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Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients

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...

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Autores principales: Mehra, Sumit, Basnayake, Thilini, Falhammar, Henrik, Heraganahally, Subash, Tripathi, Suryakant
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5473102/
https://www.ncbi.nlm.nih.gov/pubmed/28638618
http://dx.doi.org/10.1002/rcr2.245
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author Mehra, Sumit
Basnayake, Thilini
Falhammar, Henrik
Heraganahally, Subash
Tripathi, Suryakant
author_facet Mehra, Sumit
Basnayake, Thilini
Falhammar, Henrik
Heraganahally, Subash
Tripathi, Suryakant
author_sort Mehra, Sumit
collection PubMed
description 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.
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spelling pubmed-54731022017-06-21 Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients Mehra, Sumit Basnayake, Thilini Falhammar, Henrik Heraganahally, Subash Tripathi, Suryakant Respirol Case Rep Case Reports 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. John Wiley & Sons, Ltd 2017-06-16 /pmc/articles/PMC5473102/ /pubmed/28638618 http://dx.doi.org/10.1002/rcr2.245 Text en © 2017 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs (http://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Case Reports
Mehra, Sumit
Basnayake, Thilini
Falhammar, Henrik
Heraganahally, Subash
Tripathi, Suryakant
Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients
title Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients
title_full Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients
title_fullStr Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients
title_full_unstemmed Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients
title_short Swyer–James–MacLeod syndrome—a rare diagnosis presented through two adult patients
title_sort swyer–james–macleod syndrome—a rare diagnosis presented through two adult patients
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5473102/
https://www.ncbi.nlm.nih.gov/pubmed/28638618
http://dx.doi.org/10.1002/rcr2.245
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