Modified Chrispin-Norman chest radiography score for cystic fibrosis: observer agreement and correlation with lung function

Contains fulltext : 96114.pdf ( ) (Closed access)OBJECTIVE: To test observer agreement and two strategies for possible improvement (consensus meeting and reference images) for the modified Chrispin-Norman score for children with cystic fibrosis (CF). METHODS: Before and after a consensus meeting and after developing reference images three observers scored sets of 25 chest radiographs from children with CF. Observer agreement was tested for line, ring, mottled and large soft shadows, for overinflation and for the composite modified Chrispin-Norman score. Correlation with lung function was assessed. RESULTS: Before the consensus meeting agreement between observers 1 and 2 was moderate-good, but with observer 3 agreement was poor-fair. Scores correlated significantly with spirometry for observers 1 and 2 (-0.72<R<-0.42, P < 0.05), but not for observer 3. Agreement with observer 3 improved after the consensus meeting. Reference images improved agreement for overinflation and mottled and large shadows and correlation with lung function, but agreement for the modified Chrispin-Norman score did not improve further. CONCLUSION: Consensus meetings and reference images improve among-observer agreement for the modified Chrispin-Norman score, but good agreement was not achieved among all observers for the modified Chrispin-Norman score and for bronchial line and ring shadows

A pilot trial on safety and efficacy of erythrocyte-mediated steroid treatment in CF patients

<p>Abstract</p> <p>Background</p> <p>Chronic neutrophil inflammation of the respiratory tract tissues plays a key role in the pathogenesis and in prognosis of cystic fibrosis (CF). It is evident that an anti-inflammatory therapy represents an important step in the treatment of CF patients. Corticosteroids and ibuprofen have been proven to slow down the impairment of the pulmonary function in CF patients but their use is limited by the frequency of adverse events. A novel strategy for delivering low doses of steroids for long periods through the infusion of autologous erythrocytes loaded with dexamethasone has been recently set up. A recent study suggested the feasibility of therapy with low doses of corticosteroids delivered through engineered erythrocytes in CF patients. This study presents a further analysis of safety and efficacy of this therapy.</p> <p>Methods</p> <p>The treatment group was not randomised and the assignment was based on the patient's consent. Patients entered the study if they had a forced expiratory volume in 1 second (FEV1) <70%, puberty development completed, pancreatic insufficiency, and chronic pulmonary infection requiring frequent cycles of intravenous antibiotic therapy. Patients were excluded if they underwent systemic corticosteriod therapy in the three months preceding the experimental treatment or were on therapy with non-steroidal anti inflammatory drugs (NASDs), or if they had liver CF disease, allergic bronchopulmonary aspergillosis, or positive tuberculin test. Controls were patients who followed a standard treatment, who fulfilled the enrolment criteria, and who were matched to the experimental group by gender, age, and severity of the disease.</p> <p>Results</p> <p>Nine patients in the experimental group received the treatment once a month for a period of 24 month. Patients did not develop diabetes, cataract, or hypertension, or other typical side effects of steroid treatment during the follow up period. There was a constant improvement of FEV1 in patients undergoing the experimental treatment compared to a gradual decrease of the same parameter in the standard therapy group (P = 0.04). The average of clinic and radiological indexes did not vary. The number of infective relapses that have required antibiotic intravenous therapy was not different in the two groups, although the average of these episodes was slightly higher in the experimental therapy group.</p> <p>Conclusion</p> <p>Intraerythrocyte corticosteroid treatment may stabilize the respiratory function in CF patients but is often considered too invasive by patients. The results obtained by our study may help planning an experimental, controlled, randomised study. A sample size of 150 patients per group would be sufficient for demonstrating such a difference with a 95% confidence interval and a power of 90%.</p

Neutrophil glutamine deficiency in relation to genotype in children with cystic fibrosis

Pulmonary disease in cystic fibrosis (CF) is characterized by a chronic neutrophil-dominated inflammation of lung tissue. Inasmuch as some amino acids (AA) play a pivotal role in various aspects of neutrophil metabolism, the aim of this study was to investigate a possible alteration of neutrophil AA metabolism and to evaluate its relation (if any) with the genotype. We performed plasma and neutrophil AA analysis in 26 CF patients with known Genotype. 10 patients with non-CF bronchiectasis, and 20 normal subjects. The CF group showed a significant decrease of free intracellular neutrophil glutamine (Gin) content compared with controls and the non-CF bronchiectasis group. In the latter group, levels of neutrophil Gin were significantly lower compared with the controls. Amino acid plasma concentration in non-CF bronchiectasis showed a decrease of Gin and taurine compared with controls. Neutrophil Gin content showed values significantly lower in CF patients with severe mutations (class I, II and III mutations) compared with mild mutations (class IV and V mutations). Results of our study add further evidence for intrinsic neutrophil alterations that could play an important role in the pathogenesis of chronic pulmonary disease in CF patients