American journal of respiratory and critical care medicine
-
Am. J. Respir. Crit. Care Med. · May 2016
In-hospital Mortality Following Surgical Lung Biopsy for Interstitial Lung Disease in the USA: 2000-2011.
Surgical lung biopsy can help to determine a specific diagnosis in interstitial lung disease but has associated risks. Most currently available mortality data are derived from case series and may not be generalizable to broader populations. ⋯ In-hospital mortality after elective surgical lung biopsy for interstitial lung disease is just under 2% but significantly higher for nonelective procedures. Identified risk factors for death should be taken into account when counseling patients on whether to pursue a histologic diagnosis.
-
Am. J. Respir. Crit. Care Med. · May 2016
Mutations of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene Cause a Monocyte-selective Adhesion Deficiency.
Cystic fibrosis (CF) is a common genetic disease caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Persistent lung inflammation, characterized by increasing polymorphonuclear leukocyte recruitment, is a major cause of the decline in respiratory function in patients with CF and is a leading cause of morbidity and mortality. CFTR is expressed in various cell types, including leukocytes, but its involvement in the regulation of leukocyte recruitment is unknown. ⋯ Altogether, these data highlight the critical regulatory role of CFTR in integrin activation by chemoattractants in monocytes and identify CF as a new, cell type-selective leukocyte adhesion deficiency disease, providing new insights into CF pathogenesis.
-
Am. J. Respir. Crit. Care Med. · May 2016
Randomized Controlled Trial Multicenter StudyA Randomized Trial of an Intensive Physical Therapy Program for Acute Respiratory Failure Patients.
Early physical therapy (PT) interventions may benefit patients with acute respiratory failure by preventing or attenuating neuromuscular weakness. However, the optimal dosage of these interventions is currently unknown. ⋯ An intensive PT program did not improve long-term physical functional performance compared with a standard-of-care program. Clinical trial registered with www.clinicaltrials.gov (NCT01058421).
-
Am. J. Respir. Crit. Care Med. · May 2016
Desmoplakin (DSP) Variants are Associated with Idiopathic Pulmonary Fibrosis.
Sequence variation, methylation differences, and transcriptional changes in desmoplakin (DSP) have been observed in patients with idiopathic pulmonary fibrosis (IPF). ⋯ Sequence variants in DSP are associated with IPF, and rs2076295 genotype is associated with differential expression of DSP in the lung. DSP expression is increased in IPF lung and concentrated in the airway epithelia, suggesting a potential role for DSP in the pathogenesis of IPF.