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Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review
Respiratory muscle weakness is a major cause of morbidity and mortality in patients with neuromuscular diseases (NMDs). Respiratory involvement in NMDs can manifest broadly, ranging from milder insufficiency that may affect only sleep initially to severe insufficiency that can be life threatening. Patients with neuromuscular diseases exhibit very often sleep-disordered breathing, which is frequently overlooked until symptoms become more severe leading to irreversible respiratory failure necessitating noninvasive ventilation (NIV) or even tracheostomy. Close monitoring of respiratory function and sleep evaluation is currently the standard of care. Early recognition of sleep disturbances and initiation of NIV can improve the quality of life and prolong survival. This review discusses the respiratory impairment during sleep in patients with NMDs, the diagnostic tools available for early recognition of sleep-disordered breathing and the therapeutic options available for overall respiratory management of patients with NMDs.
Establishment of Reference Norms for Lung Function Parameters of Healthy Sri Lankan Tamils
Spirometry and Peak Expiratory Flow Rate (PEFR) are important measurements in diagnosing and monitoring of COPD and asthma. Ethnic specific reference equations are necessary in interpretation of these parameters. However, equations for Sri Lankan Tamil adults are not available. This study aims to establish reference equations for lung function parameters of Sri Lankan Tamils. A descriptive cross sectional study was carried out in all 5 districts of Northern Sri Lanka. Participants were selected by cluster sampling. Base line data were obtained by a questionnaire. Height, sitting height, weight, arm span, mid arm circumference, and chest expansion were measured. Respiratory functions were assessed by a calibrated spirometer (Cosmed Micro Quark, Italy) and Wright compatible peak expiratory flow meter. Means, and standard deviations for Vital Capacity (VC), Forced Vital Capacity (FVC), Forced Expiratory Volume in the first second (FEV1), FEV1%, Peak Expiratory Flow Rate (PEFR) and for other forced expiratory parameters of 775 males and 687 females were determined. Lung function parameters have significant positive correlations with most of the anthropometric measures. Age had a significant negative correlation with lung function parameters in adults >20 years and positive correlation in 14–20 years group. Step wise multiple regression analysis was used to determine the prediction equations. Also equations based on age, height and age, arm span were derived. Age, height based equations were retested in the same population. Predicted values by the developed equations had better agreement than that of GLI 2012 equations. This can be useful in assessing the respiratory function in Sri Lankan Tamil population as there are no already existing equations.
Does Pseudomonas aeruginosa Colonization Affect Exercise Capacity in CF?
Introduction. Cardio-Pulmonary Exercise Testing (CPET) has been recognized as a valuable method in assessing disease burden and exercise capacity among CF patients. Aim. To evaluate whether Pseudomonas aeruginosa colonization status affects Exercise Capacity, LCI and High-Resolution Computed Tomography (HRCT) indices among patients with CF; to check if Pseudomonas colonization can predict exercise intolerance. Subjects. Seventy-eight (78) children and adults with CF (31 males) mean (range) age 17.08 (6.75; 24.25) performed spirometry, Multiple Breath Washout (MBW) and CPET along with HRCT on the same day during their admission or follow up visit. Results. 78 CF patients (mean FEV1: 83.3% mean LCI: 10.9 and mean VO2 peak: 79.1%) were evaluated: 33 were chronically colonized with Pseudomonas aeruginosa, 24 were intermittently colonized whereas 21 were Pseudomonas free. Statistically significant differences were observed among the three groups in: peak oxygen uptake % predicted (VO2 peak% (), LCI (), as well as FEV1% () and FVC% (). Pseudomonas colonization could predict VO2 peak% (, : −0.395). Conclusion. Exercise capacity as reflected by peak oxygen uptake is reduced in Pseudomonas colonized patients and reflects lung structural damages as shown on HRCT. Pseudomonas colonization could predict exercise limitation among CF patients.
Pharmacist Led Intervention on Inhalation Technique among Asthmatic Patients for Improving Quality of Life in a Private Hospital of Nepal
Purpose. Asthma is a chronic disease which cannot be cured but can be controlled. Although drug therapy is used to relieve and prevent symptoms of asthma and treat exacerbations, still a good asthma control and a better quality of life in many patients is suboptimal due to improper use of inhalation technique. Thus, this interventional study was conducted to evaluate the effect of a pharmacist intervention on asthma control, quality of life and inhaler technique in adult asthmatic patients. Patients and Methods. A total of 72 patients who met the inclusion criteria and agreed to give written consent were enrolled in the study. These patients were randomly divided into two groups i.e., test group (36) and control group (36) by simple block randomization technique. Test group were the interventional groups. Mini Asthma Quality of Life Questionnaire (AQLQ), Asthma Control Questionnaire (ACQ) and structured questionnaires were used to sort the information like quality of life, asthma control and demographic details. They were counselled by the pharmacist about the asthma management and proper use of inhalers. Out of 72 patients, only forty six patients came for follow up after one month. Data were entered and analyzed using Statistical Package for Social Sciences (SPSS) software version 20. Results. A significant change was observed in the mean score of quality of life () in test group as well as control group, however change in the mean score of asthma control in the test group () was more significant as compared to the control group (). Inhalation technique was found to be improved significantly after intervention among patients using the metered dose inhaler and dry powder inhaler. Majority of the patients were prescribed with Methylxanthines (24.5%) followed by combined Beta 2 agonists and Inhaled Corticosteroids (21.7%). Conclusion. Pharmacist provided intervention improves the quality of life, asthma control and inhalation technique among asthmatic patients.
Regulatory T Cells in Respiratory Health and Diseases
Respiratory diseases compromise the health of millions of people all over the world and are strongly linked to the immune dysfunction. CD4+FOXP3+ T regulatory cells, also known as Tregs, have a central role maintaining tissue homeostasis during immune responses. Their activity and clinical impact have been widely studied in different clinical conditions including autoimmune diseases, inflammatory conditions, and cancer, amongst others. Tregs express transcription factor forkhead box P3 (FOXP3), which allows regulation of the immune response through anti-inflammatory cytokines such as IL-10 or transforming growth factor beta (TGF-β) and direct cell-to-cell interaction. Maintenance of immune tolerance is achieved via modulation of effector CD4+ T helper 1, 2 or 17 (Th1, Th2, Th17) cells by Tregs. This review highlights the recent progress in the understanding of Tregs in different disorders of the respiratory system.
Solid Indeterminate Nodules with a Radiological Stability Suggesting Benignity: A Texture Analysis of Computed Tomography Images Based on the Kurtosis and Skewness of the Nodule Volume Density Histogram
Background. The number of incidental findings of pulmonary nodules using imaging methods to diagnose other thoracic or extrathoracic conditions has increased, suggesting the need for in-depth radiological image analyses to identify nodule type and avoid unnecessary invasive procedures. Objectives. The present study evaluated solid indeterminate nodules with a radiological stability suggesting benignity (SINRSBs) through a texture analysis of computed tomography (CT) images. Methods. A total of 100 chest CT scans were evaluated, including 50 cases of SINRSBs and 50 cases of malignant nodules. SINRSB CT scans were performed using the same noncontrast enhanced CT protocol and equipment; the malignant nodule data were acquired from several databases. The kurtosis (KUR) and skewness (SKW) values of these tests were determined for the whole volume of each nodule, and the histograms were classified into two basic patterns: peaks or plateaus. Results. The mean (MEN) KUR values of the SINRSBs and malignant nodules were 3.37 ± 3.88 and 5.88 ± 5.11, respectively. The receiver operating characteristic (ROC) curve showed that the sensitivity and specificity for distinguishing SINRSBs from malignant nodules were 65% and 66% for KUR values >6, respectively, with an area under the curve (AUC) of 0.709 (). The MEN SKW values of the SINRSBs and malignant nodules were 1.73 ± 0.94 and 2.07 ± 1.01, respectively. The ROC curve showed that the sensitivity and specificity for distinguishing malignant nodules from SINRSBs were 65% and 66% for SKW values >3.1, respectively, with an AUC of 0.709 (). An analysis of the peak and plateau histograms revealed sensitivity, specificity, and accuracy values of 84%, 74%, and 79%, respectively. Conclusions. KUR, SKW, and histogram shape can help to noninvasively diagnose SINRSBs but should not be used alone or without considering clinical data.