Although no special recommendations exist, clearly patients with cystic fibrosis (CF) can benefit from immunisation. We reviewed the literature regarding vaccination in CF and other chronic diseases. CF subjects should follow national immunisation programmes without delay to obtain optimal vaccination coverage. Indeed they may escape normal programmes due to frequent hospital admissions and school absenteeism and may be more at risk to get "vaccine-controlled" diseases at any age. There is no uniform European immunisation schedule for basic infant and childhood vaccines or for vaccines against hepatitis A (HAV) and B (HBV), varicella (VZ) and booster vaccinations. HAV and HBV vaccination is appropriate in CF as recommended in general for patients with chronic liver disease (CLD). Varicella (VZ) vaccination is not recommended in all European countries. There are no recent data about possible worsening of pulmonary status following VZ in CF, but it is known to cause pulmonary damage in non-CF adults and to be potentially fatal post transplantation and during steroid treatment. Therefore it is recommended at least for seronegative adolescents and transplant candidates. Influenza vaccine is recommended annually for CF patients aged > or =6 months. Pneumococcal vaccine is generally indicated for CF patients. RSV infection might play a role in the initial Pseudomonas colonization and the decline in pulmonary function. However no RSV vaccine is available at present. There are no recommendations for palivizumab in CF as an alternative but expensive prophylaxis. Anti-bacterial vaccinations protecting directly against Pseudomonas aeruginosa colonisation are promising for the future, potential candidates are currently being assessed in phase III clinical trials. More studies are needed to complete recommendations especially for CF adults and transplant candidates.

To initiate the complete characterization of mutations in the CFTR gene in Greek cystic fibrosis (CF) patients, we screened 184 patients for six relatively common mutations (delta F 508, G542X, G551D, 621 + 1 G-->T, N1303K, W1282X) using allele-specific hybridization and, in addition, analyzed exons 4, 5, 7, 8, 10, 11, 17b, 19, 20 and 21 using the method of denaturing gradient gel electrophoresis (DGGE). Six mutations accounted for 65.9% of the CF alleles in Greek patients, of which the delta F 508 mutation had a frequency of 52.7%. A further 15 previously described mutations accounted for another 8.3% CF alleles and one previously undescribed mutation (3272-4A-->G) was found in one chromosome. The W1282X mutation was not detected at all. Thus, so far, we have identified 21 mutations in the CFTR gene in Greek CF patients, accounting for 74.5% of the CF alleles.

The relative frequency of the delta F508 mutation in the Greek population is 54.1%; this is similar to that reported in other Southern European populations and contrasts with the considerably higher frequencies encountered in Northern Europe and North America. The low frequency is in agreement with the linkage disequilibrium already reported between cystic fibrosis and haplotype B in this country. In contrast to the common association of pancreatic insufficiency with the homozygous delta F508 genotype, the present study revealed two homozygous children with no evidence of pancreatic failure.

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