UP-TO-DATE MANAGEMENT OF LIVER DISEASE AND COMPLICATIONS IN CYSTIC FIBROSIS AND TRANSITION OF ADOLESCENTS FROM PEDIATRIC TO ADULT CARE

Abstract

Cystic fibrosis (CF) is the most common autosomal recessive lethal hereditary disorder among whites. Survival of patients with CF has progressively improved over the last four decades. As life expectancy has been greatly extended, complications like osteopenia and CF-related diabetes mellitus (CFRDM) occur. In our article, a new management of these two complications is described. Gastrointestinal tract, exocrine pancreas and liver are also affected in CF. Maintaining good nutrition while treating pancreatic insufficiency, treating gastroesophageal reflux disease and treating liver disease are very important issues in the treatment gastrointestinal tract disease in CF. As the CF patient population median survival increases,  a growing number of adolescents require care in the adult health care system which must be capable of responding appropriately to their needs. We present a model of transition from paediatric to adult medical care in our CF center.