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Cystic interstitial lung diseases: recognizing the common and uncommon entities.

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Cystic lung diseases present a considerable diagnostic challenge because they are less frequently encountered in clinical practice and high-resolution computed tomography findings are often similar in many of these diseases.

Here, we review the high-resolution computed tomography findings of cystic lung diseases and their mimics and provide a systematic approach to their diagnosis. Specific diseases that are discussed include pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, and light chain deposition disease.

Genetic determinants of pulmonary fibrosis: evolving concepts.

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ILDInterstitial lung diseases encompass a wide range of diffuse lung disorders that are often complicated by the development of pulmonary fibrosis and that can occur in isolation or in systemic diseases. In the past decade, availability of high-throughput genotyping and large collaborative clinical networks has led to the identification of many genetic variants associated with sporadic and familial fibrotic interstitial pneumonias.

Susceptibility to idiopathic pulmonary fibrosis seems to involve a combination of polymorphisms related to epithelial cell injury and dysfunction and abnormal wound healing, whereas chronic inflammation seems important in the development of pulmonary fibrosis in sarcoidosis or collagen vascular diseases such as systemic sclerosis or rheumatoid arthritis; however, each of the disease-associated variants typically has a small effect and they are therefore not helpful in prediction of risk. Genetic studies have substantially expanded understanding of the pathogenesis of pulmonary fibrosis.

Future challenges will be to assess how multiple susceptibility alleles interact with each other and environmental factors to determine disease risk and several different phenotypes, to define the mechanism of action and cellular pathways involving susceptibility alleles, and to identify which of the gene products and molecular mechanisms implicated in disease pathobiology are good therapeutic targets.

The addition of nebulised magnesium to the standard asthma therapy for children with severe symptoms results in small improvements in asthma severity scores

The prior research has found that magnesium sulfate given intravenously acts as a bronchodilator and improves airway obstruction in status asthmaticus.
  1. The intravenous route requires substantial resources for administration and close monitoring due to concerns for adverse effects, such as hypotension. Several studies have suggested that nebulised magnesium may also have benefits.
  2. If the efficacy was demonstrated for nebulised magnesium, it could be added to bronchodilators in the standard asthma treatment prot...

Commentary on: MAGNEsium Trial In Children (MAGNETIC) : Powell C, Kolamunnage-Dona R, Lowe J, et al.
A randomised, placebo-controlled trial and economic evaluation of nebulised magnesium sulphate in acute severe asthma in children.
Health Technol Assess 2013;17:1–216.
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Evidence-based clinical decision support improves the appropriate use of antibiotics and rapid strep testing

This study addresses the clinical impact of CDS for pharyngitis on evidence-based practices and evaluates challenges to achievement of large-scale adoption rates. The research findings include outcome and process measures that can be used to determine the efficacy of clinical prediction rules within the provider work flow.

Methods A randomised clinical trial evaluated two evidence-based clinical prediction rules (CPR) used in ambulatory primary care clinics by physicians, residents, fellows and nurse practitioners.

The study used both clinical outcomes and process measures to determine the efficacy and adoption of the...

New GOLD Classification: Longitudinal Data on Group Assignment

Little is known about the longitudinal changes associated with using the 2013 update of the multidimensional GOLD strategy for chronic obstructive pulmonary disease (COPD).

Objective To determine the COPD patient distribution of the new GOLD proposal and evaluate how this classification changes over one year compared with the previous GOLD staging based on spirometry only.

Methods We analyzed data from the CHAIN study, a multicenter observational Spanish cohort of COPD patients who are monitored annually. Categories were defined according to the proposed GOLD: FEV1%, mMRC dyspnea, COPD Assessment Test (CAT), Clinical COPD Questionnaire (CCQ), and exacerbations-hospitalizations. One-year follow-up information was available for all variables except CCQ data.

Results At baseline, 828 stable COPD patients were evaluated. On the basis of mMRC dyspnea versus CAT, the patients were distributed as follows: 38.2% vs. 27.2% in group A, 17.6% vs. 28.3% in group B, 15.8% vs. 12.9% in group C, and 28.4% vs. 31.6% in group D. Information was available for 526 patients at one year: 64.2% of patients remained in the same group but groups C and D show different degrees of variability. The annual progression by group was mainly associated with one-year changes in CAT scores (RR, 1.138; 95%CI: 1.074–1.206) and BODE index values (RR, 2.012; 95%CI: 1.487–2.722).

Conclusions In the new GOLD grading classification, the type of tool used to determine the level of symptoms can substantially alter the group assignment. A change in category after one year was associated with longitudinal changes in the CAT and BODE index.

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