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.
The development of new direct acting antiviral drugs for the treatment of Hepatitis C virus infection, has made it possible to obtain an excellent cure rate and extend the indications for eradication of Hepatitis C virus to previously very difficult populations to treat like cystic fibrosis, in whom treatment with classic regimens based on pegylated Interferon plus ribavirin could favor worsening lung function. We present a case of a 41-year-old man with cystic fibrosis, diagnosed with Hepatitis C virus infection, genotype 1a, non-cirrhotic, treated with direct acting antiviral drugs for twelve weeks, obtaining a sustained viral response, without adverse effects. One year later, being on the waiting list, underwent a bipulmonary transplant.
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