CHANGES OF PANCREATIC AND RESPIRATORY-FUN CTION IN CYSTIC-FIBROSIS - THE INFLUENCE OF THE TIME OF EVOLUTION OF THE DISEASE

BACKGROUND: Cystic fibrosis is the most frequent congenital disease in Caucacians and is transmitted by recessive autosomic inheritance. It is characterized by affection of different glands of exocrine secretio n, particularly the pancreas and the lung. The aim of this study was t o analyze the degree of alteration of pulmonary and pancreatic exocrin e function in a group of patients with cystic fibrosis in relation to the time of disease evolution. METHODS: Twenty-one patients between 9 and 31 years of age were studied; 11 with an evolution of lower than o r equal to 158 months and 10 with an evolution of higher than 158 mont hs (median of the total patients). To study pancreatic exocrine functi on the BT-PABA test and immunoreactive serum trypsin test were used. T o evaluate respiratory function FEV(1), FVC, FEV(1)/FVC ratio and PaO2 were used. RESULTS: The results obtained demonstrated that in the gro up with a lower time of evolution the diagnosis had been carried out a t earlier ages (17+/-17 months versus 84+/-60 months; p=0.002) and pre sented a significantly more alterated pancreatic exocrine function (BT -PABA: 13+/-12 % versus 35+/-23 %; p=0.013). However, respiratory func tion was alterated in the group with longer time of evolution (FEV(1): 68+/-20 % versus 36+/-23 %; p=0.003; FVC: 74+/-9 versus 52+/-25 %; p= 0.013; FEV(1)/FEV: 77+/-19 versus 50+/-9 %; p<0.001; PaO2: 84+/-16 ver sus 58+/-11 %; p<0.001). CONCLUSIONS: Pancreatic exocrine function is most intensely affected in patients diagnosed with cystic fibrosis at earlier and with shorter times of evolution while patients who have th e longest time of evolution and who were diagnosed later in life prese nted greater changes in respiratory function.