Chest
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Alpha-1 antitrypsin (AAT) deficiency is a genetic disorder most commonly due to a single G to A point mutation (E342K), leading to debilitating lung and/or liver disorders and is associated with increased mortality. The E342K point mutation causes a conformational change of the AAT protein resulting in its retention in liver hepatocytes. This reduces AAT secretion into the serum resulting in higher protease activities due to the lack of inhibition from AAT, causing damage to healthy lung tissue. The current standard of care for lung manifestations involves weekly IV augmentation therapy and is considered suboptimal for these patients. Furthermore, there is currently no approved treatment for liver manifestations. The unmet medical need for patients with AAT deficiency remains high, and new treatment options are needed to treat the underlying disease etiology. ⋯ In this review, we will discuss the pathophysiology of AAT deficiency and emerging treatment options with particular focus on RNA editing as a disease-modifying treatment for both liver and lung disease.
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As interstitial lung abnormalities (ILAs) are increasingly recognized on imaging and in clinical practice, identification and appropriate management are critical. We propose an algorithmic approach to the identification and management of patients with ILAs. ⋯ We anticipate this algorithmic approach will aid clinicians in interpreting the radiologic pattern described as ILA within the clinical context of their patients.
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Pulmonary arteriovenous malformations (PAVMs) are direct connections between the pulmonary artery and vein, creating a right-to-left shunt (RLS). Embolization is indicated to prevent complications. Guidelines recommend follow-up chest CT scans to confirm persistent occlusion and embolization of all treatable PAVMs. Graded transthoracic contrast echocardiography (TTCE) after PAVM embolization may offer a reliable alternative in a subgroup of patients while preventing radiation exposure. ⋯ This study shows chest CT scan might be withheld in patients with RLS grades 0 and 1 after PAVM embolization.
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COPD inhaler regimens should be appropriate for the patient's peak inspiratory flow (PIF) and should ideally consist of single or similar device(s). ⋯ Suboptimal PIF and inappropriate devices for measured PIF are highly prevalent among outpatients from a subspecialized COPD clinic. Female sex, reduced FEV1, and low BMI are important, readily identifiable risk factors for suboptimal PIF, and point-of-care PIF can inform clinical decision-making.
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It remains uncertain how long pure ground-glass nodules (pGGNs) detected on low dose computed tomography (LDCT) should be followed. Further studies with longer follow-up periods are needed to determine the optimal follow-up duration for pGGNs. ⋯ Among pGGNs that remained stable for 10 years, 3.9% eventually grew, indicating that some pGGNs can grow even after a long period of stability. We suggest that pGGNs may need to be followed for more than 10 years to confirm growth.