Swyer-James 증후군의 임상적 고찰
Clinical Features of Swyer-James Syndrome
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참고문헌
초록
목 적 : Swyer-James 증후군은 영아기 및 소아기의 호흡기 감염에 의한 폐쇄성 세기관지염의 한 형태이며 단순 흉부 촬영에서 한쪽 폐의 방사선 과투과도를 특징으로 한다. Swyer-James증후군은 우연히 발견된 경우도 있으나 많은 환자들에서 지속적인 기침, 가래, 청진상의 이상음 및 반복적인 호흡기 감염 등을 일으킨다. 또한 폐기능 저하와 수술적 치료가 필요한 기관지확장증과 폐기종을 동반하는 중증의 만성 폐질환으로 진행하기도 한다. 이에 저자들은 Swyer-James 증후군의 원인과 경과의 임상적 특징들을 고찰하여 보고자 한다. 방 법 : 폐감염 이후 지속적인 증상을 갖는 환자들을 대상으로 단순 흉부 촬영과 고해상 흉부 전산화단층촬영을 통해 용적증가 없는 편측폐의 방사선 과투과와 공기저류의 소견을 갖는 경우로 하였다. Swyer-James 증후군으로 진단된 12명의 환자들을 대상으로 병록지, 방사선학적 검사와 시행된 임상 검사들을 후향적으로 검토하였다. 결 과 : 환자들은 심한 폐렴이나 모세기관지염의 이후에 지속적인 호흡기 증상과 이상 호흡음을 나타내었다. Adenovirus와 M. pneumoniae가 각각 3예에서 최초 감염 원인균으로 밝혀졌으며 이들 6명의 환자 중 3예에서 중복감염이 의심되었다. 환자들은 다양한 정도의 제한성 및 폐쇄성 폐기능 감소소견과 메타콜린에 기관지 과민성을 동반하고 있었다. 2명의 환자가 초기 감염 이후 발생한 폐기종 및 수포성기종으로 폐엽절제술을 받았다.
Abstract
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.
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