GUIDELINES FOR MANAGEMENT OF CHILDREN WITH CYSTIC FIBROSIS

Abstract

BACKGROUND Cystic fibrosis is the most common autosomal recessive hereditary disease. The clinicalconsequences include multisystem disease characterised by progressive pulmonary disease leading to respiratory failure, pancreatic disfunction, liver disease that may progressto chirrhosis and men infertility due to atresia of the vas deferens. Early intervention andprevention of lung disease is of paramount importance, since the prognosis of the diseaseis substantially dependent on chronic respiratory infection and inflammation. Cysticfibrosis is a complex disease requiring a multidisciplinary approach to treatment. Centercare 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 fibrosi