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斯怀尔-詹姆斯-麦克劳德综合征——通过两名成年患者呈现出的罕见诊断病例。

Swyer-James-MacLeod syndrome-a rare diagnosis presented through two adult patients.

作者信息

Mehra Sumit, Basnayake Thilini, Falhammar Henrik, Heraganahally Subash, Tripathi Suryakant

机构信息

Department of Respiratory and Sleep Medicine Royal Darwin Hospital Darwin Australia.

Department of Pulmonary Medicine King George Medical University Lucknow India.

出版信息

Respirol Case Rep. 2017 Jun 16;5(5):e00245. doi: 10.1002/rcr2.245. eCollection 2017 Sep.

Abstract

Swyer-James-MacLeod syndrome (SJMS) is a rare syndrome of acute obliterative bronchiolitis following an early childhood infective insult to the lungs. This causes arrest of alveolarization, affecting lung development with hypoplasia of the ipsilateral pulmonary artery and results in a characteristic radiological pattern, such as a unilateral hyperlucent lung with expiratory air-trapping and pruned-tree appearance on pulmonary angiogram. The clinical presentation is either recurrent chest infections, exertional dyspnoea or it may be an incidental finding. Management involves early prevention of infection, airway clearance, and regular vaccinations. We describe two adult patients with SJMS: A 51-year-old female of Indian ethnicity presenting with recurrent haemoptysis and a 40-year-old Indigenous male presenting acutely with sepsis and background history of recurrent chest infections. These cases highlight the importance of being aware of and accurately recognizing this rare condition, to be able to manage patients appropriately and avoid incorrect and unnecessary treatment.

摘要

斯怀尔-詹姆斯-麦克劳德综合征(SJMS)是一种罕见的综合征,由儿童早期肺部感染性损伤后发生急性闭塞性细支气管炎引起。这会导致肺泡化停滞,影响肺部发育,伴有同侧肺动脉发育不全,并产生特征性的放射学表现,如单侧肺透亮度增加、呼气时气体潴留以及肺血管造影显示的“枯树状”外观。临床表现为反复的胸部感染、劳力性呼吸困难,也可能是偶然发现。治疗包括早期预防感染、气道清理和定期接种疫苗。我们描述了两名成年SJMS患者:一名51岁的印度裔女性,表现为反复咯血;一名40岁的原住民男性,急性发作败血症,有反复胸部感染的病史。这些病例凸显了认识并准确识别这种罕见疾病的重要性,以便能够恰当地治疗患者,避免不正确和不必要的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f5c/5473102/6c39f335b32d/RCR2-5-na-g001.jpg

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