Respiratory medicine
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Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause or cure, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3-5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. ⋯ When community MDE is not possible, or diagnostic doubt exists, referral to an ILD center should be considered. ILD center referral may also provide access specialized care, including clinical trials and lung transplantation, and should be considered for any patient with an established diagnosis of IPF.
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Respiratory medicine · Jun 2014
Specialist respiratory physicians' attitudes to and practice of advance care planning in COPD. A pilot study.
Advance care planning (ACP) is increasingly recognised as important in chronic obstructive pulmonary disease (COPD). Specialist respiratory physicians (RPs) are crucial in enabling ACP in patients with COPD. Accordingly, understanding their practice and attitudes regarding ACP is important. ⋯ This pilot found the new instrument was acceptable. Findings suggest that ACP discussions are infrequent with the majority occurring in the inpatient setting, with key elements omitted. Participants generally had favourable attitudes to ACP and recognised for the need for ongoing training. These early findings require further investigation.
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Respiratory medicine · Jun 2014
Observational StudyTracking Lung Clearance Index and chest CT in mild cystic fibrosis lung disease over a period of three years.
Lung disease remains the main cause of morbidity and mortality in patients with Cystic Fibrosis (CF). To detect lung disease before clinical symptoms become apparent, sensitive tools are essential. Spirometry is used for monitoring, but the FEV1 remains frequently normal throughout childhood. The Lung Clearance Index (LCI) calculated from Multiple Breath Washout (MBW) was introduced at the CF centre Innsbruck in 2007 for assessing ventilation inhomogeneity in patients with mild lung disease. We hypothesized that LCIs in 2007 are of prognostic value for the presence or absence of structural lung changes in later years. ⋯ The LCI is a sensitive tool for detecting and tracking pulmonary changes. Extended structural changes are unlikely if the LCI is normal. The LCI has the potential to be used for monitoring the progression of early CF lung disease and assessing the effect of treatment in both clinical care and research settings.