Pancreatitis in Cystic Fibrosis and CFTR-Related Disorder

Abstract

Acute Pancreatitis (AP) in approximately 80% of cases, occurs as a secondary complication related to gallstone disease and alcohol misuse. However there are several other different causes that produce it such as metabolism, genetics, autoimmunity, post-ERCP, and trauma for example... This disease is commonly associated with the sudden onset of upper abdominal pain that is usually severe enough to warrant the patient seeking urgent medical attention. Overall, 10-25% of AP episodes are classified as severe. This leads to an associated mortality rate of 7-30% that has not changed in recent years. Treatment is conservative and generally performed by experienced teams often in ICUs. Although most cases of acute pancreatitis are uncomplicated and resolve spontaneously, the presence of complications has a significant prognostic importance. Necrosis, hemorrhage, and infection convey up to 25%, 50%, and 80% mortality, respectively. Other complications such as pseudocyst formation, pseudo-aneurysm formation, or venous thrombosis, increase morbidity and mortality to a lesser degree. The presence of pancreatic infection must be avoided.