Articles: function.
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Primary ciliary dyskinesia (PCD) is characterised by chronic suppurative lung disease, rhino-sinusitis, hearing impairment and sub-fertility. We have developed the first multidimensional measure to assess health-related quality of life (HRQoL) in adults with PCD (QOL-PCD). Following a literature review and expert panel meeting, open-ended interviews with patients investigated the impact of PCD on HRQoL in the UK and North America (n=21). ⋯ Cognitive testing confirmed that content was comprehensive and the items were well-understood by respondents. Content validity and cognitive testing supported the items and structure. QOL-PCD has been translated into other languages and is awaiting psychometric testing.
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Minerva anestesiologica · Aug 2015
Observational StudyFeasibility of post-intensive Care Unit Clinics: an observational cohort study of two different approaches.
Post-ICU clinics have been advocated to reduce long-term physical and psychological impairments among ICU survivors. A format for optimal structure, timing, and care content has not yet been established. We developed and implemented two post-ICU clinics in different hospital settings and evaluated the feasibility. ⋯ This study provides valuable information to support the implementation of post-ICU clinics. The use of validated screening instruments facilitates the identification of patients with need for further treatment. Early in-hospital screening and recruiting patients at highest risk for adverse outcome could be a more targeted approach to achieve greater benefit.
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Metabolomic evaluation of cystic fibrosis (CF) airway secretions could identify metabolites and metabolic pathways involved in neutrophilic airway inflammation that could serve as biomarkers and therapeutic targets. ⋯ MS metabolomics identified multiple metabolic pathways associated with neutrophilic airway inflammation. These findings provide insight into disease pathophysiology and can serve as the basis for developing disease biomarkers and therapeutic interventions for airways diseases.
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Clinical outcomes are typically assessed by trained staff. We tested the hypothesis that outcomes reported by the patient or a caregiver on the web would be correlated with a validated interview. ⋯ Web-based HRQoL assessment with NIH PROMIS and Neuro-QOL is feasible and correlated with a validated interview for the mRS. T scores distinguished between individual categories of the mRS, detecting modest differences in physical function and mobility HRQoL that are difficult to detect with the mRS. PROMIS and Neuro-QOL provide powerful and sensitive outcomes for potentially large cohorts.
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Review Historical Article
[Long QT syndrome : History, genetics, clinical symptoms, causes and therapy].
The long QT syndrome is caused by a change in cardiac repolarization due to functional ion channel defects. A differentiation is made between a congenital (cLQTS) and an acquired (aLQTS) form of the disease. ⋯ This article summarizes the current knowledge on the history, pathophysiology, clinical symptoms and therapy of cLQTS and aLQTS. This knowledge of pathophysiological features of the symptoms allows the underlying anesthesiological approach for individualized perioperative concepts for patients suffering from LQTS to be derived.