142 Immunisation coverage in children with cystic fibrosis: a French multicenter survey

Abstract

Objectives: To determine platelet aggregation capability for various variants of antigene system AB0 in patients with cystic fibrosis (CF). Methods: 55 CF children (homozygous and heterozygous on F508 del mutation) were enrolled. 46% girls and 54% boys had mild form of CF, 54% girls and 46% boys had severe form. We investigated the function of platelet aggregation with: thrombin, adenosinediphosphate (ADP) and arachidonic acid (AA). Results: Patients with severe form of CF most frequently (29%) had 0 (I); B (III) have not been revealed in this group, meanwhile 22.2% patients with mild form of CF had A (II) and AB (IV) have been revealed. Platelet aggregation with ADP has significant differences between blood groups A (II) and 0 (I), (p = 0.019). Values were ranged 2.5–97.5 percentile in patients with 0 (I) and 2.5−25 percentile in A (II). Data of aggregation with AA did not show significant differences between groups. Significant difference between homozygous F508 del and heterozygous F508 del patients was shown in aggregation with trombin (p = 0.03). There is a tendency to hyperaggregation in heterozygous patients meanwhile homozygous had both hypoaggregation and hyperaggregation. Difference on other tests between homozygous and heterozygous patients is not significant. Conclusion: First results of this study should be interpreted according to system ABO, severity of disease and genotype in patients with CF. These aspects play an important role for individual treatment.