Cystic fibrosis

Abstract

Cystic fibrosis is a complex, multiorgan disorder primarily affecting the respiratory and gastrointestinal tract, pancreas and hepatobiliary system. It has an incidence of approximately 1 in 2,500 births. Presentation of cystic fibrosis varies according to age. The commonest presentation of cystic fibrosis is with recurrent respiratory tract infections ± associated failure to thrive, secondary to pancreatic exocrine insufficiency. Eighty-five percent of people with cystic fibrosis are pancreatic insufficient and without treatment they malabsorb fat and present with failure to gain weight, abdominal cramps, abdominal distension and steatorrhea. Ten to fifteen percent of cystic fibrosis presentations are in the neonatal period with meconium ileus which may lead to bowel obstruction, perforation and peritonitis. Older children may present with finger clubbing and respiratory symptoms, nasal polyps or acute pancreatitis.