Annals of the American Thoracic Society
-
Multicenter Study Observational Study
Clinical Features and Associated Likelihood of Primary Ciliary Dyskinesia in Children and Adolescents.
Primary ciliary dyskinesia (PCD), a genetically heterogeneous, recessive disorder of motile cilia, is associated with distinct clinical features. Diagnostic tests, including ultrastructural analysis of cilia, nasal nitric oxide measurements, and molecular testing for mutations in PCD genes, have inherent limitations. ⋯ Systematically defined early clinical features could help identify children, including infants, likely to have PCD. Clinical trial registered with ClinicalTrials.gov (NCT00323167).
-
Primary ciliary dyskinesia (PCD) is a rare, heterogeneous, recessive, genetic disorder of motile cilia, leading to chronic upper and lower respiratory symptoms. Prevalence is estimated at around 1:10,000, but many patients remain undiagnosed, whereas others receive the label incorrectly. Proper diagnosis is complicated by the fact that the key symptoms, such as wet cough, chronic rhinitis, and recurrent upper and lower respiratory infection, are common and nonspecific. ⋯ However, they have been developed in tertiary care settings, where 10 to 50% of tested patients have PCD. The sensitivity and specificity of the tools are reasonable, but positive and negative predictive values may be poor in primary or secondary care settings. Although these studies are an important step toward an earlier diagnosis of PCD, more remains to be done before we have tools tailored to different health care settings.
-
The rate of global warming has accelerated over the past 50 years. Increasing surface temperature is melting glaciers and raising the sea level. More flooding, droughts, hurricanes, and heat waves are being reported. ⋯ Changes in temperature, precipitation, relative humidity, and air pollution influence viral activity and transmission. For example, in early 2000, an outbreak of Hantavirus respiratory disease was linked to a local increase in the rodent population, which in turn was attributed to a two- to threefold increase in rainfall before the outbreak. Climate-sensitive respiratory pathogens present challenges to respiratory health that may be far greater in the foreseeable future.
-
Multicenter Study
The SF-36 Offers a Strong Measure of Mental Health Symptoms in Survivors of Acute Respiratory Failure. A Tri-National Analysis.
Survivors of acute respiratory failure commonly experience long-term psychological sequelae and impaired quality of life. For researchers interested in general mental health, using multiple condition-specific instruments may be unnecessary and inefficient when using the Medical Outcomes Study Short Form (SF)-36, a recommended outcome measure, may suffice. However, relationships between the SF-36 scores and commonly used measures of psychological symptoms in acute survivors of respiratory failure are unknown. ⋯ For researchers interested in general mental health status, the SF-36 MH or MCS offers a strong measure of psychological symptoms prevalent among survivors of acute respiratory failure. For researchers interested in specific conditions, validated psychological instruments should be considered.
-
Biomarkers of pathogenesis in chronic obstructive pulmonary disease (COPD) can significantly accelerate drug development. In COPD related to alpha-1 antitrypsin deficiency, the role of neutrophil elastase and its inhibition by alpha-1 antitrypsin protein focused interest on elastin degradation and the development of pulmonary emphysema. ⋯ This review gives a perspective on what has been learned by the earliest measurements of desmosine and isodesmosine followed by later studies using methods of increased sensitivity and specificity and the meaning for developing new therapies. Also included are brief statements on the biomarkers fibrinogen, CC-16, and Aa-Val-360 in COPD.