Multisystem disorder of exocrine glands that affects the lungs, intestines, pancreas and liver
Characterised by elevated sodium and chloride ion concentrations in sweat
Organ damage due to blockage of narrow passages by thickened secretions
Chronic pulmonary disease arises from plugging of bronchi and bronchioles
Meconium may set in a sticky mass and produce intestinal obstruction (meconium ileus)
Secretions precipitate in the lumen of the pancreatic duct causing blockage, which results in duct ectasia and fatty replacement of exocrine acinar tissue
Pancreatic exocrine insufficiency leads to fat malabsorption
Steatorrhoea is usually present from birth
Liver may become cirrhotic as a result of bile duct plugging
Failure of complete rotation of the ventral pancreatic bud during development, so that a ring of pancreatic tissue surrounds the second or third part of the duodenum
Annular pancreas is most often seen in association with congenital duodenal stenosis or atresia and is therefore more prevalent in children with Down syndrome
Urgent surgical exploration, distal pancreatectomy if transection in body or tail, haemostasis and drainage for head injury, pancreatoduodenectomy for severe head and duodenal injury