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Background: The composite physiologic index (CPI) was developed to estimate the extent of interstitial lung disease (ILD) in idiopathic pulmonary fibrosis (IPF) patients based on pulmonary function tests (PFTs). The CALIPER-revised version of the CPI (CALIPER-CPI) was also developed to estimate the volume fraction of ILD measured by CALIPER, an automated quantitative CT postprocessing software. Recently, artificial intelligence-based quantitative CT image analysis software (AIQCT), which can be used to quantify the bronchial volume separately from the ILD volume, was developed and validated in IPF.

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Swyer-James-MacLeod syndrome (SJMS) also known as hyperlucent lung syndrome is a condition that occurs as a complication of infectious bronchiolitis obliterans. It is characterized by inflammation and fibrosis of the affected area of the lung resulting in ventilation and perfusion mismatch ultimately leading to underdevelopment of the affected lung. A key feature used for diagnosis is unilateral small lung with hyperlucency on a chest radiograph.

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Article Synopsis
  • Swyer-James-MacLeod syndrome (SJMS) is a rare lung disorder that leads to underdeveloped blood vessels in the lungs, often causing emphysema and sometimes bronchiectasis.
  • The exact cause is unclear, but SJMS is thought to stem from childhood respiratory infections like RSV and tuberculosis, with symptoms that are often vague and can delay diagnosis.
  • A report of a 23-year-old Kenyan woman illustrates the challenges of diagnosing SJMS given her lengthy clinical history of respiratory issues, emphasizing the need for careful evaluation and possibly advanced imaging to identify the condition.
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We present a rare finding on lung ventilation-perfusion (V/Q) scintigraphy for a woman with longstanding dyspnea. CT of the chest showed volume loss on the right side, which raised concern about possible bronchiolitis obliterans or Swyer-James-MacLeod syndrome; however, the right pulmonary artery could not be visualized. A subsequent V/Q scan showed absence of perfusion and decreased ventilation to the entire right lung, consistent with agenesis of the right pulmonary artery.

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Article Synopsis
  • Swyer-James-MacLeod syndrome makes one lung lobe look really empty on chest scans, and it is linked to other lung problems like air trapping and less blood flow.
  • If a child has a common heart issue called a ventricular septal defect and it isn't treated, it can lead to serious lung problems like Eisenmenger syndrome.
  • Identifying Swyer-James-MacLeod syndrome early is important for giving the right treatment to help patients with unusual lung conditions.
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