Impacto de la afectación hepatobiliopancreática y el estatus nutricional en la sobrevida de adultos con fibrosis quística

The increase survival of adult patients with Cystic Fibrosis (CF) has generated a greater relevance of extra pulmonary disease. Objective. Determine digestive, nutritional commitment and its impact on the survival of adult patients with CF. Methods. Retrospective, descriptive and comparative study of 100 adult patients with CF, assisted between 2003 and 2017. It was evaluated: clinical parameters, genetic, body mass index (BMI), respiratory function tests, bacteriological sputum, pancreatic insufficiency (PI), CF-related diabetes (CFRD), acute pancreatitis (AP), liver cirrhosis and biliary lithiasis. Results. 100 patients were evaluated [median age: 26 years (range: 16-65)]. Median age at diagnosis: 2 years. Mean BMI 21.5 ± 2.6 SD kg/m2. PI 69/100 (69%), CFRD 29 patients (29%), AP 4 patients (4%). Liver cirrhosis 6 patients (6%) and biliary lithiasis 21 patients (21%).Median Forced Expiratory Volume in first second (FEV1) 50%. Most frequent mutation ΔF508 (54%). Fourteen patients (14%) malnourished. Pseudomonas aeruginosa was the most frequent colonizing microorganism (67%), with a median overall survival of 160 months (95% CI 131-160). We found lower survival in those patients who had PI [(mean 124.2 vs. 136 months (p = 0.4)], malnutrition [median 60 vs. 160 months (p = 0.001)] and cirrhosis [median 36 vs. 160 months (p = 0.003).] Conclusion. Digestive diseases and malnutrition influence negatively in the survival of adult patients with CF.