RESUMO
Las calcificaciones pulmonares metastásicas, hacen referencia a una enfermedad metabólica, caracterizada por depósitos de calcio en tejido pulmonar sano. La etiología es amplia e incluye enfermedades malignas y benignas, siendo la falla renal la causa más frecuente. Es una condición, que, a pesar de ser frecuente, suele ser subdiagnosticada, por presentar pocos o ningún síntoma. Presentamos tres casos clínicos asociados a enfermedad renal crónica, pre y post trasplante.
Metastatic pulmonary calcifications refer to a metabolic disease, characterized by calcium deposits in healthy lung tissue. The etiology is broad and includes malignant and benign diseases, the kidney failure being the most frequent cause. It is a condition, which, despite being frequent, is usually underdiagnosed, because it presents few or no symptoms. We present three clinical cases associated with pre- and post-transplant kidney disease.
Assuntos
Humanos , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Calcinose/etiologia , Insuficiência Renal Crônica/complicações , Pneumopatias/etiologia , Testes de Função Respiratória , Calcinose/diagnóstico por imagem , Radiografia Torácica , Tomografia Computadorizada por Raios X , Pneumopatias/fisiopatologia , Pneumopatias/diagnóstico por imagemRESUMO
Objetivo: determinar a incidência, os fatores associados e o impacto das complicações pulmonares no pós-operatório de cirurgia cardíaca pediátrica. Metodologia: estudo de coorte, prospectivo, que incluiu lactentes e crianças submetidas à cirurgia cardíaca em um hospital pediátrico, no período de novembro de 2016 a julho de 2019. Foram coletados dados dos prontuários referentes ao sexo, idade, presença de outras malformações associadas, tipo de cardiopatia, ocorrência de complicações pulmonares, tempo de ventilação mecânica (VM) e de internamento na unidade de terapia intensiva (UTI) e óbito. Resultados: a amostra foi constituída por 111 lactentes e crianças, mediana da idade de 13 meses (7-32 meses), 54,1% do sexo feminino. Quanto ao tipo de cardiopatia, 80,2% foram cianogênicas. As complicações pulmonares ocorreram em 44,1% dos casos, sendo a mais frequente a atelectasia. A mediana do tempo de VM foi 8 horas (1-48h) e 45 (40,5%) permaneceram na VM por mais de 24h. A mediana do tempo de internamento na UTI foi de 7 dias (4-12dias). Evoluíram a óbito 7 (14,3%) pacientes. Conclusão: a amostra investigada apresentou incidência elevada de complicações pulmonares no pós-operatório de cirurgia cardíaca
Objective: to determine the incidence, associated factors and impact of pulmonary complications in the postoperative period of pediatric cardiac surgery. Methodology: prospective cohort study, which included infants and children undergoing cardiac surgery in a pediatric hospital, from November 2016 to July 2019. Data were collected from medical records regarding sex, age, presence of other associated malformations, type of heart disease, occurrence of pulmonary complications, duration of mechanical ventilation (MV) and admission to the intensive care unit (ICU) stay and death. Results: the sample consisted of 111 infants and children, median age 13 months (7-32 months), 54.1% female. As for the type of heart disease, 80.2% were acyanotic. Pulmonary complications occurred in 44.1% of cases, with atelectasis being the most frequent. The median time on mechanical ventilation (MV) was 8 hours (1-48h) and 45 (40.5%) remained on MV for more than 24h. The median length of stay in the ICU was 7 days (4-12 days). 7 (14.3%) patients died. Conclusion: the investigated sample had a high incidence of pulmonary complications in the postoperative period of cardiac surgery
Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Complicações Pós-Operatórias , Cardiopatias Congênitas/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Pneumopatias/etiologia , Incidência , Estudos ProspectivosRESUMO
Neurocritical care (NCC) is not only generally guided by principles of general intensive care, but also directed by specific goals and methods. This review summarizes the common pulmonary diseases and pathophysiology affecting NCC patients and the progress made in strategies of respiratory support in NCC. This review highlights the possible interactions and pathways that have been revealed between neurological injuries and respiratory diseases, including the catecholamine pathway, systemic inflammatory reactions, adrenergic hypersensitivity, and dopaminergic signaling. Pulmonary complications of neurocritical patients include pneumonia, neurological pulmonary edema, and respiratory distress. Specific aspects of respiratory management include prioritizing the protection of the brain, and the goal of respiratory management is to avoid inappropriate blood gas composition levels and intracranial hypertension. Compared with the traditional mode of protective mechanical ventilation with low tidal volume (Vt), high positive end-expiratory pressure (PEEP), and recruitment maneuvers, low PEEP might yield a potential benefit in closing and protecting the lung tissue. Multimodal neuromonitoring can ensure the safety of respiratory maneuvers in clinical and scientific practice. Future studies are required to develop guidelines for respiratory management in NCC.
Assuntos
Humanos , Pulmão , Pneumopatias/etiologia , Respiração com Pressão Positiva/métodos , Respiração Artificial/efeitos adversos , Volume de Ventilação PulmonarRESUMO
Resumen La leptospirosis es una de las zoonosis endémicas más importantes en el mundo con un aumento de la incidencia en los últimos años. En el personal militar podría ser catalogada como una enfermedad ocupacional dado sus actividades específicas en áreas rurales. Su presentación clínica es variable siendo en la mayoría de los casos una enfermedad febril autolimitada. De acuerdo con diversos factores dependientes del patógeno y del hospedero pueden presentarse manifestaciones severas de la enfermedad dentro de la cual destaca el compromiso pulmonar con una alta tasa de mortalidad. Existe evidencia del uso de esteroide sistémico como parte del tratamiento de esta complicación. Presentamos el caso de un paciente joven, militar, que debuta con síndrome de hemorragia alveolar difusa secundario a leptospirosis y presenta una excelente respuesta al tratamiento con altas dosis de metilprednisolona, con una discusión del proceso diagnóstico y aspectos fisiopatológicos de esta condición.
Leptospirosis is one of the most important endemic zoonoses in the world with an increase in incidence in recent years. In military personnel it could be classified as an occupational disease given their specific activities in rural areas. Its clinical presentation is variable being in most cases a self-limited febrile disease. According to various factors dependent on the pathogen and the host, severe manifestations of the disease may occur within which the pulmonary involvement with a high mortality rate stands out. There is evidence of systemic steroid use as part of the treatment of this complication. We present a case of a young, military patient who debuts with diffuse alveolar hemorrhage syndrome secondary to leptospirosis and presents an excellent response to treatment with high doses of methylprednisolone, with a discussion of the diagnostic process and pathophysiological aspects of this condition.
Assuntos
Humanos , Masculino , Adulto Jovem , Alvéolos Pulmonares/patologia , Hemorragia/etiologia , Leptospirose/complicações , Pneumopatias/etiologia , Esteroides/uso terapêutico , Doença de Weil , Zoonoses , Zona Tropical , Leptospirose/diagnóstico , Leptospirose/tratamento farmacológico , Militares , Doenças ProfissionaisRESUMO
ABSTRACT Objective: To determine the CT findings of multiple cavitary lung lesions that allow the differentiation between benign and malignant etiologies. Methods: We reviewed CT scans, including patients with two or more cavitary lung lesions. We evaluated the number of cavitary lesions, their location, cavity wall thickness, and additional findings, correlating the variables with the diagnosis of a benign or malignant lesion. Results: We reviewed the chest CT scans of 102 patients, 58 (56.9%) of whom were male. The average age was 50.5 ± 18.0 years. Benign and malignant lesions were diagnosed in 74 (72.6%) and 28 (27.4%) of the patients, respectively. On the CT scans, the mean number of cavities was 3, the mean wall thickness of the largest lesions was 6.0 mm, and the mean diameter of the largest lesions was 27.0 mm. The lesions were predominantly in the upper lobes, especially on the right (in 43.1%). In our comparison of the variables studied, a diagnosis of malignancy was not found to correlate significantly with the wall thickness of the largest cavity, lymph node enlargement, emphysema, consolidation, bronchiectasis, or bronchial obstruction. The presence of centrilobular nodules correlated significantly with the absence of malignant disease (p < 0.05). In contrast, a greater number of cavities correlated significantly with malignancy (p < 0.026). Conclusions: A larger number of cavitary lung lesions and the absence of centrilobular nodules may be characteristic of a malignant etiology. However, on the basis of our evaluation of the lesions in our sample, we cannot state that wall thickness is a good indicator of a benign or malignant etiology.
RESUMO Objetivo: Determinar os achados tomográficos de lesões escavadas pulmonares múltiplas que contribuem para a diferenciação entre etiologia benigna e maligna. Métodos: Foram revisados exames de TC, sendo incluídos pacientes com duas ou mais lesões pulmonares escavadas. Avaliaram-se a quantidade de lesões escavadas, sua localização, espessura parietal das lesões e achados adicionais, correlacionando as variáveis com a presença de diagnóstico de benignidade ou de malignidade. Resultados: Foram revisadas TCs de tórax de 102 pacientes, sendo 58 (56,9%) desses do sexo masculino. A média de idade foi de 50,5 ± 18,0 anos. Lesões benignas e malignas foram diagnosticadas em 74 pacientes (72,6%) e em 28 (27,4%), respectivamente. Quanto aos achados tomográficos, a média do número de cavidades foi 3, a da espessura média da parede da maior lesão foi de 6,0 mm, e a do diâmetro da maior lesão foi de 27,0 mm. Houve predomínio das lesões em lobos superiores, especialmente no direito (43,1%). Após a comparação das variáveis estudadas, a espessura parietal da maior escavação, assim como a presença de linfonodomegalia, enfisema, consolidação, bronquiectasias e obstrução brônquica, não apresentaram diferenças estatisticamente significativas para o diagnóstico de malignidade. A presença de nódulos centrolobulares correlacionou-se significativamente com a ausência de doença maligna (p < 0,05). Observou-se também que um número maior de cavidades se correlacionou significativamente com malignidade (p < 0,026). Conclusões: Um maior número de lesões pulmonares escavadas e a ausência de nódulos centrolobulares podem ser características relacionadas à etiologia maligna. Por outro lado, a espessura parietal não permitiu a diferenciação entre etiologia benigna e maligna das lesões em nossa amostra.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Tomografia Computadorizada por Raios X/métodos , Pneumopatias/diagnóstico por imagem , Tuberculose/etiologia , Tuberculose/diagnóstico por imagem , Estudos Transversais , Estudos Retrospectivos , Diagnóstico Diferencial , Tolerância Imunológica , Pneumopatias/etiologia , Neoplasias Pulmonares/etiologia , Neoplasias Pulmonares/diagnóstico por imagemRESUMO
Abstract Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. A small number of studies have examined the role of AMPs on autoimmune diseases which has not been studied in scleroderma yet. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc. Methods: Forty-two patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) were enrolled. For SSc patients, the following data were recorded: disease subset (limited/diffuse), autoantibodies (antinuclear, anti-centromere (ACA), and anti-SCL-70), blood tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), modified Rodnan skin score, presence and history of digital ulcers, kidney, gastrointestinal disease and lung involvement assessed by computed tomography and pulmonary function tests. Association between serum AMPs and disease characteristics were analysed. Results: Twenty-nine of the patients had diffuse (69%) and 13 of the patients had limited (31%) systemic sclerosis. Average disease duration was 5.5 years. Pulmonary involvement was detected in 20 patients (47.6%). Serum concentration of alpha defensin was higher than healthy subjects (563 ± 415 vs 377 ± 269 ng/mL, p = 0.02). However, no difference was observed for beta-1 and beta-2 defensins in SSc patients and healthy controls. In sub-group analysis patients with interstitial lung disease had higher levels of alpha defensin than those without lung involvement (684 ± 473 vs 430 ± 299 ng/ml, p = 0.04). There was also correlation between alfa defensin serum concentrations and CRP (r = 0.34). Conclusions: Alpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease. Trial registration: This study is not a clinical trial study.(AU)
Assuntos
Humanos , Escleroderma Sistêmico/patologia , Peptídeos Catiônicos Antimicrobianos/sangue , alfa-Defensinas/sangue , beta-Defensinas/sangue , Pneumopatias/etiologiaRESUMO
Resumen:La granulomatosis con poliangeítis (GPA) es una vasculitis sistémica de pequeño vaso, que afecta más frecuentemente el tracto respiratorio y el riñón. Sus criterios diagnósticos se basan en la clínica, exámenes de laboratorio, imágenes e histología. El 90% son ANCA (anticuerpos anticitoplasma de neutrófilos) positivos. La histología muestra inflamación granulomatosa, necrosis y vasculitis. Los exámenes de imagen son de vital importancia en su estudio inicial y seguimiento, correspondiendo principalmente a técnicas tomográficas. La tomografía Computada (TC) es el método de elección para la evaluación de vía aérea superior y pulmón, con alta sensibilidad en afectación de cavidades nasal/paranasales, árbol bronquial y pulmón. La Resonancia Magnética está indicada en compromiso del sistema nervioso central y corazón. El PET/CT presenta alta sensibilidad en enfermedad tóraco-abdominal, es de utilidad en detectar lesiones no visibles con otras técnicas, y en control de tratamiento. El compromiso renal, de alta ocurrencia en GPA, presenta escasa traducción en las imágenes y es frecuentemente indetectable con imágenes, aunque el PET/CT puede ser positivo en casos de glomerulonefritis acentuada. La radiología simple no debe ser utilizada en el estudio de GPA dado su bajo rendimiento diagnóstico. El tratamiento se basa en terapia corticoidea e inmunosupresora. Las recaídas son frecuentes, por lo que estos pacientes requieren seguimiento a largo plazo.
Abstract:Granulomatosis with polyangiitis (GPA) is a systemic type of vasculitis that affects small vessels, most commonly involving the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, imaging and histology. Ninety percent are ANCA (anti-neutrophilic cytoplasmic antibodies) positive. Histology demonstrates granulomatous inflammation, necrosis and vasculitis. Imaging studies are vital for the initial work-up and follow-up. Computed Tomography (CT) is the method of choice for evaluation of the upper airway and lungs, because of its high sensitivity detecting anomalies of paranasal sinuses, bronchial tree and lungs. Magnetic Resonance is indicated for evaluation of the central nervous system and heart. PET/CT has high sensitivity for thoracic and abdominal disease, is useful at detecting lesions not seen with other imaging techniques, and for follow-up. Renal involvement, very frequent on GPA, is usually undetectable at imaging, but may be seen at PET/CT in cases of marked glomerulonephritis. Plain X-rays should not be used for evaluation of GPA because of their low diagnostic performance. Treatment is based on corticosteroid and immunosuppressive therapy. Relapses are frequent, so these patients require long-term follow-up.
Assuntos
Humanos , Tomografia Computadorizada por Raios X , Granulomatose com Poliangiite/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/patologia , Nefropatias/etiologia , Pneumopatias/etiologiaRESUMO
Resumen Objetivo: Determinar la frecuencia, tipo y predictores de complicaciones pleuropulmonares en los primeros 30 días de postoperatorio de pacientes intervenidos de cirugía cardiovascular sin apoyo de circulación extracorpórea. Métodos: Se realizó un estudio de cohorte retrospectivo durante el periodo comprendido del 1 de enero de 2013 al 31 de diciembre 2014. Incluyó a todos los pacientes portadores de cardiopatías congénitas intervenidos de cirugía cardiaca con abordaje esternal o torácico, sin soporte de circulación extracorpórea con ingreso registrado a Unidad de Cuidados Intensivos del Hospital de Pediatría del Centro Médico Nacional Siglo XXI, IMSS. Se cuantificó la frecuencia de eventos de las complicaciones pleuropulmonares y se realizó un análisis multivariado de regresión logística para identificar los factores de riesgo asociados a complicaciones pleuropulmonares, calculándose odds ratio (OR) e intervalos de confianza al 95% (IC 95%). Resultados: Se incluyeron un total de 139 pacientes, en los cuales la frecuencia de complicaciones pleuropulmonares fue del 42.4% (n = 59), y los tipos más frecuentes fueron atelectasia (28 eventos), neumonía asociada a ventilador (24 eventos), neumotórax (20 eventos), pudiéndose encontrar más de una complicación por paciente. Los predictores más significativos de complicaciones pleuropulmonares fueron las cardiopatías congénitas cianógenas (OR = 3.58; IC 95%: 1.10-7.50; p = 0.001), el abordaje por toracotomía (OR = 1.46; IC 95%: 1.18-1.12; p = 0.008) y el evento quirúrgico realizado de urgencia (OR = 3.46; IC 95%: 1.51-7.95; p = 0.002). Conclusiones: La principal complicación pleuropulmonar fue la atelectasia lo cual concuerda con lo reportado en la literatura internacional. Los pacientes que presenten alguno de los predictores identificados en el presente estudio deben ser monitorizados de manera especial para prevenir, detectar y/o tratar oportunamente las complicaciones pleuropulmonares tras cirugía cardiaca.
Abstract Objective: To determine the frequency and type of pleuropulmonary complications and their predictors in the first thirty postoperative days of patients undergoing surgery without cardiopulmonary bypass. Methods: A retrospective cohort study was carried out between January 2013 and December 2014. It included all patients with congenital heart disease who underwent cardiac surgery using a sternal or thoracic approach, without cardiopulmonary bypass with a registered admission to a Neonatal or Paediatric Intensive Care. The frequency of events of pleuropulmonary complications and logistic regression analysis was performed, and the adjusted odds ratio (OR) and confidence intervals at 95% (95% CI) were calculated. Results: A total of 139 patients were included. The frequency of pleuropulmonary complications was 42.4% (N = 59), and the most frequent types were atelectasis (28 events), ventilator-associated pneumonia (24 events), pneumothorax (20 events), with more than one complication per patient occasionally being found. Significant risk factors were cyanogenic congenital heart disease (OR = 3.58, 95% CI: 1.10-7.50, P =.001), thoracotomy approach (OR = 1.46, 95% CI: 1.18-1.12, P = .008), and an emergency surgical event (OR = 3.46, 95% CI: 1.51-7.95, P = .002). Conclusions: The main pleuropulmonary complication was atelectasis, which is consistent with that reported in the international literature. Patients with any of the predictors identified in the present study should be closely monitored in order to prevent, detect and/or treat pleuropulmonary complications in a timely manner after cardiac surgery.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Doenças Pleurais/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Procedimentos Cirúrgicos Cardíacos/métodos , Pneumopatias/epidemiologia , Doenças Pleurais/etiologia , Doenças Pleurais/fisiopatologia , Complicações Pós-Operatórias/fisiopatologia , Atelectasia Pulmonar/etiologia , Atelectasia Pulmonar/epidemiologia , Unidades de Terapia Intensiva Pediátrica , Unidades de Terapia Intensiva Neonatal , Modelos Logísticos , Estudos Retrospectivos , Estudos de Coortes , Cardiopatias Congênitas/cirurgia , Pneumopatias/etiologia , Pneumopatias/fisiopatologiaRESUMO
Abstract Objective: To compare the effects of extended- versus short-time noninvasive positive pressure ventilation on pulmonary function, tissue perfusion, and clinical outcomes in the early postoperative period following coronary artery bypass surgery in patients with preserved left ventricular function. Methods: Patients were randomized into two groups according to noninvasive positive pressure ventilation intensity: short-time noninvasive positive pressure ventilation n=20 (S-NPPV) and extended-time noninvasive positive pressure ventilation n=21 (E-NPPV). S-NPPV was applied for 60 minutes during immediate postoperative period and 10 minutes, twice daily, from postoperative days 1-5. E-NPPV was performed for at least six hours during immediate postoperative period and 60 minutes, twice daily, from postoperative days 1-5. As a primary outcome, tissue perfusion was determined by central venous oxygen saturation and blood lactate level measured after anesthetic induction, immediately after extubation and following noninvasive positive pressure ventilation protocols. As a secondary outcome, pulmonary function tests were performed preoperatively and in the postoperative days 1, 3, and 5; clinical outcomes were recorded. Results: Significant drop in blood lactate levels and an improvement in central venous oxygen saturation values in the E-NPPV group were observed when compared with S-NPPV group after study protocol (P<0.01). The E-NPPV group presented higher preservation of postoperative pulmonary function as well as lower incidence of respiratory events and shorter postoperative hospital stay (P<0.05). Conclusion: Prophylactic E-NPPV administered in the early postoperative period of coronary artery bypass surgery resulted in greater improvements in tissue perfusion, pulmonary function and clinical outcomes than S-NPPV, in patients with preserved left ventricular function. Trial Registration: Brazilian Registry of Clinical trial - RBR7sqj78 - http://www.ensaiosclinicos.gov.br
Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Ponte de Artéria Coronária/métodos , Respiração com Pressão Positiva/métodos , Ventilação não Invasiva/métodos , Pneumopatias/prevenção & controle , Oxigênio/sangue , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/sangue , Fatores de Tempo , Capacidade Vital , Volume Expiratório Forçado , Ponte de Artéria Coronária/efeitos adversos , Fatores de Risco , Análise de Variância , Resultado do Tratamento , Estatísticas não Paramétricas , Ácido Láctico/sangue , Tempo de Internação , Pneumopatias/etiologia , Pneumopatias/sangueRESUMO
Spirometry has been used as the main strategy for assessing ventilatory changes related to occupational exposure to particulate matter (OEPM). However, in some cases, as one of its limitations, it may not be sensitive enough to show abnormalities before extensive damage, as seen in restrictive lung diseases. Therefore, we hypothesized that cardiopulmonary exercise testing (CPET) may be better than spirometry to detect early ventilatory impairment caused by OEPM. We selected 135 male workers with at least one year of exposure. After collection of self-reported socioeconomic status, educational level, and cardiovascular risk data, participants underwent spirometry, CPET, body composition assessment (bioelectrical impedance), and triaxial accelerometry (for level of physical activity in daily life). CPET was performed using a ramp protocol on a treadmill. Metabolic, cardiovascular, ventilatory, and submaximal relationships were measured. We compared 52 exposed to 83 non-exposed workers. Multiple linear regressions were developed using spirometry and CPET variables as outcomes and OEPM as the main predictor, and adjusted by the main covariates. Our results showed that OEPM was associated with significant reductions in peak minute ventilation, peak tidal volume, and breathing reserve index. Exposed participants presented shallower slope of ΔVT/ΔlnV̇E (breathing pattern), i.e., increased tachypneic breathing pattern. The OEPM explained 7.4% of the ΔVT/ΔlnV̇E variability. We found no significant influence of spirometric indices after multiple linear regressions. We conclude that CPET might be a more sensitive feature of assessing early pulmonary impairment related to OEPM. Our cross-sectional results suggested that CPET is a promising tool for the screening of asymptomatic male workers.
Assuntos
Humanos , Adulto , Exposição Ocupacional/efeitos adversos , Exposição Ambiental/efeitos adversos , Material Particulado/efeitos adversos , Aptidão Cardiorrespiratória/fisiologia , Pneumopatias/etiologia , Espirometria , Troca Gasosa Pulmonar , Estudos Transversais , Fatores de Risco , Teste de Esforço/métodos , Pneumopatias/diagnóstico , Pneumopatias/fisiopatologiaRESUMO
ABSTRACT Objective: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. Methods: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen. Results: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone. Conclusions: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.
RESUMO Objetivo: Analisar os achados de TCAR em pacientes com doença de Niemann-Pick (DNP) tipo B a fim de avaliar a frequência dos padrões tomográficos e sua distribuição no parênquima pulmonar, além das características clínicas mais frequentes. Métodos: Foram estudados 13 pacientes (3 do sexo masculino e 10 do sexo feminino) com idades variando de 5 a 56 anos. As imagens de TCAR foram avaliadas por dois observadores de forma independente, e os casos discordantes foram resolvidos por consenso. Os critérios de inclusão foram presença de anormalidades na TCAR e diagnóstico confirmado de DNP tipo B por exame anatomopatológico através de biópsias de medula óssea, pulmão ou fígado. Resultados: Os achados clínicos mais comuns foram hepatoesplenomegalia e dispneia leve a moderada. Os padrões tomográficos mais frequentes foram espessamento liso de septos interlobulares e opacidades em vidro fosco, presentes em todos os pacientes. Linhas intralobulares estiveram presentes em 12 pacientes (92,3%). O padrão de pavimentação em mosaico foi observado em 5 pacientes (38,4%). Áreas de aprisionamento aéreo foram identificadas em 1 dos casos (7,6%). O comprometimento pulmonar foi bilateral em todos os casos, sendo o terço inferior dos pulmões a região mais envolvida. Conclusões: O achado de espessamento liso de septos interlobulares, com ou sem opacidades em vidro fosco associadas, em pacientes com hepatoesplenomegalia é o achado mais frequente na DNP tipo B.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Doenças de Niemann-Pick/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Doenças de Niemann-Pick/complicações , Estudos Retrospectivos , Pneumopatias/etiologiaRESUMO
ABSTRACT We describe the case of a 33-year-old man, a chronic user of powder cocaine, who presented with dyspnea, fever, night sweats, and significant weight loss. Chest HRCT revealed centrilobular nodules, giving an initial impression of miliary tuberculosis. Therefore, he was started on an empirical, four-drug antituberculosis treatment regimen. Four weeks later, despite the tuberculosis treatment, he continued to have the same symptoms. We then performed transbronchial lung biopsy. Histopathological analysis of the biopsy sample revealed birefringent foreign body granuloma. A corroborative history of cocaine snorting, the presence of centrilobular nodules, and the foreign body-related histopathological findings led to a diagnosis of pulmonary foreign body granulomatosis. This report underscores the fact that pulmonary foreign body granulomatosis should be included in the differential diagnosis of clinical profiles resembling tuberculosis.
RESUMO Descrevemos o caso de um homem de 33 anos de idade, usuário crônico de cocaína em pó, que apresentava dispneia, febre, sudorese noturna e perda de peso significativa. A TCAR de tórax revelou nódulos centrolobulares, dando uma impressão inicial de tuberculose miliar. Por isso, o paciente passou a receber tratamento empírico com quatro tuberculostáticos. Quatro semanas depois, apesar do tratamento antituberculose, o paciente continuou a apresentar os mesmos sintomas. Foi então realizada a biópsia pulmonar transbrônquica. A análise histopatológica da amostra obtida revelou granuloma de corpo estranho birrefringente. A história de uso de cocaína por inalação, a presença de nódulos centrolobulares e os achados histopatológicos de corpos estranhos confirmaram o diagnóstico de granulomatose pulmonar de corpo estranho. Este relato destaca o fato de que a granulomatose pulmonar de corpo estranho deve ser incluída no diagnóstico diferencial de perfis clínicos que se assemelham a tuberculose.
Assuntos
Humanos , Masculino , Adulto , Granuloma de Corpo Estranho/etiologia , Transtornos Relacionados ao Uso de Cocaína/complicações , Pneumopatias/etiologia , Granuloma de Corpo Estranho/diagnóstico , Evolução Fatal , Pneumopatias/diagnósticoRESUMO
ABSTRACT Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions. When combined with clinical and laboratory findings, HRCT is often sufficient for the etiological definition of diffuse lung cysts, avoiding the need for lung biopsy. The differential diagnoses of diffuse cystic lung diseases are myriad, including neoplastic, inflammatory, and infectious etiologies. Pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, lymphocytic interstitial pneumonia, and follicular bronchiolitis are the most common diseases that produce this CT pattern. However, new diseases have been included as potential determinants of this pattern.
RESUMO As doenças pulmonares císticas difusas se caracterizam pela presença de cistos envolvendo mais de um lobo pulmonar, que se originam por diversos mecanismos, incluindo dilatação dos espaços aéreos distais por obstrução, necrose das paredes das vias aéreas e destruição do parênquima. Essas doenças apresentam evolução variável. A TCAR é fundamental na avaliação dessas doenças uma vez que permite uma melhor caracterização dos cistos pulmonares, incluindo sua distribuição, tamanho, extensão e regularidade das paredes, assim como a determinação de outras lesões pulmonares e extrapulmonares associadas. Frequentemente a TCAR é suficiente para a definição etiológica dos cistos pulmonares difusos, associada a achados clínicos e laboratoriais, sem a necessidade de realização de biópsia pulmonar. O diagnóstico diferencial das doenças pulmonares císticas difusas é extenso, incluindo etiologias neoplásicas, inflamatórias e infecciosas, sendo as mais frequentes determinantes desse padrão tomográfico a histiocitose pulmonar de células de Langerhans, a linfangioleiomiomatose, a pneumonia intersticial linfocitária e a bronquiolite folicular. Novas etiologias foram incluídas como potenciais determinantes desse padrão.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Pneumopatias/complicações , Diagnóstico Diferencial , Pneumopatias/classificação , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: To evaluate serum levels of C-reactive protein and erythrocyte sedimentation rates in patients with untreated newly diagnosed dermatomyositis or polymyositis and their correlation with clinical and laboratory parameters. METHODS: A cross-sectional study including 48 consecutive patients with untreated newly diagnosed dermatomyositis and polymyositis reviewed between 2002 and 2015 was conducted. Fifty healthy subjects were enrolled as controls. RESULTS: Patients with dermatomyositis and polymyositis had higher levels of C-reactive protein and erythrocyte sedimentation rate than healthy controls, but these values were not associated with clinical or laboratory parameters of disease activity either for dermatomyositis or for polymyositis. Additionally, erythrocyte sedimentation rate values correlated with pulmonary involvement as evidenced through computer tomography imaging (OR 1.15; 95%CI 1.01-1.31) only in patients with polymyositis. CONCLUSIONS: Although elevated, C-reactive protein and erythrocyte sedimentation rate are not sensitive parameters for measuring clinical and laboratory activity of dermatomyositis nor for polymiositis. However, erythrocyte sedimentation rate may be a valid parameter for screening pulmonary involvement, particularly in patients with polymyositis.
OBJETIVOS: Avaliar os níveis séricos da proteína C reativa (PCR) e da velocidade de hemossedimentação (VHS) em pacientes recém-diagnosticados com dermatomiosite (DM) e polimiosite (PM), sem tratamento prévio, correlacionando-os com parâmetros clínico-laboratoriais. MÉTODOS: Estudo transversal que incluiu 48 pacientes consecutivos com DM e PM (critérios de Bohan e Peter) recém-diagnosticados, sem tratamento medicamentoso, no período de 2002 a 2015. Foram incluídos 50 indivíduos saudáveis como grupo controle. RESULTADOS: Os pacientes apresentaram níveis mais elevados de VHS e PCR comparativamente aos controles saudáveis. Estes valores, porém, não se correlacionaram com os parâmetros clínicos e laboratoriais da atividade da doença (DM e PM). Somente em pacientes com PM a VHS apresentou relação com acometimento pulmonar na tomografia computadorizada [OR 1,15 (IC 95% 1,01-1,31)]. CONCLUSÕES: Apesar de aumentadas, a PCR e a VHS não são parâmetros sensíveis para a mensuração da atividade clínica e laboratorial de DM e PM., No entanto, a VHS pode ter validade no rastreio do acometimento pulmonar, particularmente em pacientes com PM.
Assuntos
Humanos , Proteína C-Reativa/análise , Polimiosite , Dermatomiosite , Sedimentação Sanguínea , Estudos Transversais , Pneumopatias/etiologiaRESUMO
La osificación pulmonar es un hallazgo poco usual, generalmente asintomático, que se reporta como incidental en biopsias de pulmón. Asimismo, la hemosiderosis pulmonar idiopática es una causa poco frecuente de infiltración pulmonar. Se presenta el caso de un hombre de 64 años con síntomas respiratorios crónicos, en quien se detectaron estas dos condiciones en el estudio histopatológico.
Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Ossificação Heterotópica/patologia , Hemossiderose/patologia , Pneumopatias/patologia , Toracoscopia , Cintilografia , Tomografia Computadorizada por Raios X , Ossificação Heterotópica/etiologia , Ossificação Heterotópica/diagnóstico por imagem , Hemossiderose/complicações , Pneumopatias/etiologia , Pneumopatias/diagnóstico por imagem , Hipóxia/etiologiaRESUMO
La púrpura de Schonlein-Henoch (PSH) es la vasculitis más frecuente en los niños. Los procesos vasculíticos pueden afectar el pulmón. Si bien la hemorragia alveolar difusa puede considerarse una de las manifestaciones de la PSH, no es un cuadro frecuente. En este artículo presentamos el caso de una niña de 10 años con nefritis por PSH que sufrió hemorragia pulmonar. La paciente recibió un tratamiento satisfactorio con metilprednisolona intravenosa. La revisión de las publicaciones reveló que la edad temprana puede influir de manera positiva en el pronóstico, y que los inmunosupresores y el tratamiento complementario son fundamentales.
Henoch-Schonlein purpura (HSP) is the most common vasculitis in children. Vasculitic processes can involve the lung. Although diffuse alveolar hemorrhage may be seen as one of the manifestation of HSP, it is not a frequent presentation. Here we reported the case of a 10-year-old girl with HSP nephritis who developed pulmonary hemorrhage. The patient was treated successfully with intravenous methylprednisolone. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment.
Assuntos
Humanos , Feminino , Criança , Vasculite por IgA/complicações , Hemorragia/etiologia , Pneumopatias/etiologiaRESUMO
AbstractObjective: To determine whether simple diagnostic methods can yield relevant disease information in patients with rheumatoid arthritis (RA).Methods: Patients with RA were randomly selected for inclusion in a cross-sectional study involving clinical evaluation of pulmonary function, including pulse oximetry (determination of SpO2, at rest), chest X-ray, and spirometry.Results: A total of 246 RA patients underwent complete assessments. Half of the patients in our sample reported a history of smoking. Spirometry was abnormal in 30% of the patients; the chest X-ray was abnormal in 45%; and the SpO2 was abnormal in 13%. Normal chest X-ray, spirometry, and SpO2 were observed simultaneously in only 41% of the RA patients. A history of smoking was associated with abnormal spirometry findings, including evidence of obstructive or restrictive lung disease, and with abnormal chest X-ray findings, as well as with an interstitial pattern on the chest X-ray. Comparing the patients in whom all test results were normal (n = 101) with those in whom abnormal test results were obtained (n = 145), we found a statistically significant difference between the two groups, in terms of age and smoking status. Notably, there were signs of airway disease in nearly half of the patients with minimal or no history of tobacco smoke exposure.Conclusions: Pulmonary involvement in RA can be identified through the use of a combination of diagnostic methods that are simple, safe, and inexpensive. Our results lead us to suggest that RA patients with signs of lung involvement should be screened for lung abnormalities, even if presenting with no respiratory symptoms.
ResumoObjetivo: Determinar se métodos diagnósticos de baixa complexidade podem fornecer informações relevantes sobre doença pulmonar em pacientes com artrite reumatoide (AR).Métodos: Pacientes com AR foram selecionados aleatoriamente para um estudo transversal envolvendo avaliação clínica pulmonar, oximetria de pulso (SpO2) em repouso, radiografia de tórax e espirometria.Resultados: Um total de 246 pacientes foi submetido à avaliação completa. Metade dos pacientes na amostra relatou história de tabagismo. A proporção de pacientes com resultados anormais na espirometria, radiografia de tórax e SpO2 foi de, respectivamente, 30%, 45% e 13%. Resultados normais em radiografia de tórax, espirometria e SpO2 foram observados simultaneamente em apenas 41% dos pacientes com AR. História de tabagismo foi associada a achados espirométricos anormais, de doença pulmonar obstrutiva e de doença pulmonar restritiva, assim como radiografia de tórax anormal e com padrão intersticial. Na comparação dos pacientes com exames normais (n = 101) com aqueles com exames com alguma alteração (n = 145), houve uma diferença estatisticamente significante entre os dois grupos em relação a idade e história de tabagismo. Interessantemente, sinais de doença de vias aéreas foram observados em quase metade dos pacientes com relato de baixa exposição ao tabagismo ou de nunca ter sido fumante.Conclusões: O comprometimento pulmonar na AR pode ser identificado através de uma combinação de métodos diagnósticos simples, seguros e de baixo custo. Nossos resultados sugerem que pacientes com AR e sinais de acometimento pulmonar devem ser avaliados quanto a possíveis anormalidades pulmonares, mesmo na ausência de sintomas respiratórios.
Assuntos
Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Artrite Reumatoide/complicações , Pneumopatias/etiologia , Pneumopatias , Fatores Etários , Estudos Transversais , Dispneia/etiologia , Dispneia , Pneumopatias/epidemiologia , Oximetria , Prevalência , Testes de Função Respiratória/métodos , Fatores Sexuais , Espirometria , Fumar/epidemiologiaRESUMO
Objetivo: rever a fisiopatologia da doença pulmonar e analisar sua correspondência com a avaliação radiográfica do tórax com base em escores na fibrose cística. Métodos: o levantamento bibliográfico foi realizado a partir das informações disponibilizadas pelas bases de dados Medline, Highwire, LILACS e por pesquisa direta, utilizando os termos Cystic Fibrosis, chest x-ray, lung disease. Resultados: para a elaboração desta revisão narrativa foram selecionados 25 referências bibliográficas abordando a fisiopatologia da doença pulmonar, os fatores que podem influenciar a evolução da fibrose cística e os escores radiográficos utilizados para avaliação do estágio de gravidade da lesão pulmonar. Os escores permitem quantificar, objetivamente, os danos pulmonares detectados na radiografia de tórax. Os artigos originais das descrições dos escores demonstraram de forma individualizada os objetivos de cada um deles, ressaltando sua utilidade e limitações. Conclusão: o conhecimento da fisiopatologia da fibrose cística é fundamental para a compreensão dos escores radiográficos e, principalmente, para o conhecimento de suas limitações. Não há um escore ideal e perfeito, portanto, é necessário conhecer a fisiopatologia da doença e os escores existentes, para então optar pelo escore ou escores a serem utilizados em diferentes situações clínicas.
Objective: To review the pathophysiology of pulmonary disease with emphasis on radiographic evaluation of the chest through scores in cystic fibrosis. Methods: The literature review was performed from the information provided by Medline, Highwire, LILACS and direct search using the Cystic Fibrosis terms, chest x-ray, lung disease. Results: A total of 25 references in the no systematically review addressing the pathophysiology of pulmonary disease, the factors that can influence the course of cystic fibrosis and radiographic scores used to stage the assessment of severity of lung injury. The creation of scores enabled, objectively quantify the lung damage shown on chest radiography. Original articles descriptionsof scores showed individually the objectives of each one of them, pointing their values and their failures. Conclusions: The pathophysiology of cystic fibrosis is critical for the comprehension of radiographic scores, and especially to the knowledge of scoring limitations. There is not a perfect score, so it is necessary to know the pathophysiology of the disease and scores to choose the aproppriate score or scores in defferent clinical situations.