ABSTRACT
El síndrome de Swyer-James-MacLeod (SSJM) ocurre como el resultado de una bronquio-litis obliterante en la niñez. Generalmente, este desorden es diagnosticado en niños, después de evaluar infecciones respiratorias recurrentes. Una de las razones para explicar la dificultad en el diagnóstico es que cuando los pacientes desarrollan pequeñas bronquiecta-sias y pocos síntomas no es fácil reconocer sino hasta la edad adulta. Existen pocas referencias de la enfermedad en niños, por lo que consideramos de interés su descripción. Se presenta el caso de un paciente de 4 años con síntomas respiratorios de neumonía aguda y se señalan los aspectos diagnósticos de mayor relevancia clínica actual.
Swyer-James-MacLeod Syndrome (SJMS) occurs as a result of childhood bronchiolitis obliterans. Typically, this disorder is diagnosed in childhood after evaluations for recu-rrent respiratory infections. One of the reasons to explain the difficulty in diagnosis is that when patients develop little bronchiectasis, and therefore, few symptoms, then this syndrome may not be recognized until adulthood. There are few references of the disease in children sowe think your description is interes-ting, we present the case of a 4 year old patient with acute respiratory symptoms of pneumonia, high lighting the most important aspects of clinical diagnosis today.
ABSTRACT
Swyer-James syndrome (SJS) is characterized by a small- or normal-sized unilateral hyperlucent lung with decreased vascularity and air trapping on plain radiographs. SJS is considered to be a postinfectious form of bronchiolitis obliterans and has occurred following childhood pulmonary infection by adenovirus, measles, influenza virus, Bordetella pertussis, Mycobacterium tuberculosis or Mycoplasma pneumoniae. The standard treatment modality for SJS is supportive care. Despite the prominent role of inflammation in the pathogenesis of SJS, the use of corticosteroids has remained controversial. We report herein a case of SJS which was successfully treated with monthly methylprednisolone pulse therapy, resulting in complete remission as verified by high resolution computed tomography.
Subject(s)
Adenoviridae , Adrenal Cortex Hormones , Bordetella pertussis , Bronchiolitis Obliterans , Inflammation , Lung, Hyperlucent , Measles , Methylprednisolone , Mycobacterium tuberculosis , Mycoplasma pneumoniae , Orthomyxoviridae , Pneumonia, MycoplasmaABSTRACT
PURPOSE: Swyer-James syndrome is an uncommon abnormality characterized radiographically by a hyperluncent lobe or lung and functionally by normal or reduced volume during inspiration and air trapping during expiration. The condition typically follows certain respiratory infections in infancy or early childhood. Thus, it is a postinfectious form of bronchiolitis obliterans. Most patients have chronic cough, sputum, and abnormal breathing sounds, and present with repeated pulmonary infections. The syndrome affects pulmonary functions and may cause anatomical changes such as bronchiectasis and emphysema. We reviewed cases of Swyer-James syndrome, in order to better understand the clinical manifestations and outcomes of the disease. METHODS: The diagnostic criteria were small or normal-sized unilateral hyperlucent lung with air-trapping during expiration based on chest X-ray or CT and history of severe lung infection. A total of 12 patients were found. Medical records, radiological studies, and other clinical test results were reviewed. RESULTS: Most of the patients had chronic respiratory symptoms and signs. All patients had pneumonia or bronchiolitis as initial insult. Microbial agents were determined in six patients, of whom three were adenovirus and the others, Mycoplasma pneumoniae. They had restrictive and obstructive patterns of pulmonary function with bronchial hyperresponsiveness for methacholine. Two patients underwent lobectomy for emphysema and bullous emphysema, respectively. CONCLUSION: In this study, Swyer-James syndrome is a severe chronic pulmonary disease, presenting with respiratory symptoms, and functional and anatomical changes including bronchiectasis and emphysema. Further rejearch is needed to investigate initial etiologic agents and pathogenesis; further research is also needed for the care of chronic respiratory problems.
Subject(s)
Humans , Adenoviridae , Bronchiectasis , Bronchiolitis , Bronchiolitis Obliterans , Cough , Emphysema , Lung , Lung Diseases , Lung, Hyperlucent , Medical Records , Methacholine Chloride , Mycoplasma pneumoniae , Pneumonia , Pneumonia, Mycoplasma , Respiratory Sounds , Respiratory Tract Infections , Sputum , ThoraxABSTRACT
The Swyer-James Syndrome is a acquired pulmonary disease following bronchiolitis obliterans in young aged children, in which characteristic unilateral hyperlucence radiologically. Since Swyer and James first reported a case in 1953, it also has been named unilateral hyperlucent lung syndrome or MacLeod syndrome. Frequently there are repeated pulmonary infections which cause bacterial pneumonia and brochiectasis. In cases of pulmonary symptoms, it is diagnosed at early childhood, but there will be delayed detection until later ages in asymptomatic ones unless occasional chest radiography. Altogether the syndrome begins at childhood and manifested decreased exercise tolerance and failure to thrive, which means important disorder in pediatrics. Presently we experienced Swyer-James syndrome of 4 years old boy who had productive cough for 1 month diagnosed with a plain chest X-ray, lung computerized tomography and isotope lung scanning.
Subject(s)
Child , Child, Preschool , Humans , Male , Bronchiolitis Obliterans , Cough , Exercise Tolerance , Failure to Thrive , Lung , Lung Diseases , Lung, Hyperlucent , Pediatrics , Pneumonia, Bacterial , Radiography , ThoraxABSTRACT
Objective To explore CT and HRCT manifestations of Swyer-James syndrome(SJS).Methods CT and HRCT were performed in 19 patients,3 of them underwent inspiratory and expiratory HRCT,1 patient surgically proved with bronchiolitis obliterans and bronchiectasis.Results CT demonstrated 37 lobes were involved appearing as hyperlucent and with decreased vascularity ,the volume of lobes decreased in 35(95%) ; CT showed 19 patients with brochiectasis , 2 patients with pulmonary tuberculosis , 6 patients with brochiolitis , and 3 with segmental atelectasis.Conclusion CT and HRCT are very useful for the diagnosis of SJS.
ABSTRACT
The Swyer-James syndrome is a relatively uncommon disease entity presented with unilateral hyperlucent lung due to hypoplasia of a pulmonary artery and bronchiectasis of the affected lung. The main finding is a hyperlucent lung with small hilar shadows on the chest X-ray. Pulmonary angiography is the standard method for diagnosis. We report a case of the Swyer-James syndrome with a brief review of literature.