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1.
Ann Vasc Surg ; 72: 666.e23-666.e32, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33333194

RESUMO

Saccular superior vena cava aneurysms (SVCAs) are one of the rarest causes of mediastinal masses. Seventy years after the first published case report, to the best of our knowledge, fewer than 30 cases have been described in the literature. This article provides an overview of the peculiarities regarding diagnosis and treatment performed in these cases and addresses the protocol adopted in our case report. We present a saccular aneurysm resected through median sternotomy, under cardiopulmonary bypass and superior vena cava reconstruction using a bovine pericardial patch.


Assuntos
Aneurisma/cirurgia , Pericárdio/transplante , Procedimentos de Cirurgia Plástica , Procedimentos Cirúrgicos Vasculares , Veia Cava Superior/cirurgia , Adolescente , Adulto , Idoso , Aneurisma/diagnóstico por imagem , Animais , Ponte Cardiopulmonar , Bovinos , Feminino , Xenoenxertos , Humanos , Masculino , Pessoa de Meia-Idade , Esternotomia , Resultado do Tratamento , Veia Cava Superior/diagnóstico por imagem , Adulto Jovem
2.
Lung ; 195(6): 769-774, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-29032479

RESUMO

PURPOSES: Considering that pulmonary arterial obstruction decreases venous flow, we hypothesized that filling defects in pulmonary veins can be identified in areas adjacent to pulmonary embolism (PE). This sign was named the "pulmonary vein sign" (PVS), and we evaluated its prevalence and performance for PE diagnosis in computed tomography pulmonary angiography (CTPA). METHODS: This retrospective study enrolled consecutive patients with clinical suspicion of PE who underwent CTPA scan. The PVS was defined by the following criteria: (a) presence of a homogeneous filling defect of at least 2 cm in a pulmonary vein; (b) attenuation of the left atrium > 160 Hounsfield units. Using the cases that presented PE on CTPA as reference, sensitivity, specificity, and positive and negative predictive values were calculated for PVS. RESULTS: In total, 119 patients (73 female; mean age, 62 years) were included in this study. PE was diagnosed in 44 (35.8%) patients. The PVS was present in 16 out of 44 patients with PE. Sensitivity was 36.36% (95% confidence interval (CI) 22.83-52.26%); specificity, 98.67% (95% CI 91.79-99.93%); positive predictive value, 94.12% (95% CI 69.24-99.69%); negative predictive value, 72.55% (95% CI 62.67-80.70%). The Kappa index for the PVS was good (0.801; 95% CI 0.645-0.957). PVS was correlated with lobar and segmental pulmonary embolism (p < 0.01). CONCLUSION: Despite a low sensitivity, presence of the pulmonary vein sign was highly specific for PE, with a good agreement between readers. This sign could contribute for PE diagnosis on CTPA studies.


Assuntos
Angiografia por Tomografia Computadorizada , Embolia Pulmonar/diagnóstico por imagem , Veias Pulmonares/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Átrios do Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Estudos Retrospectivos , Adulto Jovem
3.
Lung ; 195(4): 389-395, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28612239

RESUMO

Dengue fever is an arboviral disease transmitted to humans through the bites of infected female Aedes mosquitoes. Dengue virus is a member of the Flaviviridae family, and human infection can be caused by any of the four antigenically distinct serotypes (DENV 1-4). The infection has become recognized as the most important and prevalent arboviral disease in humans, endemic in almost 100 countries worldwide. Nearly 3 billion people live in areas with transmission risk. Autochthonous transmission of the virus in previously disease-free areas, increased incidence in endemic areas, and epidemic resurgence in controlled regions could increase the risk of contracting more severe forms of the disease, such as dengue hemorrhagic fever (DHF)/dengue shock syndrome (DSS). Symptomatic dengue virus infection can present with a wide range of clinical manifestations, from mild fever to life-threatening DSS. Thoracic complications may manifest as pleural effusion, pneumonitis, non-cardiogenic pulmonary edema, and hemorrhage/hemoptysis. No vaccine is currently available and no specific treatment for dengue fever exists, but prevention and prompt management of complications in patients with DHF can help reduce mortality. This review describes the main clinical, pathological, and imaging findings of thoracic involvement in DHF.


Assuntos
Aedes/virologia , Vírus da Dengue/patogenicidade , Hemoptise/virologia , Pulmão/virologia , Dengue Grave/virologia , Animais , Biópsia , Vacinas contra Dengue/uso terapêutico , Diagnóstico Diferencial , Hemoptise/diagnóstico , Hemoptise/mortalidade , Hemoptise/terapia , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Dengue Grave/diagnóstico , Dengue Grave/mortalidade , Dengue Grave/terapia , Tomografia Computadorizada por Raios X
4.
Lung ; 194(4): 501-9, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27300447

RESUMO

The aim of this review was to present the main aspects of pleural diseases seen with conventional and advanced magnetic resonance imaging (MRI) techniques. This modality is considered to be the gold standard for the evaluation of the pleural interface, characterization of complex pleural effusion, and identification of exudate and hemorrhage, as well as in the analysis of superior sulcus tumors, as it enables more accurate staging. The indication for MRI of the thorax in the identification of these conditions is increasing in comparison to computerized tomography, and it can also be used to support the diagnosis of pulmonary illnesses. This literature review describes the morphological and functional aspects of the main benign and malignant pleural diseases assessed with MRI, including mesothelioma, metastasis, lymphoma, fibroma, lipoma, endometriosis, asbestos-related pleural disease, empyema, textiloma, and splenosis.


Assuntos
Fibroma/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Mesotelioma/diagnóstico por imagem , Neoplasias Pleurais/diagnóstico por imagem , Empiema/diagnóstico por imagem , Endometriose/diagnóstico por imagem , Feminino , Corpos Estranhos/diagnóstico por imagem , Humanos , Linfoma/diagnóstico por imagem , Masculino , Derrame Pleural/diagnóstico por imagem , Esplenose/diagnóstico por imagem , Tórax/diagnóstico por imagem
5.
Lung ; 193(5): 619-27, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26104489

RESUMO

Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous disease with high phenotypic variability. The incidence is approximately one in 5000-10,000 births. TSC is characterized by widespread hamartomas and benign or rarely malignant neoplasms affecting various organs, most commonly the brain, skin, retinas, kidneys, heart, and lungs. The wide range of organs affected reflects the roles of TSC1 and TSC2 genes in the regulation of cell proliferation and differentiation. Clinical diagnostic criteria are important because genetic testing does not identify the mutation in up to 25% of patients. Imaging is pivotal, as it allows a presumptive diagnosis of TSC and definition of the extent of the disease. Common manifestations of TSC include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis (LAM), renal angiomyolipoma, and skin lesions. Pulmonary involvement consists of LAM and, less commonly, multifocal micronodular pneumocyte hyperplasia (MMPH), which causes cystic and nodular diseases, respectively. Recent reports indicate that pulmonary LAM is found by computed tomography in up to 35% of the female patients with TSC. MMPH is rare and may be associated with LAM or, less frequently, occurs as an isolated pulmonary manifestation in women with TSC. Dyspnea and pneumothorax are common clinical presentations of LAM, whereas MMPH is usually asymptomatic. The aim of this review is to describe the main clinical, imaging, and pathological aspects of TSC, with a focus on pulmonary involvement.


Assuntos
Angiomiolipoma/etiologia , Carcinoma de Células Renais/etiologia , Neoplasias Renais/etiologia , Neoplasias Pulmonares/etiologia , Pulmão/patologia , Linfangioleiomiomatose/etiologia , Doenças do Sistema Nervoso/etiologia , Esclerose Tuberosa/complicações , Cistos/etiologia , Humanos , Hiperplasia/epidemiologia , Hiperplasia/patologia , Neoplasias Pulmonares/patologia , Linfangioleiomiomatose/patologia , Dermatopatias/etiologia
6.
Lung ; 192(2): 225-33, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24429586

RESUMO

Cocaine is the most commonly used illicit drug among patients presenting at hospital emergency departments and the most frequent cause of drug-related deaths reported by medical examiners. Various respiratory problems temporally associated with cocaine use have been reported. Acute and chronic uses also are responsible for lung complications, such as pulmonary edema, alveolar hemorrhage, pulmonary hypertension, organizing pneumonia, emphysema, barotrauma, infection, cancer, eosinophilic disease, and aspiration pneumonia. Although most imaging findings are nonspecific, they may raise suspicion of a cocaine-related etiology when considered together with patients' profiles and medical histories. This literature review describes cocaine-induced diseases with pulmonary involvement, with an emphasis on high-resolution chest computed tomographic findings and patterns.


Assuntos
Transtornos Relacionados ao Uso de Cocaína/complicações , Pneumopatias/induzido quimicamente , Pneumopatias/diagnóstico por imagem , Pulmão/efeitos dos fármacos , Pulmão/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto , Feminino , Humanos , Pneumopatias/terapia , Masculino , Valor Preditivo dos Testes , Prognóstico , Adulto Jovem
7.
AJR Am J Roentgenol ; 199(3): W331-4, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22915423

RESUMO

OBJECTIVE: The aim of this study was to show the usefulness of chemical-shift MRI in the diagnosis of intranodular fat in seven patients with pulmonary hamartomas and indeterminate CT findings. CONCLUSION: In the setting of chemical-shift MRI, the average nodule signal intensity index of pulmonary hamartomas was 45.3% (SD = 25.5%). The correlation between average nodule signal intensity and CT attenuation in Hounsfield units was -0.94. Chemical-shift MRI could be an important tool for the detection of fat in pulmonary hamartomas with inconclusive CT findings.


Assuntos
Tecido Adiposo/patologia , Hamartoma/diagnóstico , Pneumopatias/diagnóstico , Imageamento por Ressonância Magnética , Idoso , Hamartoma/diagnóstico por imagem , Hamartoma/patologia , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
8.
AJR Am J Roentgenol ; 197(1): W69-75, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21700998

RESUMO

OBJECTIVE: The purpose of this article is to describe diseases that may present with the reversed halo sign on high-resolution CT. We emphasize the tomographic features most frequently associated with this sign and correlate them with histologic findings. CONCLUSION: A wide spectrum of infectious and noninfectious diseases may present with the reversed halo sign on chest CT. The nonspecific nature of this sign should not cloud an otherwise fairly straightforward diagnosis, especially when associated background findings are typical. Although a rigorous analysis of associated CT findings may help with the differential diagnosis, histologic assessment is often needed for a definitive determination of the cause.


Assuntos
Pneumopatias/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
9.
Pediatr Cardiol ; 32(1): 108-10, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20978753

RESUMO

Unilateral pulmonary vein atresia is a rare congenital heart disease. Its symptoms begin to manifest in childhood and may be similar to those of other left-side heart obstructions. The diagnosis of this disorder is difficult and usually requires several imaging methods. This report presents the case of a 7-year-old girl whose diagnosis was aided through the use of multislice computed tomography.


Assuntos
Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Criança , Feminino , Humanos , Tomografia Computadorizada por Raios X
10.
Eur J Cardiothorac Surg ; 59(1): 276-277, 2021 01 04.
Artigo em Inglês | MEDLINE | ID: mdl-32840317

RESUMO

Superior vena cava aneurysms are rare mediastinal vascular lesions. Aneurysms are classified as fusiform and saccular, with the latter being rarer than the former. We report the case of an asymptomatic 75-year-old woman who presented with a superior mediastinal mass. She underwent chest computed tomography angiography, which demonstrated a saccular aneurysm in the superior vena cava.


Assuntos
Aneurisma , Doenças do Mediastino , Idoso , Aneurisma/diagnóstico por imagem , Aneurisma/cirurgia , Feminino , Humanos , Mediastino/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Veia Cava Superior/diagnóstico por imagem
11.
Radiol Bras ; 54(6): 375-380, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34866697

RESUMO

OBJECTIVE: The aim of this study was to evaluate chest computed tomography (CT) findings in patients diagnosed with neurofibromatosis type 1 (NF1). MATERIAL AND METHODS: This was a retrospective study in which we reviewed the chest CT scans of 14 patients diagnosed with NF1 and neurofibromatosis-associated diffuse lung disease (NF-DLD). The sample comprised eight women and six men. The median age was 55 years (range, 11-75 years). The diagnosis of NF1 was made on the basis of the diagnostic criteria established by the U.S. National Institutes of Health. The images were analyzed by two chest radiologists, who reached decisions by consensus. RESULTS: The predominant CT finding of NF-DLD was multiple cysts, which were observed in 13 patients (92.9%), followed by emphysema, in eight (57.1%) and subpleural bullae, in six (42.9%). Other findings included subcutaneous neurofibromas, in 12 patients (85.7%), ground-glass opacities, in one (7.1%), and tracheobronchial neurofibromas, in one (7.1%). The pulmonary abnormalities were bilateral in 12 cases (85.7%). The abnormalities were predominantly in the upper lung fields in eight cases (57.1%), and their distribution was random in 11 (78.6%). CONCLUSION: Pulmonary cysts, emphysema, and subpleural bullae appear to be the chest CT findings that are most characteristic of NF-DLD.


OBJETIVO: O objetivo deste estudo foi avaliar os achados pulmonares na tomografia computadorizada do tórax de pacientes diagnosticados com neurofibromatose tipo 1 (NF1). MATERIAIS E MÉTODOS: Foram revisados, retrospectivamente, os achados tomográficos de 14 pacientes com doença pulmonar difusa associada à NF1 (NF-DPD). A amostra incluiu oito mulheres e seis homens, com idade entre 11 e 75 anos (mediana de idade de 55 anos). O diagnóstico foi estabelecido com base em critérios diagnósticos predeterminados pelo National Institutes of Health dos Estados Unidos. As imagens foram analisadas de forma independente por dois radiologistas, que chegaram a um consenso. RESULTADOS: Os achados tomográficos predominantes foram múltiplos cistos em 13 pacientes (92,9%), enfisema em oito (57,1%) e bolhas subpleurais em seis (42,9%). Achados associados incluíram neurofibromas cutâneos e subcutâneos em 12 pacientes (85,7%), opacidades em vidro fosco em um (7,1%) e neurofibromas traqueobrônquicos em um (7,1%). As anormalidades pulmonares foram bilaterais em 12 casos (85,7%). Houve predomínio nos terços superiores em oito (57,1%) pacientes e se distribuíram randomicamente pelos pulmões em 11 (78,6%). CONCLUSÃO: Os achados tomográficos pulmonares mais frequentes na NF-DPD foram os cistos pulmonares, o enfisema e as bolhas subpleurais.

12.
Radiol Bras ; 54(1): 9-14, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33574627

RESUMO

OBJECTIVE: To evaluate computed tomography (CT) findings in 23 patients with bronchial atresia. MATERIALS AND METHODS: The CT images were reviewed by two radiologists who reached decisions by consensus. We included only patients who presented with abnormalities on CT and in whom the diagnosis had been confirmed by pathological examination of the surgical specimen (if the lesion was resected). The CT scans were assessed in order to identify the main findings and to map the distribution of the lesions (i.e., to determine whether the pulmonary involvement was unilateral or bilateral). RESULTS: The main CT finding was the combination of bronchocele and hyperinflation of the distal lung. That combination was observed in all of the patients. The lesions were unilateral in all 23 cases, being seen predominantly in the left upper lobe, followed by the right lower lobe, right upper lobe, middle lobe, and left lower lobe. CONCLUSION: The diagnosis of bronchial atresia can be reliably made on the basis of a finding of bronchocele accompanied by hyperinflation of the adjacent lung parenchyma.


OBJETIVO: Analisar os achados na tomografia computadorizada (TC) de tórax de 23 pacientes com atresia brônquica. MATERIAIS E MÉTODOS: As imagens de TC foram avaliadas por dois observadores e os casos discordantes foram resolvidos por consenso. Os critérios de inclusão foram a presença de anormalidades na TC compatíveis com atresia brônquica e/ou diagnóstico confirmado por exame anatomopatológico das peças cirúrgicas para os pacientes submetidos a ressecção cirúrgica. As TCs foram avaliadas quanto aos principais achados de imagem, à distribuição das lesões, ao envolvimento pulmonar unilateral ou bilateral. RESULTADOS: Os principais achados na TC foram a presença de broncocele, hiperinsuflação do parênquima pulmonar ou ambos. A combinação desses achados foi encontrada em todos os pacientes. Em relação à distribuição, o envolvimento foi unilateral nos 23 casos. Quando se consideraram os lobos mais acometidos, o lobo superior esquerdo foi o mais acometido, seguido do lobo inferior direito, lobo superior direito, lobo médio e lobo inferior esquerdo. CONCLUSÃO: O diagnóstico de atresia brônquica pode ser feito em presença de broncocele associada com hiperinsuflação do parênquima pulmonar adjacente.

13.
J Bras Pneumol ; 47(5): e20200595, 2021.
Artigo em Inglês, Português | MEDLINE | ID: mdl-34669832

RESUMO

Effective communication among members of medical teams is an important factor for early and appropriate diagnosis. The terminology used in radiology reports appears in this context as an important link between radiologists and other members of the medical team. Therefore, heterogeneity in the use of terms in reports is an important but little discussed issue. This article is the result of an extensive review of nomenclature in thoracic radiology, including for the first time terms used in X-rays, CT, and MRI, conducted by radiologists from Brazil and Portugal. The objective of this review of medical terminology was to create a standardized language for medical professionals and multidisciplinary teams.


Assuntos
Idioma , Radiologia , Brasil , Consenso , Humanos , Portugal
14.
Radiol Bras ; 53(5): 287-292, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33071371

RESUMO

OBJECTIVE: To analyze the computed tomography (CT) findings of lymphocytic interstitial pneumonia (LIP). MATERIALS AND METHODS: We retrospectively reviewed the clinical and CT findings of 36 patients with LIP, including 25 women and 11 men, with a mean age of 52.5 years (age range, 22-78 years). RESULTS: The main associated diseases with LIP were Sjögren syndrome (42%), human immunodeficiency virus infection (17%), amyloidosis (17%), Sjögren syndrome associated with secondary amyloidosis (11%), idiopathic (8%), and systemic lupus erythematosus (5%). The predominant CT abnormalities were multiple cystic airspaces (n = 35), small nodules (n = 15), ground-glass opacities (n = 13), bronchiectasis and/or bronchiolectasis (n = 8), and thickening of the bronchovascular bundles (n = 8). Other CT findings included reticular opacities (n = 7), calcified nodules (n = 4), airspace consolidation (n = 4), emphysema (n = 3), honeycombing (n = 3), lymph node enlargement (n = 2), mosaic attenuation pattern (n = 1), and cavitated nodules (n = 1). CONCLUSION: The main CT findings of LIP were multiple cysts, small nodules, and ground-glass opacities.


OBJETIVO: Analisar os achados da tomografia computadorizada da pneumonia intersticial linfocítica. MATERIAIS E MÉTODOS: Trinta e seis pacientes com diagnóstico de pneumonia intersticial linfocítica (25 mulheres, 11 homens; faixa etária de 22 a 78 anos; idade média de 52,5 anos) tiveram os achados clínicos e tomográficos revisados retrospectivamente. RESULTADOS: As principais doenças associadas à pneumonia intersticial linfocítica foram síndrome de Sjögren (42%), infecção pelo HIV (17%), amiloidose (17%), síndrome de Sjögren associada a amiloidose secundária (11%), síndrome de Sjögren idiopática (8%) e lúpus eritematoso sistêmico (5%). As alterações predominantes na TC consistiram em múltiplos espaços aéreos císticos (n = 35), pequenos nódulos (n = 15), opacidades em vidro fosco (n = 13), bronquiectasias e bronquiolectasias (n = 8) e espessamento de feixes broncovasculares (n = 8). Outros achados da TC incluíram opacidades reticulares (n = 7), nódulos calcificados (n = 4), consolidação do espaço aéreo (n = 4), enfisema (n = 3), faveolamento (n = 3), linfonodomegalia linfática (n = 2), padrão de atenuação em mosaico (n = 1) e nódulos escavados (n = 1). CONCLUSÃO: Os principais achados tomográficos observados em pacientes com pneumonia intersticial linfocítica foram múltiplos cistos, pequenos nódulos e opacidades em vidro fosco.

17.
Arch Bronconeumol (Engl Ed) ; 55(6): 297-305, 2019 Jun.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30527558

RESUMO

OBJECTIVE: To assess the impact of patient-, lesion- and procedure-related factors on the risks of complications and final diagnosis of malignancy in PCNB of mediastinal and lung lesions. MATERIAL AND METHODS: We studied a large single-center cohort of 235 consecutive patients (66.8% men; 58.5±18.0 years) with a range of thoracic benign and malignant lesions, who underwent PCNB performed along 24 months by a single experienced radiologist. Diagnostic accuracy analyses of PCNB for malignancy were performed, as well as estimations of relative risk and logistic regression models in order to assess possible associations between such factors and malignancy/complications. RESULTS: 155 lesions (65.9%) were diagnosed as malignant. Overall accuracy was 91.1%, with sensitivity of 87.1%, specificity of 98.8%, positive predictive value of 99.3%, and negative predictive value of 79.8%. Pneumothorax (49/235; 20.8%) and hemorrhage (37/235; 15.7%) were the most common complications. Emphysema, smoking, older age, intrapulmonary location, deeper location, smaller size, presence of cavitations and irregular contours of the lesions, and smaller needle-pleural angles were the most consistent factors related to the occurrence of complications. Emphysema, older age, smoking, solid and deeper lesions were also significantly associated with a final diagnosis of malignancy after PCNB. CONCLUSION: CT-guided PCNB of mediastinal and lung lesions is a safe procedure with high diagnostic accuracy for malignancy.


Assuntos
Biópsia com Agulha de Grande Calibre , Biópsia Guiada por Imagem , Neoplasias Pulmonares/patologia , Pulmão/patologia , Mediastino/patologia , Tomografia Computadorizada por Raios X , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Biópsia com Agulha de Grande Calibre/efeitos adversos , Biópsia com Agulha de Grande Calibre/métodos , Criança , Pré-Escolar , Enfisema/complicações , Feminino , Hemorragia/etiologia , Humanos , Biópsia Guiada por Imagem/efeitos adversos , Lactente , Pneumopatias/diagnóstico , Pneumopatias/patologia , Neoplasias Pulmonares/diagnóstico , Masculino , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Pessoa de Meia-Idade , Pneumotórax/etiologia , Valor Preditivo dos Testes , Risco , Fatores de Risco , Sensibilidade e Especificidade , Fumar/efeitos adversos , Adulto Jovem
19.
Respir Med ; 149: 9-15, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30885426

RESUMO

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen's disease, is an autosomal dominant dysplasia of the ectoderm and mesoderm with a variable clinical expression, but near-complete penetrance before the age of 5 years. The estimated incidence is 1 in 3000 births. NF-1 is characterized by collections of neurofibromas, café-au-lait spots, axillary and inguinal freckling, and pigmented hamartomas in the iris (Lisch nodules). Pulmonary manifestations of NF-1, which usually include bilateral basal reticulations and apical bullae and cysts, are reported in 10-20% of adult patients. Clinically, neurofibromatosis-associated diffuse lung disease (NF-DLD) usually presents with nonspecific respiratory symptoms, including dyspnea on exertion, shortness of breath, and chronic cough or chest pain, at the time of diagnosis. Computed tomography (CT) is highly accurate for the identification and characterization of NF-DLD; it is the most reliable method for the diagnosis of this lung involvement. Various CT findings of NF-DLD, including cysts, bullae, ground-glass opacities, bibasilar reticular opacities, and emphysema, have been described in patients with NF-1. The typical CT pattern, however, is characterized by upper-lobe cystic and bullous disease, and basilar interstitial lung disease. Currently, the goal of NF-DLD treatment is the earliest possible diagnosis, focusing on symptom relief and interventions that positively alter the course of the disease, such as smoking cessation. The aim of this review is to describe the main clinical, pathological, and imaging aspects of NF-1, with a focus on pulmonary involvement.


Assuntos
Doenças Pulmonares Intersticiais/etiologia , Pneumopatias/etiologia , Neurofibromatose 1/epidemiologia , Neurofibromatose 1/patologia , Acrilonitrila/análogos & derivados , Acrilonitrila/uso terapêutico , Idoso , Compostos de Anilina/uso terapêutico , Antineoplásicos/uso terapêutico , Benzimidazóis/uso terapêutico , Vesícula/patologia , Criança , Feminino , Aconselhamento Genético/métodos , Humanos , Hipertensão Pulmonar/etiologia , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Neurofibromatose 1/complicações , Neurofibromatose 1/terapia , Enfisema Pulmonar/etiologia , Tomografia Computadorizada por Raios X/métodos , Tretinoína/uso terapêutico , Adulto Jovem
20.
J Comput Assist Tomogr ; 32(5): 788-91, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18830113

RESUMO

OBJECTIVE: To evaluate the high-resolution computed tomographic (CT) findings of patients with tracheal paracoccidioidomycosis. METHODS: The high-resolution CT scans of 178 patients with pulmonary paracoccidioidomycosis were reviewed, and 4 cases of proven tracheal involvement were studied. There were 3 male and 1 female patients, with ages ranging from 44 to 62 years (average, 49.2 years). The CT scans were retrospectively analyzed with special attention to the location of airway lesions, patterns of wall thickening, and occurrence of abnormal adjacent lymph nodes. Images were reviewed by 2 radiologists who reached decisions by consensus. RESULTS: The CT findings were circumferential parietal thickening (n = 4) and irregular (n = 3) or smooth (n = 1), with submucosal nodules (n = 2). Enlarged mediastinal lymph nodes were seen in 1 patient. CONCLUSIONS: The CT findings in patients with tracheal paracoccidioidomycosis were circumferential irregular thickening of the tracheal wall, with submucosal nodules.


Assuntos
Paracoccidioidomicose/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Doenças da Traqueia/diagnóstico por imagem , Adulto , Feminino , Humanos , Pneumopatias Fúngicas/complicações , Masculino , Pessoa de Meia-Idade
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