Saliva/química , Digestão/fisiologia , Ácido Aspártico Proteases/metabolismo , Fenômenos Fisiológicos da Nutrição Animal , Masculino , Dieta/veterináriaRESUMO
The objective of this work was to evaluate the effect of a phytogenic compound for pigs in the growing and finishing phases as a possible substitute for ractopamine. A total of 140 pigs with an average initial weight of 48.8 kg ± 5.9 kg were used, distributed in a randomized block design, in a 3 × 2 factorial scheme (control diet (CONT), diet with inclusion of 2.5 kg per ton of a phytogenic compound (PC), and diet with 10 ppm of ractopamine (RAC), and two sexes: gilts and barrows), distributed in eight pens per treatment. The performance parameters were measured, and, at the end of the experimental period, the animals were slaughtered for carcass characteristics and pork quality analysis. The animals consuming RAC showed a better feed conversion, 4% improvement in relation to the group with the PC (p < 0.05). For daily weight gain, the animals supplemented with the PC showed 4.46% lower gain compared to RAC, and 3% greater gain compared to the CONT (p < 0.05). The animals that consumed the PC showed 5.6% lower shear force of the pork (p < 0.05) in relation to the CONT group and 29% lower in relation to the RAC group. The TBARS value presented a significant difference (p < 0.05), the CONTT group was 29% higher than the RAC, and the PC was 15.5% higher than the RAC. For chroma, the pork of the RAC group was 14% lower than the CONTT group and 10.3% less than the PC. There was no significant difference for the carcass parameters. It was concluded that the pigs in the ractopamine group presented the best performance; however, the phytogenic compound can be used against ractopamine's restriction because it improves daily weight gain and promotes a softer and less pale meat when compared with ractopamine.
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BACKGROUND: Scimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel. CASE SUMMARY: A 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the 'scimitar sign'. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign. DISCUSSION: Although the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.
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Parathyroidectomy (PTX) may cause low levels of PTH, leading to an excessive reduction of bone turnover, which is associated with poor outcomes in dialysis patients, including vascular calcification (VC). We aimed to prospectively investigate the impact of PTX on bone remodeling and its potential consequence on the progression of VC in hemodialysis patients. In this prospective study, 19 hemodialysis patients with severe secondary hyperparathyroidism (sHPT) were evaluated. All patients underwent laboratorial tests and coronary tomography at baseline and, 6 and 12 months after PTX; bone biopsy was performed at baseline and 12-month. At baseline, all patients had increased PTH levels up to 2500 pg/mL and high turnover bone disease in their bone biopsies. Fourteen (74%) patients had VC. During the follow-up, there was a significant decrease of PTH at 6 and 12-month. At 12-month, 90% of the patients evolved to low turnover bone disease. During the period of the hungry bone syndrome (first 6 months), no change of coronary calcium score was observed. However, calcium score increased significantly thereafter (12th month). There was an association between VC progression and the severity of low turnover bone disease. In conclusion, the shift from high to low turnover bone disease after PTX occurs in parallel to VC progression, contributing to the understanding of the complex pathophysiology involving mineral metabolism and cardiovascular disease in hemodialysis patients.
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Remodelação Óssea , Paratireoidectomia , Diálise Renal , Calcificação Vascular/patologia , Adulto , Biópsia , Osso e Ossos/patologia , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos ProspectivosAssuntos
Oncologia , Neoplasias , Brasil , Diagnóstico por Imagem , Humanos , Neoplasias/diagnóstico por imagemRESUMO
Silicosis is a disease caused by inhalation and deposition of crystalline silica that produces a fibrous tissue reaction. Bronchoalveolar lavage is a noninvasive technique that allows identifying particles, thus characterizing exposure to mineral dust. The aim of this work was to study bronchoalveolar lavage as a mean of estimating the amount of alveolar particles in different forms of silicosis. Bronchoalveolar lavage was carried out in 27 silicotic patients with simple (n = 12), complicated (n = 13), and acute (n = 2) forms of the disease and 7 healthy individuals as a control group. Macrophages were scored enumerating the percentage of cells with particles by polarizing light microscopy. Silicotic patients showed a marked increase in BAL cell particles compared to control population, however, relating simple to complicated silicosis, no significant difference was observed. In acute silicotic patients with massive silica dust exposure, more than 70% of BAL alveolar macrophages contained dust particles. When duration of exposure and retirement from work were evaluated, an inverse correlation between duration of exposure and the amount of alveolar dust, and a tendency to a smaller involvement on macrophages with the greater period of retirement from work were observed. These results suggest that particle analysis in BAL may be important in order to establish the nature and intensity of exposure.
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Líquido da Lavagem Broncoalveolar/química , Dióxido de Silício/análise , Silicose/diagnóstico , Adulto , Líquido da Lavagem Broncoalveolar/citologia , Humanos , Inalação , Macrófagos , Masculino , Pessoa de Meia-IdadeRESUMO
Abstract Background: Although associated with traditional cardiovascular risk factors, it is unclear whether obesity alone is associated with coronary artery disease (CAD). Objective: To investigate the role of obesity as a risk factor for CAD, defined by coronary computed tomography angiography (CCTA). Methods: This study retrospectively included 1,814 patients referred for CCTA in a hospital in São Paulo, from August 2010 to July 2012. CAD was identified by coronary calcium score and presence of coronary stenosis > 50%. Images were analyzed by two specialists, and the coronary findings were compared between obese and non-obese groups. A multivariate analysis model was used to assess obesity as an independent variable for the occurrence of obstructive CAD. Results: Among the study population, mean age was 58.5 +/- 11.5 years, 22.8% were obese (BMI = 30 kg/m2) and 66.3% were male. The prevalence of obstructive CAD was 18.4% in both groups. Obese patients had higher median calcium score compared to non-obese subjects (14.7 vs. 1.4, respectively, p = 0.019). In the multivariate analysis, obesity was not an independent factor for obstructive CAD (coefficient = -0.035, p = 0.102). Conclusion: Although no differences were observed in the prevalence of obstructive CAD between obese and non-obese individuals, coronary calcium scores were significantly in lower the latter group.
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Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Doença da Artéria Coronariana/complicações , Obesidade/complicações , Doença da Artéria Coronariana/epidemiologia , Índice de Massa Corporal , Cálcio/sangue , Prevalência , Estudos Retrospectivos , Dislipidemias , Angiografia por Tomografia Computadorizada/métodos , Fatores de Risco de Doenças CardíacasRESUMO
The diagnosis of silicosis is based on the history of exposure to silica dust associated with the radiological alterations compatible with the disease. With the main objective of comparing the alterations found in the chest radiography to those of high resolution computed tomography 49 patients with silicosis were selected. These imaging methods were evaluated separately by three readers and the results summarized through the median of the readings. The following alterations were analysed: nodules, coalescences, large opacities, lynfadenopathy. The patients were male, at an average age of 47.1 years, the majority being sandblasters, with an average exposure time to dust of 15.3 years. When the chest radiography was compared to high resolution computed tomography the following alterations were observed: i) the high resolution computed tomography showed greater profusion of nodules in 19 cases; ii) the high resolution computed tomography detected isolated coalescence and large opacities in 28 cases, seven of them who were classified initially as having simple silicosis were later changed to the complicated form; iii) the high resolution computed tomography was superior in the evaluation of lynfadenopathy in 16 cases. So, from the 49 analysed patients the high resolution computed tomography was more sensitive than the chest radiography in the evaluation of the alterations studied in 38 (77,5%) cases. Other alterations such as cavities, pleural thickening and emphysema were also observed only by this method in 5 cases. In the last 6 (12,2%) cases the high resolution computed tomography did not add any data when compared to chest radiography, four of these showed the initial form of the disease.
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Silicose/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Radiografia TorácicaRESUMO
The exogenous lipoid pneumonia, uncommon in adults, is the result of the inhalation and/or aspiration of lipid material into the tracheobronchial tree. This is often confused with bacterial pneumonia and pulmonary tuberculosis due to a nonspecific clinical and radiologic picture. It presents acutely or chronically and may result in pulmonary fibrosis. We describe here a case of lipoid pneumonia in a gas station attendant who siphoned gasoline to fill motorcycles; he was hospitalized due to presenting with a respiratory infection that was hard to resolve. The patient underwent bronchoscopy with bronchoalveolar lavage, which, on cytochemical (oil red O) evaluation, was slightly positive for lipid material in the foamy cytoplasm of alveolar macrophages. Due to his occupational history and radiographic abnormalities suggestive of lipoid pneumonia, a lung biopsy was performed to confirm the diagnosis. The patient was serially treated with segmental lung lavage and showed clinical, functional, and radiological improvement.
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OBJECTIVE: To compare the efficacy of aspirin and warfarin for prophylaxis of thrombosis in patients undergoing total cavopulmonary anastomosis. Evaluate whether coagulation factors (VII, VIII and protein C), clinical data, fenestration or hemodynamic factors, interfere with postoperative thrombosis. METHODS: A prospective, randomized study of 30 patients, randomized into Group I (Warfarin) and Group II (AAS), underwent total cavopulmonary shunt with extracardiac conduit, between 2008 and 2011, with follow-up by clinical visits to evaluate side effects and adhesion. Performed transesophageal echocardiography in post operatory time, 3, 6,12 and 24 months; angiotomography at 6, 12 and 24 months to evaluate changes in the internal tube wall or thrombi and pulmonary scintigraphy to evaluate possible PTE. RESULTS: Two deaths in group I; 33.3% of patients had thrombus (46.7% in Group II). The previous occurrence of thrombus and low levels of coagulation protein C were the only factors that influenced the time free of thrombus (P=0.035 and 0.047). Angiotomographic evaluation: 35.7% in group II presented material accumulation greater than 2 mm (P=0.082). Scintigraphy: two patients had PTE in group II. Five patients had difficulty to comply with the treatment, 4 in group I with INR ranging from 1 to 6.4. CONCLUSION: The previous occurrence of thrombus is a risk factor for thrombosis in the postoperative period. Patients using AAS tend to deposit material in the tube wall. The small sample size did not allow to conclude which is the most effective drug in the prevention of thrombosis in this population.
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Anticoagulantes/uso terapêutico , Aspirina/uso terapêutico , Técnica de Fontan/métodos , Inibidores da Agregação Plaquetária/uso terapêutico , Trombose/prevenção & controle , Varfarina/uso terapêutico , Adolescente , Criança , Pré-Escolar , Ecocardiografia , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Humanos , Estimativa de Kaplan-Meier , Masculino , Complicações Pós-Operatórias/tratamento farmacológico , Complicações Pós-Operatórias/prevenção & controle , Estudos Prospectivos , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/prevenção & controle , Cintilografia , Estatísticas não Paramétricas , Trombose/tratamento farmacológico , Trombose/mortalidade , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Disfunção Ventricular/fisiopatologiaRESUMO
BACKGROUND: Coronary computed tomography angiography (CTA) is a well-established diagnostic tool for coronary artery disease (CAD). However, coronary segments with prior stent implantation visualized with CTA may have limited evaluation and reduced accuracy. OBJECTIVE: We assessed the incremental value of stress myocardial CT perfusion (CTP) over anatomical assessment by coronary CTA alone in patients with stents, using quantitative invasive coronary angiography (≥50%) as reference. METHODS: Forty-six patients (56.9 ± 7.2 years; 28 men) referred to invasive coronary angiography were evaluated, combining coronary CTA and dipyridamole stress myocardial CTP with 64 detector-row CT. Coronary CTA was evaluated for ≥50% coronary stenosis, and myocardial CTP was used to potentially reclassify coronary territories, including those with stents and poorly evaluated stents because of artifacts. RESULTS: We evaluated 138 coronary territories, 62 with ≥1 stent. From these, 21 (34%) territories had adequately evaluated stents, 28 (45%) had limitedly evaluated stents still allowing diagnosis, and 13 (21%) had inadequately evaluated stents (no luminal assessment possible). Sensitivity, specificity, positive predictive value, negative predictive value and accuracy for coronary CTA in stent territories were, respectively, 85%, 77%, 87%, 74%, and 81%, and the combined use of coronary CTA and myocardial CTP were 88%, 95%, 97%, 81%, and 91% (P = 0.0292). In territories with impaired stent evaluation (limited or inadequate), the diagnostic performance of coronary CTA alone was 83%, 72%, 79%, 76% and 77%, and combined with myocardial CTP were 87%, 94%, 95%, 85%, and 91% (P = 0.036). CONCLUSION: The combined evaluation of coronary CTA and stress myocardial CTP improved the diagnostic accuracy for the detection of significant obstructive CAD in patients with stents.
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Angioplastia Coronária com Balão/instrumentação , Circulação Coronária , Reestenose Coronária/diagnóstico por imagem , Dipiridamol , Imagem de Perfusão do Miocárdio/métodos , Stents , Tomografia Computadorizada Espiral , Vasodilatadores , Angioplastia Coronária com Balão/efeitos adversos , Artefatos , Brasil , Distribuição de Qui-Quadrado , Angiografia Coronária , Reestenose Coronária/etiologia , Reestenose Coronária/fisiopatologia , Feminino , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Curva ROC , Sensibilidade e EspecificidadeRESUMO
CONTEXT: Man-made vitreous fibers (MMVFs) are noncrystalline inorganic fibrous material used for thermal and acoustical insulation (e.g., rock wool, glass wool, glass microfibers, and refractory ceramic fibers). Neither epidemiologic studies of human exposure nor animal studies have shown a noticeable hazardous effect of glass wools on health. However, MMVFs have been anecdotally associated with granulomatous lung disease in several case reports. CASE PRESENTATION: Here, we describe the case of a patient with multiple bilateral nodular opacities who was exposed to glass wool fibers and coating materials for 7 years. Bronchoalveolar lavage fluid revealed an increased total cell count (predominantly macrophages) with numerous cytoplasmic particles. Lung biopsy showed peribronchiolar infiltration of lymphoid cells and many foreign-body-type granulomas. Alveolar macrophages had numerous round and elongated platelike particles inside the cytoplasm. X-ray microanalysis of these particles detected mainly oxygen/aluminum/silicon and oxygen/magnesium/silicon, compatible with kaolinite and talc, respectively. No elemental evidence for glass fibers was found in lung biopsy. DISCUSSION: The contribution of analytical electron microscopy applied in the lung biopsy was imperative to confirm the diagnosis of pneumoconiosis associated with a complex occupational exposure that included both MMVFs and coating materials. RELEVANCE TO CLINICAL OR PROFESSIONAL PRACTICE: This case study points out the possible participation of other components (coating materials), beyond MMVFs, in the etiology of pneumoconiosis.
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Poluentes Ocupacionais do Ar/efeitos adversos , Vidro , Exposição Ocupacional/efeitos adversos , Pneumoconiose/diagnóstico , Pneumoconiose/etiologia , Adulto , Humanos , Pulmão/patologia , Pulmão/ultraestrutura , Masculino , Microscopia Eletrônica de Transmissão , Pneumoconiose/metabolismoRESUMO
BACKGROUND: Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction. METHODS: Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period. RESULTS: Histopathologic data revealed hypertrophy of the RV cardiomyocytes and increased interstitial collagen in both ventricles. Mean values of RV isovolumic acceleration decreased significantly at the third evaluation compared with the preoperative values (P = .006). RV myocardial fibrosis greater than 8.3% was associated with a probability of altered E' of at least 0.7 (odds ratio = 2.31). CONCLUSION: Preoperative histologic myocardial remodeling influenced the postoperative RV function in this group of patients with late repair. Further studies are necessary to evaluate the myocardium in younger patients and to define its influence in the long-term follow-up.