Hypersensitivity pneumonitis in a cystic fibrosis patient

2019 ◽  
Author(s):  
A P Bellanger ◽  
H Morisse-Pradier ◽  
G Reboux ◽  
E Scherer ◽  
S Pramil ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Patient ◽  
Pulmonary Disease ◽  
Hypersensitivity Pneumonitis ◽  
Metalworking Fluids ◽  
Aetiological Agent ◽  
Pseudomonas Oleovorans ◽  
Inflammatory Lung Disease

Abstract Hypersensitivity pneumonitis (HP) is a chronic inflammatory lung disease caused by repeated inhalation of antigenic substances. We present a case of metalworking fluids (MWFs)-HP sensitized to Pseudomonas oleovorans in a cystic fibrosis patient. This case illustrates that HP diagnosis remains challenging, especially in patients with another pulmonary disease, and that serodiagnosis contributes to identifying the precise microorganism involved. It also demonstrates that P. oleovorans is an important secondary aetiological agent in MWF-HP, less known than Mycobacterium immunogenum.

Wisconsin Cystic Fibrosis Chest Radiograph Scoring System

PEDIATRICS ◽  
1993 ◽  
Vol 91 (2) ◽  
pp. 488-495 ◽  
Author(s):  
Mark R. Weatherly ◽  
Christina G.S. Palmer ◽  
Mary Ellen Peters ◽  
Christopher G. Green ◽  
Dennis Fryback ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Pulmonary Disease ◽  
Chest Radiograph ◽  
Scoring System ◽  
Objective Measure ◽  
Scoring Systems ◽  
Chest Radiographs ◽  
Pilot Studies ◽  
Mild Disease

A new clinical scoring system for patients with cystic fibrosis is needed because of recent advances in diagnosis and treatment which have changed the course of this disease. Chest radiograph scoring is the best objective measure of pulmonary disease for longitudinal studies beginning with infants; however, based on pilot studies, previous scoring systems are not sensitive enough in discriminating between degrees of mild lung disease. Therefore, a new radiographic scoring system was developed with the goal of achieving both sensitivity and reproducibility. This objective was pursued by applying multiattribute utility theory, using a panel of interpreters with expertise in cystic fibrosis radiology, and employing mathematical modeling techniques to weight the various components. The system was developed and validated in three phases including comparison to the Brasfield method of quantitative radiology. The data demonstrate that the new system can be applied reliably and conveniently to generate reproducible scores of pulmonary disease severity. Evaluation of the scores by four independent raters using chest radiographs from 61 patients at an average age of 8.37 years revealed good agreement with a .714 Kendall coefficient of concordance. Assessment of serial changes over time was performed using a group of 176 chest radiographs from 25 patients ranging from 4 weeks to 6 years old; this showed that the Wisconsin system generates score differences that are greater in magnitude with disease progression compared with the Brasfield method. Therefore, the new method is more sensitive to progression of mild disease and should be superior to prior radiographic scoring systems for evaluating therapies designed to modify the early course of disease. The Wisconsin system is designed to be useful in longitudinal clinical studies involving young children with cystic fibrosis and is capable to detecting progression from normality to mild lung disease.

Characteristics of Mycobacterium massiliense in a Cystic Fibrosis Patient Who Died With Nontuberculous Mycobacterial Pulmonary Disease

CHEST Journal ◽  
2010 ◽  
Vol 138 (4) ◽  
pp. 685A
Author(s):  
Young Soon Yoon ◽  
Adrian M. Zelazny ◽  
Kenneth N. Olivier ◽  
Aaron M. Milstone ◽  
Steven M. Holland ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Pulmonary Disease ◽  
Mycobacterium Massiliense

scholarly journals Mucin Overproduction in Chronic Inflammatory Lung Disease

2006 ◽  
Vol 13 (6) ◽  
pp. 327-335 ◽  
Author(s):  
Hans-Peter Hauber ◽  
Susan C Foley ◽  
Qutayba Hamid
Keyword(s):  
Cystic Fibrosis ◽  
Growth Factors ◽  
Lung Disease ◽  
Neutrophil Elastase ◽  
Present Review ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Inflammatory Lung Disease ◽  
Cystic Fibrosis Transmembrane

Mucus overproduction and hypersecretion are commonly observed in chronic inflammatory lung disease. Mucins are gel-forming glycoproteins that can be stimulated by a variety of mediators. The present review addresses the mechanisms involved in the upregulation of secreted mucins. Mucin induction by neutrophil elastase, bacteria, cytokines, growth factors, smoke and cystic fibrosis transmembrane conductance regulator malfunction are also discussed.

Lung Disease and Genomics

2018 ◽  
Vol 29 (1) ◽  
pp. 74-83 ◽  
Author(s):  
Kenneth Wysocki
Keyword(s):  
Lung Cancer ◽  
Chronic Obstructive Pulmonary Disease ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Precision Medicine ◽  
Pulmonary Disease ◽  
The Body ◽  
Chronic Obstructive ◽  
Past Century ◽  
Obstructive Pulmonary Disease

Research and application of genomic medicine in lung disease during the past century has clarified our understanding and focus on specific phenotypes, helping clinicians tailor treatment for individual patients. Cystic fibrosis and lung cancer have been researched extensively; specific genotypes have been instrumental in precision medicine to treat these lung diseases. Asthma and chronic obstructive pulmonary disease are more complex and heterogeneous in their pathogenesis, genotypic profile, and phenotypic expression, making treatment more difficult with increasing disease severity. This article focuses on the evolving state of the science of precision medicine in lung cancer, chronic obstructive pulmonary disease, asthma, and cystic fibrosis. The body of knowledge in lung disease is growing related to pharmacogenomics, clinical guidelines, genome editing, and approaches to genomic health that will guide clinical treatment options, reduce risk, and promote health.

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