الفهرس | Only 14 pages are availabe for public view |
Abstract from this study, we may conclude that: • Cystic fibrosis related liver disease was found in 38% of the studied patients and it was significantly correlated with age at diagnosis, disease duration, male sex, history of meconium ileus, diabetes mellitus, impaired growth and nutrition, severe cystic fibrosis, severe mutations of the cystic fibrosis transmembrane conductance regulator gene (the CFTR gene) and patients uncompliant on pancreatic enzyme replacement therapy and fat soluble vitamins replacement therapy. • The longer the duration of disease and the smaller the age at diagnosis, the more liability to CFLD development. • Male sex and severe genetic mutations are independent risk factors for CFLD development. • A history of meconium ileus and pancreatic insufficiency are predictive of liver disease. • Diabetes mellitus was found to significantly increase the risk of developing CFLD. • Patients with CFLD have impaired growth and nutrition. • Incidence of CFLD increased in patients uncompliant on pancreatic enzyme replacement therapy and fat soluble vitamins replacement therapy. |