Involvement of Abdominal Organs in Cystic Fibrosis of the Pancreas

Abstract

Summary Cystic fibrosis of the pancreas is a generalized, hereditary disease affecting many or all exocrine glands. It is not primarily a disease of the pancreas, but one in which this organ is frequently, but not necessarily, involved. In fully developed cases there are chronic lung disease, pancreatic deficiency, abnormally high electrolytes, and, at times, cirrhosis of the liver. Characteristic of the disorder is involvement of various organs or glandular systems, leading to marked variations in the clinical picture. The sweat test because of its simplicity and reliability, is the cornerstone for diagnosis of cystic fibrosis. Involvement of abdominal organs in cystic fibrosis and its consequences are described. Meconium ileus, pancreatic achylia, prolapse of the rectum, cirrhosis of the liver, fecal masses, pancreatic calcifications and diabetes mellitus, and pancreatitis and abdominal pain are described. Case reports are presented on 4 patients with cystic fibrosis exemplifying complications of this disease not recorded previously. Studies relating cystic fibrosis to some chronic pulmonary and gastrointestinal disorders of adults are briefly related and thought to be one of the most challenging recent developments.