hepatobiliary disease in cystic fibrosis

... Colin AA: Cystic fibrosis: a system for assessing and predicting progression AJR Am J Roentgenol 1998, 170:1067-1072 Cleveland RH, Neish AS, Zurakowski D, Nichols DP, Wohl ME, Colin AA: Cystic fibrosis: ... cystic fibrosis, nonsense mutations in each cystic fibrosis gene, and mild pulmonary disease N Engl J Med 1990, 323:1685-1689 Zerhusen B, Ma J: Function of the second nucleotide-binding fold in the ... Residue 1219 is the first amino acid of the second nucleotide-binding domain (NBD2) of CFTR Numerous investigators have examined the functionality of NBD2 mutants and C-terminal CFTR truncation mutants...

Ngày tải lên: 11/08/2014, 12:20

... Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis N Engl J Med 2006; 354: 229-240 Robinson M, Daviskas ... fucose/galacatose to other inhalations (eg hypertonic saline) In conclusion the findings in this report show that inhalation with fucose/galactose solution could reduce P aeruginosa in sputum of adult ... chronic infection with P aeruginosa as at least the PA-II acts on cilia of CF patients in vitro in the same way as in normal controls (8) twice with PBS containing 2% FCS PMBC were counted, cytospins...

Ngày tải lên: 03/11/2012, 11:48

... sustained release and delivery of FDA approved proteasome inhibitor drug, PS341 in murine lungs by its ability to control Pseudomonas aeruginosa LPS induced CF lung disease in murine model In this ... with PLGA nanoparticles using ring-opening polymerization method, results in increased residence in blood (intravenous) or airway (intranasal) and enhanced accumulation in target tissues or cells ... chronic obstructive airway diseases, including COPD and CF Distinct etiologies and inflammatory responses drive mucous hypersecretion in these diseases In CF and COPD, the inflammatory response is...

Ngày tải lên: 11/08/2014, 00:22

... protein:detection of vitamin K deficiency and liver disease in patients with cystic fibrosis J Pediatr 1981, 99(2):254-257 13 Durie PR: Vitamin K and the management of patients with cystic fibrosis ... disease, and in 14, of vitamin K deficiency [12] In 1994 Durie assessed the need for routine vitamin K supplementation in patients with CF [13] Factors in CF that predispose patients to vitamin K deficiency ... 2.13% incidence of CD among 230 CF patients [18] Taking into consideration the incidences of these two diseases, the chance of their occurring together in the general population is one in 2,000,000...

Ngày tải lên: 11/08/2014, 00:23

... palisades of PMNs surrounding and trying to engulf/phagocytose droplets of marijuana There have been no previous reports of airway PMNs containing unusual inclusion bodies in marijuana smokers, but ... with increasing airway sepsis Consideration should be given to sputum cytology and CT scanning when CF patients develop an unexplained deterioration in respiratory status Consent Written informed ... importance of considering drug abuse/marijuana use in CF patients, particularly in the context of atypical paraseptal bullae, recurrent pneumothoraces, accelerated decline in lung function and...

Ngày tải lên: 11/08/2014, 02:22

... we investigated the following two hypotheses: 1) To determine if the signaling pathway going through the MAPKs: p38, extracellularregulated protein kinase (ERK), and Jun-N terminal protein kinase ... pretreatment results in an inhibition of inflammatory signaling in cells with CF defects Production of IL-8 and other pro-inflammatory cytokines is also governed by mechanisms regulating in their mRNA ... Auricchio, S Cimmino, M Cavaliere, M Nardone, M Cesaro, A Malcolm, J Quaratino, S Londei, M: Inhibition of p38 mitogen activated protein kinase controls airway inflammation in cystic fibrosis Thorax...

Ngày tải lên: 11/08/2014, 03:20

... [14,15] Platinum wire is inserted into glass capillaries (Kimax-51, Kimble products) and placed inside a heated platinum coil The glass is pulled to create a thin insulating layer on the platinum wire ... severe disease related CFTR mutation will result in an increase in membrane cholesterol, with a larger magnitude increase in the ΔF/ΔF tissue The magnitude increase in membrane cholesterol in ΔF/ΔF ... findings of cholesterol synthesis inhibition by resveratrol A similar increase in HMGCoAR expression is seen in mevastatin treated cells as a positive control These results support the findings...

Ngày tải lên: 12/08/2014, 11:22

... (A) H&E stain: inflammatory infiltrate (arrows) and (B) PAS stain: mucin producing airway lining cells (Pink, arrows) Controls showed much less inflammatory infiltrates or mucin producing cells ... lysed using ACK lysing buffer and then the remaining cells were resuspended in staining buffer [37] containing 10% FBS for differential cell counts using flow cytometry Cells were double stained ... Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease Eur Respir J 1999, 14:339-346 Cantin A: Cystic fibrosis lung inflammation: early, sustained, and severe...

Ngày tải lên: 12/08/2014, 13:22

... Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line J Clin Invest 1992, 89:1478-1484 ... Endobronchial infection in cystic fibrosis Acta Paediatr Scand Suppl 1989, 363:31-36 Armstrong DS, Grimwood K, Carzino R, Carlin JB, Olinsky A, Phelan PD: Lower respiratory infection and inflammation in infants ... cells in primary cultures were identified by immunostaining for smooth muscle cell specific α-actin, and Western blotting for myosin light chain kinase and calponin Many inflammatory cytokines...

Ngày tải lên: 12/08/2014, 14:20

... in the ionic strength and pH of the airway lining fluid and the increased levels of inflammatory cells present in the inflamed tissues [16] The defects in innate defences become self-sustaining, ... predominantly seen within the inflammatory cell mass in the airway lumen or in isolated cells either infiltrating the epithelium or within the sub epithelial layer The lack of SPLUNC1 staining in ... putative innate defence molecule expressed in the upper airways and in submucosal glands, SPLUNC1 could potentially be involved in combating the chronic infections seen in CF and other lung diseases...

Ngày tải lên: 12/08/2014, 15:21

... been reported to inhibit airway inflammation in a murine model of asthma [18] and a model of airway infection [19] by inhibiting eosinophil, lymphocyte and neutrophil influx into the lung Moreover, ... PPAR DNA binding activity (AU) PPAR C CF Figure Differential PPARα binding to PPRE in peripheral lymphocytes Differential PPARα binding to PPRE in peripheral lymphocytes PPARα DNA binding was analyzed ... infiltrating the cystic fibrosis airway mucosa Clin Exp Immunol 2001, 124:69-76 Moss RB: Lymphocytes in cystic fibrosis lung disease: a tale of two immunities Clin Exp Immunol 2004, 135:358-360 Reineck...

Ngày tải lên: 12/08/2014, 16:20

... lung disease and survival in cystic fibrosis J Clin Invest 1999, 104:431–437 Gabolde M, Guilloud-Bataille M, Feingold J, Besmond C: Association of variant alleles of mannose binding lectin with ... reasoned that genes involved in innate defense against bacteria outside CF might also be relevant in the context of CF They examined CF patients for alleles of the mannose-binding lectin (MBL) gene ... of susceptibility to infection in cystic fibrosis J Pathol 1998, 184:323–331 Kelley TJ, Drumm ML: Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway...

Ngày tải lên: 12/08/2014, 18:20

... Diversity in Cystic Fibrosis Lung Infections Using 16S rDNA Terminal Restriction Fragment Length Polymorphism Profiling J Clin Microbiol in press Brook I, Fink R: Transtracheal aspiration in pulmonary ... adults with cystic fibrosis born in the United Kingdom between 1947 and 1967 Thorax 1999, 54:420-422 Lyczak JB, Cannon CL, Pier GB: Lung infections associated with cystic fibrosis Clin Microbiol ... in the corresponding T-RFLP profile Where a band of a given length was detected in profiles generated in a sample, it was present in both T-RFLP and RT-T-RFLP profiles in 33.7% of instances In...

Ngày tải lên: 12/08/2014, 18:21

... decline of 14 ml in 9T/9T, 3.8 ml in 7T/9T, 61 ml in 6T/ 7T, 23 ml in 5T/9T, ml in 5T/7T, 31 ml in 9T/9T F508del, ml in 7T/9T F508del, 72 ml in 7T/7T F508del, and 72 ml in 5T/9T F508del individuals ... allele in the CFTR gene [1], S and Z alleles in the Serine Protease Inhibitor-A1 gene [42], and B, C, and D alleles in the Mannose-Binding Lectin-2 gene [43] were identified using polymerase chain ... lung disease such as tobacco smoke or familial predisposition to lung disease Variations in the genes for mannose-binding lectin and α1-antitrypsin have been studied as modifiers of cystic fibrosis...

Ngày tải lên: 12/08/2014, 18:22

... sensitivity in a tissue-specific manner As the inflammatory process in CF is dominated by a neutrophil influx in the airways, our present findings reporting that CF patients carrying the BclI ... polymorphisms in candidate genes involved in the inflammatory cascade have been shown to modulate the expression of the clinical phenotype [18,19] In several inflammatory diseases, variations in glucocorticoid ... and OT have been involved in the interpretation of the data AH-C has been involved in collecting the patients DNA and the phenotypical data BF and JF have been involved in revisiting the manuscript...

Ngày tải lên: 12/08/2014, 15:21

... air-drying for hrs Samples were fixed in 4% paraformaldehyde in PBS for 20 min, protein hydrolyzed in 20 µg/ml proteinase K for 7.5 min, and then post-fixed for in 4% paraformaldehyde in PBS ... S100A8 staining in B6-CF lungs (Figure 3K and 3L, respectively), indicating that its increased levels in whole lungs were not the effect of induction in such cells, but exclusively due to the increased ... (calprotectin), but not S100A9 alone, as having a possible role in progression of the inflammatory lung phenotype in CF mice Finally, since both the B6-CF and Bc-CF mice were maintained in identical...

Ngày tải lên: 12/08/2014, 16:20

... all impacts of a disease on daily life, they allow investigations in large populations Considering the advances in CF therapy and with regard to the recent increase in interest in PROs, the German ... further investigation Both self-rating and proxy rating provide important complementary sources of information, and these should be assessed in combination Use of the CFQ-R in clinical studies and in ... evaluation of mortality in cystic fibrosis Pediatr Pumlonol 2002, 33(2):99-105 Demko CA, Byard PJ, Davis PB: Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection J Clin Epidemiol 1995,...

Ngày tải lên: 18/06/2014, 19:20

... differences in pain, coping, and mood in individuals having osteoarthritic knee pain: a within-day analysis Pain 2004, 110:571-577 Wilson G, Pritchard ME, Revalee B: Individual differences in adolescent ... FitzSimmons SC: The changing epidemiology of cystic fibrosis J Pediatr 1993, 122:1-9 Demko CA: Gender differences in cystic fibrosis: Pseudomonas aeurginosa infection J Clin Epidemiol 1995, 48:1041-1049 ... Cincinnati Children's Hospital Medical Center in Cincinnati, Ohio and The Children's Medical Center in Dayton, Ohio between 1997–2001 Patients and their families were invited to participate in...

Ngày tải lên: 20/06/2014, 15:20

... Musculoskeletal manifestations in cystic fibrosis Joint Bone Spine 2003, 70:327-335 Festini F, Ballarin S, Codamo T, Doro R, Loganes C: Prevalence of pain in adults with cystic fibrosis J Cyst Fibros ... Sauer-Heilborn A, Köhnlein T, Seidler U, Lamprecht G: Cystic fibrosis in adults Internist 2010, 51(Suppl 1):277-288 Ravilly S, Robinson W, Suresh S, Wohl ME, Berde CB: Chronic pain in cystic fibrosis Pediatrics ... mobilization maneuvers involving intensified compression techniques during assisted autogenous drainage and jumping on a trampoline or a pezzi ball Opioids may also interfere with mucus drainage by a reduction...

Ngày tải lên: 10/08/2014, 23:20