Background Inhaled hypertonic saline (HS) reduces pulmonary exacerbations in patients with cystic fibrosis (CF) aged 6 or more years. However, the effectiveness of HS in improving clinical outcomes in younger children aged 6 or less years is not established. This study examines the efficacy of HS in younger CF patients. Methods Searches were conducted across three databases (Medline, Cochrane Central and EMBASE) from inception through July 2022. Randomized controlled trials assessing the impact of HS in younger CF patients were included. Trials involving only patients greater than 6 years or control group other than isotonic saline (IS) were excluded. Outcomes measured included lung clearance index (LCI), cystic fibrosis questionnaire (CFQ-R) score, spirometry measures, oxygen saturation, respiratory rate, height and weight. Outcomes were reported as mean differences (MDs) with 95% confidence intervals. Results Seven studies ( n = 390 patients) were included in this review. HS significantly reduced the LCI (MD: -0.67; 95%CI, -1.05 to 0.29, P = 0.0006) compared to IS. In addition, HS was associated with significant improvements in height (MD: 2.23; 95%CI, -0.00 to 4.46, P = 0.05) and CFQ-R (MD: 4.30; 95%CI, 0.65–7.95, P = 0.02), but not in oxygen saturation (MD: -0.15; 95%CI, -0.54 to 0.25, P = 0.47), respiratory rate (MD: -0.21; 95%CI, -2.19 to 1.77, P = 0.83) or weight (MD: 0.70; 95%CI, -0.47 to 1.87, P = 0.24). Furthermore, HS did not significantly improve spirometry measures, including FEV 1 (MD: -0.11; 95%CI, -0.21 to 0.43, P = 0.51) and forced vital capacity (MD: 0.27; 95%CI, -0.49 to 1.04, P = 0.48), but significantly improved FEF 25-75 (MD: 0.12; 95% CI, 0.05–0.20; P = 0.002). Discussion Treatment with HS in younger children with CF improves lung clearance, symptoms and quality of life. FEF 25-75 may prove a more sensitive measure for assessing intervention related improvements in pediatric CF trials. Conclusion The findings support HS as a therapeutic method in CF-affected children.
Introduction: Hyper-lucent lung syndrome or Swyer-James-Macleod syndrome is a rare, often an incidental finding, primarily showing unilateral hyper-lucency as a consequence of bronchiolitis obliterans. Often diagnosed in early childhood; though in some cases it may only be discovered in adulthood or on account of recurrent chest infections. Case Presentation: A patient presented to our outpatient center with complaints of shortness of breath with mild to moderate exertional dyspnea & exacerbations of symptoms classically associated with chronic obstructive pulmonary disease. Complaints have persisted for several years with waxing & waning intensity. There is a history of migraine, atrial fibrillation, hypothyroidism, and colon & breast cancer without recurrence. Computed tomography of the chest with intravenous contrast showed asymmetric emphysematous changes with marked hyperinflation & attenuated vasculatures confined to the middle lobe of the right lung. The patient was started on a combination of inhaled long-acting beta-agonists, corticosteroids & long-acting muscarinic antagonists and is almost asymptomatic. Conclusion: Geriatric patients are at increased risk of various age-related diseases, respiratory disorders being among them. Knowledge of this rare condition allows for its appropriate management as it often coexists with bronchiectasis & results in frequent respiratory infections, which may be preventable.
Introduction: Lady Windermere Syndrome is the bronchiectasis of the right middle lobe or the lingula region due to Mycobacterium avium complex (MAC) infection. It was historically described in elderly Caucasian female patients who were immunocompetent but subsequently developed MAC infection, theoretically due to chronic cough suppression. The diagnosis is often di cult or missed because it is not usually considered a differential by radiologists and clinicians.Case Presentation: Here we describe a classic case of a 61-year-old female patient with Lady Windermere Syndrome who is also riddled with prominent kyphoscoliosis with supportive chest radiographs and CT ndings. Conclusion: LadyWindermere syndrome is a signi cant yet frequently overlooked disease among older women who exhibit non-speci c clinical symptoms. The majority of cases experience positive clinical outcomes within a period of three to six months when provided with timely pharmaceutical intervention. It is crucial to consider Lady Windermere syndrome as a potential differential diagnosis, particularly in regions where there are concerns about the presence of MAC bacilli in the soil and water.
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