Chest
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We designed an Internet-Based Monitoring Systems (IBS) survey to facilitate monitoring of asthma symptoms and asthma exacerbations in the Severe Asthma Research Program (SARP). Our objective was to evaluate compliance with the IBS survey tool and to explore how data from an IBS tool can inform understanding of asthma phenotypes. ⋯ Active participation with an Internet-based symptom survey tool in patients with severe asthma is influenced by race, socioeconomic status, and asthma control. Among survey participants, a group with highly variable (labile) asthma control is identifiable as a specific subgroup with unmet treatment needs. The association of asthma lability, increased susceptibility to adverse asthma effects of cold and flulike illnesses, and metabolic dysfunction provides clues for potentially effective intervention strategies.
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Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women caused by proliferation of neoplastic-like LAM cells, with mutations in the TSC1/2 tumor suppressor genes. Based on case reports, levels of cancer antigen 125 (CA-125), an ovarian cancer biomarker, can be elevated in patients with LAM. We hypothesized that elevated serum CA-125 levels seen in some patients with LAM were due to LAM, not other malignancies, and might respond to sirolimus treatment. ⋯ Higher serum CA-125 levels were associated with pleural effusions and reduced pulmonary function and were decreased with sirolimus therapy. LAM cells express CA-125. Some elevated serum CA-125 levels may reflect serosal membrane involvement.
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Asthma during pregnancy poses a common, increasingly prevalent threat to the health of women and their children. The present article reviews recent insights gained from the epidemiology of asthma during pregnancy, demonstrating the many short- and long-term risks to mother and fetus incurred by poorly controlled maternal asthma. We further discuss emerging evidence that active management of asthma during pregnancy can positively influence and perhaps completely mitigate these poor outcomes. ⋯ The cardiovascular and respiratory physiology of pregnancy is reviewed, as well as its implications for the management of patients with asthma, including patients requiring intubation and mechanical ventilation. For the situation when outpatient asthma management has failed, an approach to the critically ill pregnant patient with status asthmaticus is detailed. Multidisciplinary teams that include pulmonary specialists, obstetricians, primary care providers, nurses, pharmacists, and asthma educators improve the care of pregnant women with asthma.
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Ivacaftor produces significant clinical benefit in patients with cystic fibrosis (CF) with the G551D mutation. Prevalence of this mutation at the Cork CF Centre is 23%. This study assessed the impact of cystic fibrosis transmembrane conductance regulator modulation on multiple modalities of patient assessment. ⋯ Early and sustained improvements on ultra-low-dose CT scores suggest it may be a useful method of evaluating treatment response. It paralleled improvement in symptoms, circulating inflammatory markers, and changes in the lung microbiota.