162 in Vitro Lymphocyte Functions in the Presence of Bovine Surfactant and Its Phospholipid Fractioins

Abstract

The AF508 deletion is found i n 70%. the R553X mutation i n 5.3% of CF chromosomes in Switzerland. Both mutations show strong linkage to specific haplotypes generated from the marker allele constellation of XV-2c, KM19, MP6d-9, and J3.11 suggesting that patients carrying the same haplotypes may probably show the same mutation. Age of onset of chronic Pse~cdomonas acruginosa colonization (AOCP), X-ray scores (Chrispin-Norman), and relative underweight of 35 patients horliozygous for AF508 (m), 8 patients co111pound heterozygous for AF508 and R553X (AF13) and 13 patients compound heterozygous for AF508 and h;iplotype 6 (AFl6) were compared. I n AF2 and AF13 patients AOCP begins at the age of 7.0 years (1.3-17.4), whereas in the AF16 group the colonisation is already present at the age of 4.3 years (0.4-15.7). The severity of lung disease r;~diogrsphically detennined by the Chrispin-Norman scores is significantly (p=0.03) more progressed in AFl6 patients (score 7) at the age of