Elpis Hatziagorou

Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry

Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF). We analysed forced expiratory volume in 1 s (FEV1) data of 14 732 patients registered in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database in 2007. We used linear and logistic regressions to investigate associations between FEV1 % predicted and clinical outcomes. Body mass index (BMI), chronic infection by Pseudomonas aeruginosa, pancreatic status and CF-related diabetes (CFRD) showed a statistically significant (all p<0.0001) and clinically relevant effect on FEV1 % pred after adjusting for age. Patients with a lower BMI experience a six-fold increased odds ratio (95% CI 5.0–7.3) of having severe lung disease (FEV1 <40% pred) compared to patients with normal BMI. Being chronically infected with P. aeruginosa increases the odds ratio of severe lung disease by 2.4 (95% CI 2.0–2.7), and patients with pancreatic insufficiency experience a 2.0-fold increased odds ratio (95% CI 1.6–2.5) of severe lung disease compared to pancreatic sufficient patients. Patients with CFRD have a 1.8-fold increased odds ratio (95% CI 1.6–2.2) compared to patients not affected. These potential risk factors for pulmonary disease in patients with CF are to some degree preventable or treatable. We emphasise the importance of their early identification through frequent routine tests, the implementation of infection control measures, and a timely initiation of relevant therapies. Independent effect of nutrition, chronic Pseudomonas aeruginosa infection and CF-related diabetes on FEV1 in CF patients http://ow.ly/qhAXJ

Determinants of Quality of Life in Children With Asthma

HRQoL in children with asthma depends on multiple factors, among which asthma severity and level of control are believed to play a vital role. The determinants of the connection between asthma severity and asthma control with quality of life remain unclear.

Aspergillus and the paediatric lung

Aspergillus spp produce a wide range of saprophytic and invasive syndromes in the lungs, including allergic bronchopulmonary aspergillosis (ABPA), aspergilloma and invasive pulmonary aspergillosis (IPA). ABPA results from hypersensitivity to the fungus, and mainly affects patients with asthma or cystic fibrosis (CF). The treatment of choice consists of systemic corticosteroids and itraconazole. Aspergilloma is managed by observation or surgery. IPA is predominantly seen in patients with haematological malignancies, chronic granulomatous disease or immunosuppressive treatment. With the use of aggressive therapies for end-stage CF, such as heart-lung transplantation, the potential for a patient to convert from colonization or ABPA to IPA has increased. Suggestive clinical and radiological findings, supplemented with mycological data using serology and molecular biology, have enhanced the capacity to diagnose IPA in paediatric patients. While voriconazole is considered the first-line therapy in IPA, several other antifungal agents may be appropriate alternatives.

High resolution computed tomography of the chest in cystic fibrosis (CF): is simplification of scoring systems feasible?

The purpose of this study was to simplify HRCT scoring systems (SS) for CF by selecting representative HRCT parameters. Forty-two consecutive patients with CF underwent baseline and follow-up chest HRCT. Three radiologists evaluated 84 HRCTs employing five SS. “Simplified” HRCT SS were formed by selection of parameters exhibiting statistically significant relations with FEV1. Pulmonary function tests (PFTs) and nutrition (IBW%) were recorded. Regression analysis, Pearson correlation and T-test were used for statistical analysis. Three HRCT parameters were selected for the formation of “simplified” HRCT SS (severity of bronchiectasis, bronchial wall thickening, atelectasis-consolidation) using regression analysis. There was excellent correlation between each “simplified” and corresponding complete score (0.892 < r < 0.0967, p < 0.0001) or the remaining four complete scores (0.786 < r < 0.961, p < 0.0001). Strong correlation was found among the five “simplified” scores (0.803 < r < 0.997, p < 0.0001). Comparing baseline complete and “simplified” scores with corresponding follow-up ones, significant worsening was observed (p < 0.0001). PFTs and IBW% did not change significantly. HRCT scores correlated moderately with FVC and FEV1, but there was no correlation with FEF25-75 and IBW%. “Simplified” HRCT SS are as reliable as the complete ones and detect progression of lung disease earlier than clinical parameters. They are easy to use and could be adopted in clinical practice.

Comparison in asthma and allergy prevalence in the two major cities in Greece: the ISAAC phase II survey

BACKGROUND Variability in the prevalence of allergic diseases has been detected not only between different countries, but also between cities with similar national and different socio-economic or climatic characteristics. The aim of this study was to determine the prevalence of childhood asthma and allergies and which factors are associated with them, in the two largest Greek cities, Athens and Thessaloniki. METHODS Two thousand and twenty-three Greek 9-10-year-old schoolchildren from Athens (Central Greece) and Thessaloniki (North Greece, more humid), were included in ISAAC-II study. All participants followed the ISAAC-II protocol by questionnaire, skin prick testing and flexural dermatitis examination. RESULTS Compared with Athens, the prevalence of current wheezing (8.4% vs. 5.7%, p=0.002), lifetime asthma (11.5% vs. 7.7%, p=0.004), atopic current asthma (3.2% vs. 1.6%, p=0.02), allergic rhinitis (8.2% vs. 5.2%, p=0.007), and hay fever (21.7% vs. 12.5%, p<0.001) were higher in Thessaloniki. The overall sensitisation rate was also higher in Thessaloniki than in Athens (25.2% vs. 16%, p<0.001) with more prevalent sensitising due to the perennial allergens (D. pteronyssinus, D. farinae, Alternaria tenuis) and cat dander. Perennial allergens sensitisation was a risk factor for current asthma in both cities. CONCLUSION A higher prevalence of asthma symptoms, allergic rhinitis, and sensitisation rate was detected in Greek schoolchildren living in Thessaloniki compared to those in Athens. Allergy to mites and mould was more prevalent in Thessaloniki. The more humid weather of Thessaloniki may be implicated.

Exercise responses are related to structural lung damage in CF pulmonary disease

Early detection of lung disease is a primary objective in monitoring patients with Cystic Fibrosis (CF); High‐Resolution‐Computed‐Tomography (HRCT) assesses structural damage. Spirometry and cardiopulmonary exercise testing are used for functional evaluation of CF lung disease.

Long‐term outcome of parapneumonic effusions in children: Lung function and exercise tolerance

a: To evaluate the long‐term outcome of parapneumonic effusions (PPE) in children regarding lung function and exercise tolerance, (b) to investigate the role of bronchial asthma in the outcome of PPE.

Pancreatic Cystosis in Two Adolescents with Cystic Fibrosis

We present pancreatic cystosis in two adolescents with cystic fibrosis, a 13-year-old girl and an 18-year-old boy. In pancreatic cystosis, which is a rare manifestation of CF, the pancreatic parenchyma is replaced with multiple cysts of different sizes. Pancreatic cystosis is mainly an imaging based diagnosis and frequent follow-up should be recommended.

WS13.3 Lung clearance index: A tool to assess the response to intravenous treatment among children with cystic fibrosis

Lung Clearance Index (LCI) is a promising endpoint for use in CF clinical trials. Since correlation with validated clinical endpoints has not yet been established, we investigated the association between baseline LCI and risk of respiratory tract exacerbations (RTE) in children with CF. Methods: During a prospective observational study, baseline LCI (N2 washout), FEV1 and CFQR respiratory domain (CFQRres) were measured. RTE, defined as an increase in respiratory symptoms treated with IV antibiotics, were recorded during one year. Whether basline LCI predicted RTE was assessed with a Poisson regression model and Kaplan–Meier plots. LCI z-scores were calculated from values in 57 healthy children. Results: In 63 children with CF (median age 12.4 years, range 5−19), mean LCI z-score was 5.3 (SD 4.6) and mean FEV1 z-score −0.9 (SD 1.3). CFQRres correlated with LCI (R = −0.43, p< 0.001), but not with FEV1 (R=0.24, p = 0.051). In the 53 patients with a normal FEV1, CFQRres and LCI were still correlated (R = −0.44, p = 0.002). During the 12 months follow up, 25 patients (40%) experienced 47 RTE. LCI and FEV1 were predictors of RTE. Time to first RTE decreased with worsening LCI quartiles (Log Rank test, p< 0.001). Similarly, compared to the quartile with the lowest LCI, yearly RTE rate ratio in increasing LCI quartiles was 2.8 (95%CI 0.6–13.9, p = 0.205), 4.7 (95%CI 1.0–21.4, p = 0.046) and 13.6 (95%CI 3.2–57.0, p< 0.001). In the group with normal FEV1, LCI but not FEV1 z-score was still a predictor of RTE. Conclusion: Baseline LCI predicts the risk of RTE in children with CF, even in the subgroup with normal FEV1. These data further support the use of LCI as surrogate outcome in CF clinical trials.

Effect of allergic bronchopulmonary aspergillosis on FEV 1 in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis

Objective To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV1 percent predicted in children and adolescents with cystic fibrosis. Design Longitudinal data analysis (2008–2010). Setting Patients participating in the European Cystic Fibrosis Society Patient Registry. Participants 3350 patients aged 6–17 years. Main outcome measure FEV1 percent predicted was the main outcome measure (one measurement per year per child). To describe the effect of ABPA (main explanatory variable) on FEV1 while controlling for other prognostic factors, a linear mixed effects regression model was applied. Results In 2008, the mean (±SD) FEV1 percent predicted was 78.6 (±20.6) in patients with ABPA (n=346) and 88 (±19.8) in those without ABPA (n=2806). After considering other variables, FEV1 in subjects with ABPA on entry to the study was 1.47 percentage points lower than FEV1 in patients of similar age without ABPA (p=0.003). There was no FEV1 decline associated with ABPA over the subsequent study years as the interaction of ABPA with age was not significant (p>0.05). For patients aged 11.82 years (population mean age), poor body mass index had the greatest impact on FEV1 in 2008, followed by high-risk genotype (two severe mutations), female gender, diabetes mellitus, chronic Pseudomonas aeruginosa infection and ABPA in descending order of effect size. Conclusions In contrast to the common clinical belief of ABPA having a serious impact on lung function, the difference in FEV1 between young patients with and without the complication was found to be modest when the effect of other prognostic factors was considered.