Background Urban-rural differences in the prevalence of atopy and associated allergic diseases have been reported in several non-European countries. Within Europe, where such variations are less striking, a farm childhood has been postulated to be protective. Objective We aimed to compare the prevalence of atopy in urban and rural children living in Crete and to examine the role of early exposure to a farming environment in explaining any measured differences. Methods A cross-sectional survey of children attending secondary schools in Iraklion, the capital city, and five villages 100 km to the south. Atopy was determined by the responses to skin prick tests with seven common aeroallergens. Results Nine-nundred and ninety-seven children participated in the survey. 19.6% of those living in Iraklion, but only 9.6% of those from the villages, had a positive response to one or more skin prick tests. Among urban children there were significant gradients in the prevalence of atopy across several categories of animal contact and consumption of farm (unpasteurized) milk products ± before the age of 5 years. These differences, however, were not apparent among the rural children.

While farming in Poland differed from that in the Alpine areas as did the exposure-response associations, we found in communities engaged in small-scale, mixed farming, there was a protective farming effect against objective measures of atopy potentially related to contact with grain or associated farm activities.

The very striking differences in the prevalence of allergy between these two neighbouring communities of central Europe reflect the pan-continental population movements that may have been responsible for the emergence of childhood allergies in Europe.

Adult Primary Ciliary Dyskinesia (PCD) has not been well characterised. Patients have varied radiological severity of disease and lung function impairment and limited data is available regarding prognosis. In this retrospective study we describe and characterise a large adult PCD cohort, and identify determinates of disease progression using longitudinal lung function data.We retrospectively analysed 151 adult patients at a single tertiary centre. Overall mortality was 4.6% over a 7-year median follow-up period. Lung function decline was estimated at 0.49%FEV1predicted/year. Older age at diagnosis showed moderate negative correlation with FEV1%predicted at diagnosis (r = -0.30; p = 0.01) and increased Pseudomonas aeruginosa colonisation (p < 0.01) but not longitudinal FEV1%predicted (β = 0.001; (95% CI:-0.35,0.35)). Within multivariate mixed models of FEV1 adjusting for ciliary ultrastructure, HRCT scoring of severity of bronchial wall dilatation (p < 0.01) and extent of bronchiectasis (p = 0.03) showed evidence of modifying the decline in FEV1 with age. Lung function decline additionally differed by ciliary ultrastructure (p = 0.04) with patients with microtubular defects having the greatest decline.Our study reveals a large proportion of adult PCD patients are diagnosed late with lower FEV1 and increased P. aeruginosa colonisation at diagnosis. Increased disease burden on HRCT and microtubular defects on ciliary ultrastructure predicts progressive lung function decline. This study highlights the need for early diagnosis alongside prospective multi-centre disease-specific trials to confirm triggers for lung function decline and identify potential novel therapeutic strategies.

ObjectivesThe UK CF Registry annual reports include comparisons between centres on key outcomes such as FEV1 using rankings. While illustrating the distribution between centres, they promote the assumption that those with the highest measures provide “better” care. We hypothesised a more scientific approach based on statistical “process control” using funnel plots and adjustment for case-mix may help to identify exceptional CF care services in terms of clinically meaningful outcomes.MethodsWe extracted data from annual reviews (2007–2012) on the CF Registry. Our outcomes included FEV1 (% predicted) at 15 years and change in FEV1 between 18 and 21 years. Funnel plots were generated with confidence limits at 2 and 3 standard deviations (SD). Centres with mean values outside these limits are said to display “special cause variation” -variability outside what one would expect. Outcomes were then adjusted for case mix (including gender, genotype, pancreatic sufficiency and socio-economic deprivation) and analysed using funnel plots.Results31 paediatric centres provided FEV1 data on 15 year olds between 2007 and 2012. Funnel plots of unadjusted FEV1 (% predicted) showed few centres with evidence of special cause variation (2SD limits). Initial case-mix adjustment reduced the number of centres outside these limits to 3. We also identified 28 adult centres providing sufficient data to calculate change in FEV1 (% predicted) between 18–21 years. While there was some evidence of special cause variation (at 2SD limits) in prior to case-mix adjustment, after adjustment none were outside the 2SD limits. None of the centres were outside the 3SD limits in either analysis.ConclusionIn conclusion the work to-date illustrates that funnel plots can be used to explore potential differences in FEV1 between specialist centres. Case-mix adjustment models should develop into a useful tool for making centre comparisons which can continue to be used by stakeholders. This is early work, however, and we need to bear in mind that by examining outcomes in small populations risk missing true differences due to low statistical power. Further work is required to assess whether any observed differences are due to chance or are related to the care patients receive.