GUIDELINES FOR MANAGEMENT OF CHILDREN WITH CYSTIC FIBROSIS

Background Cystic fibrosis is the most common autosomal recessive hereditary disease. The clinical consequences include multisystem disease characterised by progressive pulmonary disease leading to respiratory failure, pancreatic disfunction, liver disease that may progress to chirrhosis and men infertility due to atresia of the vas deferens. Early intervention and prevention of lung disease is of paramount importance, since the prognosis of the disease is substantially dependent on chronic respiratory infection and inflammation. Cystic fibrosis is a complex disease requiring a multidisciplinary approach to treatment. Center care by a team of experienced health professionals is essential for optimal patient management and outcome. Conclusions The article presents diagnostic and therapeutic recommendations for the management of children with cystic fibrosis.