Cystic Fibrosis Research - Symptoms, Genetics, Treatment, Information

Cystic Fibrosis Research Today is a free monthly online journal that collates and summarizes the latest research about Cystic Fibrosis, including details on symptoms, genetics, treatment, information.


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The effect of chronic infection with Aspergillus fumigatus on lung function and hospitalization in patients with cystic fibrosis.

Amin R, Dupuis A, Aaron SD, Ratjen F

Department of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada. reshma.amin@sickkids.ca

BACKGROUND: The relevance of Aspergillus fumigatus in patients with cystic fibrosis (CF) not affected by allergic bronchopulmonary aspergillosis is unclear. Our aim was to determine the effect of persistent infection with A fumigatus on pulmonary exacerbations and lung function in children with CF. METHODS: This was a retrospective cohort study of patients with CF followed at The Hospital for Sick Children from 1999 to 2006. Persistent A fumigatus infection was defined as the presence of two or more positive sputum or bronchoalveolar cultures for A fumigatus in a given year. The primary outcome measure was the annual number of hospitalizations for pulmonary exacerbations. RESULTS: Two hundred thirty patients with CF were included in the analysis. The FEV(1) of patients persistently infected with A fumigatus was 3.61% (P< or =.0001) lower during the study period compared with uninfected patients. There was a significant interaction between A fumigatus and Pseudomonas aeruginosa on lung function (P=.0006). Patients not infected with either organism had the highest pulmonary function. Persistent A fumigatus infection (relative risk [RR]=1.94, P=.0002) and CF-related diabetes (RR=1.64, P=.028) were associated with an increased risk of pulmonary exacerbations requiring hospitalization, whereas there was no increased risk of pulmonary exacerbations among patients with allergic bronchopulmonary aspergillosis (RR=1.02, P=.94). When adjusted for baseline pulmonary function, none of these variables were associated with a significantly increased risk of pulmonary exacerbations, with only chronic A fumigatus infection trending toward significance (RR=1.40, P=.065). CONCLUSIONS: Persistent A fumigatus infection is an independent risk factor for hospital admissions in patients with CF.

Published 6 January 2010 in Chest, 137(1): 171-6.
Full-text of this article is available online (may require subscription).


Articles on Cystic Fibrosis published 5 January 2010:

Neonatal screening for treatable and untreatable disorders: prospective parents' opinions.   Pediatrics, 125(1): e99-106.

OBJECTIVE: In the Netherlands, in 2007, the national newborn screening program was expanded from 3 to 17 disorders that met the World Health Organization's Wilson and Jungner screening criteria, especially regarding treatability. The decision of whether to add diseases to the program is generally based on experts' advice, whereas the opinion of those whom it concerns--prospective parents--remains unknown. In this study, we investigated the opinion of prospective parents concerning newborn ... [Abstract] [Full-text]


Articles on Cystic Fibrosis published 17 December 2009:

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.   Nat Chem Biol, 6(1): 25-33.

Chemical modulation of histone deacetylase (HDAC) activity by HDAC inhibitors (HDACi) is an increasingly important approach for modifying the etiology of human disease. Loss-of-function diseases arise as a consequence of protein misfolding and degradation, which lead to system failures. The DeltaF508 mutation in cystic fibrosis transmembrane conductance regulator (CFTR) results in the absence of the cell surface chloride channel and a loss of airway hydration, leading to the premature lung ... [Abstract] [Full-text]


Articles on Cystic Fibrosis published 16 December 2009:

Association between carrier screening and incidence of cystic fibrosis.   JAMA, 302(23): 2573-9.

CONTEXT: A downward trend in cystic fibrosis (CF) birth incidence has been reported in some areas. OBJECTIVE: To evaluate the association between carrier screening and CF birth incidence. DESIGN, SETTING, AND PARTICIPANTS: In northeastern Italy, CF birth incidence is monitored by means of a long-standing neonatal screening program. In the same area, 2 sections using different carrier detection approaches were identified--the western region, in which CF carrier tests are offered only to ... [Abstract] [Full-text]

Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages.   J Biol Chem, 284(51): 35926-38.

Alveolar macrophages (AMs) play a major role in host defense against microbial infections in the lung. To perform this function, these cells must ingest and destroy pathogens, generally in phagosomes, as well as secrete a number of products that signal other immune cells to respond. Recently, we demonstrated that murine alveolar macrophages employ the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel as a determinant in lysosomal acidification (Di, A., Brown, M. E., ... [Abstract] [Full-text]

Subinhibitory concentrations of moxifloxacin decrease adhesion and biofilm formation of Stenotrophomonas maltophilia from cystic fibrosis.   J Med Microbiol, 59: 76-81.

Stenotrophomonas maltophilia is an emerging nosocomial bacterial pathogen that is currently isolated with increasing frequency from the airways of cystic fibrosis (CF) patients. In this study the effect of subinhibitory concentrations (subMICs) of moxifloxacin on adhesion, biofilm formation and cell-surface hydrophobicity of two strains of S. maltophilia isolated from CF patients were evaluated. Adhesion and biofilm formation assays were carried out on polystyrene and quantified by colony ... [Abstract] [Full-text]


Articles on Cystic Fibrosis published 9 December 2009:

Sputum desmosine during hospital admission for pulmonary exacerbation in cystic fibrosis.   Chest, 136(6): 1561-8.

BACKGROUND: Cystic fibrosis (CF) lung disease is characterized by structural changes in the airways and parenchyma. No sputum biomarker exists to measure the degree of active structural destruction during pulmonary exacerbation in patients with CF. The noninvasive measurement of desmosine, a breakdown product of elastin, may reflect ongoing lung injury and serve as a biomarker of short-term damage. Our objectives were to measure desmosine in the sputum of patients with CF hospitalized for ... [Abstract] [Full-text]

Respiratory microbiology of patients with cystic fibrosis in the United States, 1995 to 2005.   Chest, 136(6): 1554-60.

BACKGROUND: Numerous improvements in diagnostic and therapeutic strategies for patients with cystic fibrosis (CF) have occurred during the past 2 decades. We hypothesized that these changes could impact trends in respiratory microbiology. METHODS: Data from the Cystic Fibrosis Foundation Patient Registry were used to examine trends in the incidence and prevalence of bacterial pathogens isolated from patients with CF in the United States from 1995 to 2005. RESULTS: The number of patients with CF ... [Abstract] [Full-text]


Articles on Cystic Fibrosis published 1 December 2009:

CFTR knockdown stimulates lipid synthesis and transport in intestinal Caco-2/15 cells.   Am J Physiol Gastrointest Liver Physiol, 297(6): G1239-49.

Cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel highly expressed in epithelial cells of the gastrointestinal tract. Mutations in the CFTR gene cause cystic fibrosis (CF), a disease characterized by pancreatic insufficiency, fat malabsorption, and steatorrhea. Despite the administration of pancreatic enzymes to normalize malabsorption, CF patients still experienced lipid fecal loss, nutritional deficiencies, and abnormalities in serum lipid profile, suggesting ... [Abstract] [Full-text]


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Cystic Fibrosis Research Today Archive:

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Volume 7 (2010)
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Cystic Fibrosis Books

Coughing the Distance - from Paris to Istanbul with Cystic Fibrosis

Coughing the Distance - from Paris to Istanbul with Cystic Fibrosis